2020

Risk Factors for Seizures and Epilepsy in Children with Congenital Heart Disease

Risk Factors for Seizures and Epilepsy in Children with Congenital Heart Disease Ghosh S, Philip J, Patel N, Munoz-Pareja J, Lopez-Colon D, Bleiweis M, Winesett SP. J Child Neurol. 2020 Feb 27:883073820904912. doi: 10.1177/0883073820904912. [Epub ahead of print] PMID: 32103693 Similar articles Select item 32107587   Take Home Points: Neonates and infants <3 months with CHD undergoing cardiac surgery are at risk for seizures during the perioperative period and years after cardiac surgery. Children who were more likely to have seizures include those with brain injury, lower birth weight, higher STAT scores, high RACHS category, and genetic syndromes. Those children with CHD who went on to develop epilepsy were more likely to have had an ischemic or hemorrhagic stroke but not necessarily a history of perioperative seizures.   Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch: This is a single institution retrospective cohort study of neonates and infants <3 months of age with congenital heart disease undergoing cardiopulmonary bypass. The objective of this study was to identify potential risk factors for pre- and postoperative seizures and epilepsy in children with congenital heart disease.   The incidence of seizures in children with CHD during their hospitalization is estimated at 8% but increases to 11.5% in children assessed with 48-hour video EEG monitoring. The overall incidence of epilepsy in CHD, but operated and unoperated CHD, is 5% by 15 years of age. Seizure in CHD has been found to be associated with higher RACHS scores, delayed sternal closure, longer hospital stays, and use of ECMO; while epilepsy has been associated with ECMO use and longer hospital stay.   Methods included inclusion of all neonates and infants <3 months undergoing cardiopulmonary bypass. Seizures were identified as clinical with electrographic correlate or electrographic correlate only. All patients underwent imaging (brain MRI or head CT) prior to or after cardiac surgery. Patients were excluded if they did not complete the required postoperative follow-up visits.     Results: In those infants with seizures prior to surgery (n=6), none progressed to epilepsy during their follow up (mean follow up 4.1 years). Early post-operative seizures occurred in 4 patients and only 1 progressed to epilepsy (mean follow up 5.5 years). Children who were more likely to have seizures include those with brain injury, lower birth weight, higher STAT scores, high RACHS category, and genetic syndromes and were associated with delayed sternal closures and longer hospital stay. Epilepsy occurred in 5.3% of this cohort at a mean age of 1.53 years and only a single patient had a seizure during their initial ICU hospitalization. Of those children with epilepsy, 5 weaned off medications, 3 died due to cardiac complications, and 4 developed intractable epilepsy. Children with CHD who went on to develop epilepsy were more likely to have had an ischemic or hemorrhagic stroke.   Discussion: Children with CHD who also suffered a stroke (either ischemic or hemorrhagic) were more likely to develop epilepsy. Other risk factors for seizures include high risk surgery, low birth weight, presence of a genetic syndrome and delayed sternal closure. These findings support that seizures seen during the initial perioperative hospitalization may not lead to the diagnosis of epilepsy.   Limitations: this is a retrospective and single center study. The use of preoperative imaging (head CT and brain MRI) as well as EEG monitoring is not routinely used before or after cardiac surgery and may have contributed to preselection bias. Longer duration of follow up is needed to fully assess the risk for epilepsy in children with CHD undergoing cardiopulmonary bypass surgery.   Next steps: Neonates and infants <30 days with CHD undergoing cardiopulmonary bypass surgery are risk for developing seizures and epilepsy. This requires diligent monitoring with clinical examination and EEG assessment during the perioperative period and later in childhood in those children at higher risk.    

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Identification of Risk Factors for Early Fontan Failure

Identification of Risk Factors for Early Fontan Failure Rochelson E, Richmond ME, LaPar DJ, Torres A, Anderson BR. Semin Thorac Cardiovasc Surg. 2020 Feb 19. pii: S1043-0679(20)30033-2. doi: 10.1053/j.semtcvs.2020.02.018. [Epub ahead of print] PMID: 32087242 Similar articles Select item 32145462   Take Home Points: Despite significant improvements in the perioperative care of single ventricle patients, the risk for lifelong morbidity and mortality following the Fontan procedure persists. Neonates undergoing balloon atrial septostomy are at significant risk for Fontan failure later in life. Other patient characteristics and perioperative events were not associated with Fontan failure in this cohort.   Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch: This is a single center retrospective study to evaluate all patients undergoing a Fontan procedure. The objective of this study was to identify characteristics throughout a patient’s lifespan that might predict early Fontan failure (death, Fontan takedown, heart transplant listing before hospital discharge or <30 days postoperatively).   Methods: Data collected included perioperative patient care (stage I, stage II and stage III), patient and operative characteristics, as well as outcomes. Patients were excluded if they underwent a hybrid stage I or if they underwent stage I and/or stage 2 surgery elsewhere.   Results: A total of 191 patients met inclusion criteria with the most common anatomy being HLHS followed by tricuspid atresia. Relevant stage 1 perioperative characteristics including 8% undergoing balloon atrial septostomy; 2 of which required RF perforation; 56% underwent the Norwood procedure with 29% undergoing isolated shunt placement. Relevant stage II preoperative characteristics were notable for moderate or severe AV valve regurgitation in 12% and moderate or severe systemic ventricular systolic dysfunction in 5%. Most patients underwent a unilateral or bilateral Glenn procedure with approximately half of stage II patients also requiring a pulmonary arterioplasty. Post stage II median chest tube duration was 4 days and medial hospital LOS was 6 days. Prior to stage III, 12% had moderate or severe AV valve regurgitation by echocardiogram. The degree of valvar regurgitation and ventricular dysfunction was highly associated with pre-Stage 2 AV valve regurgitation and ventricular dysfunction. Approximately 50% of pre-Fontan patients required an intervention during their pre-Fontan cath with the most common intervention being coiling of collaterals. Of the Fontans performed, 56% were extracardiac conduits while the remaining were lateral tunnel procedures; 48% were fenestrated. Outcomes included operative deaths (6 patients), Fontan takedown (2 patients) and no patients listed for cardiac transplantation before discharge/30 days post-op.   A neonatal balloon atrial septostomy (BAS) was the only characteristic associated with early Fontan failure at an odds ratio of 8.5. This was not associated with pre-Stage 2 or pre-Fontan cardiac catheterization hemodynamics. No other perioperative characteristic was associated with Fontan failure in this cohort.     Discussion: In this cohort, the incidence of Fontan failure was low and only a single patient characteristic (neonatal BAS) was associated with failure. It is likely that BAS in this cohort represents the physiology of a restrictive atrial septum which has been previously reported to be associated with poorer   Limitations: There is potential selection bias in that patients who died prior to the Fontan procedure or those not deemed good Fontan candidates were excluded from this cohort. This study is also limited by the small number of Fontan failures making regression analyses difficult.   Next Steps: A multicenter study of a larger single ventricle cohort, including patients undergoing Fontan procedure as well as those who died or were felt to be poor Fontan candidates are needed to better determine patient and perioperative characteristics contributing to the inability to undergo a Fontan or subsequent Fontan failure.    

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Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis

Prediction of prognosis in patients with tetralogy of Fallot based on deep learning imaging analysis Diller GP, Orwat S, Vahle J, Bauer UMM, Urban A, Sarikouch S, Berger F, Beerbaum P, Baumgartner H; German Competence Network for Congenital Heart Defects Investigators. Heart. 2020 Mar 11. pii: heartjnl-2019-315962. doi: 10.1136/heartjnl-2019-315962. [Epub ahead of print] PMID: 32161041 Similar articles Select item 32159755   Take Home Points Machine learning algorithms applied to cardiac magnetic resonance (CMR) images can automatically estimate prognosis in patients with repaired tetralogy of Fallot (ToF) The current study highlights the prognostic value of automatically derived right atrial area and biventricular dysfunction in patients with ToF. Enlarged right atrial median area (HR 1.11/ cm², p=0.003) and reduced right ventricular long-axis strain (HR 0.80/%, p=0.009) was associated with adverse endpoint of death/aborted cardiac arrest or documented ventricular tachycardia (defined as >3 documented consecutive ventricular beats). Machine learning algorithms trained on external imaging datasets can automatically estimate prognosis in patients with ToF.     Comment from Dr. Shaji Menon (Salt Lake City, Utah), Lead Section editor of Pediatric Cardiology Journal Watch: This study assesses the utility of machine learning algorithms for automatically estimating prognosis in patients with repaired tetralogy of Fallot (ToF) using Cardiac magnetic resonance (CMR). The study included 372 patients with ToF who had undergone CMR imaging as part of German National Registry for Congenital Heart Disease between 2003 and 2009. Cine loops were retrieved and subjected to automatic deep learning (DL)-based image analysis, trained on independent, local CMR data, to derive measures of cardiac dimensions and function. This information was combined with established clinical parameters and ECG markers of prognosis. Over a median follow-up period of 10 years, 23 patients experienced an endpoint of death/aborted cardiac arrest or documented ventricular tachycardia (defined as >3 documented consecutive ventricular beats). On univariate Cox analysis, various DL parameters, including right atrial median area (HR 1.11/ cm², p=0.003) and right ventricular long-axis strain (HR 0.80/%, p=0.009) emerged as significant predictors of outcome. DL parameters were related to adverse outcome independently of left and right ventricular ejection fraction and peak oxygen uptake (p<0.05 for all). A composite score of enlarged right atrial area and depressed right ventricular longitudinal function identified a ToF subgroup at significantly increased risk of adverse outcome (HR 2.1/unit, p=0.007). Risk stratification models for patients with ToF have traditionally included a variety of parameters, including age at repair, duration of preoperative cyanosis, previous atrial arrhythmias, QRS duration or fragmentation on ECG, biventricular dysfunction, right atrial area, RV hypertrophy, elevated left ventricular end-diastolic pressure and natriuretic peptides. This study for the first time highlights the prognostic value of automatically derived right atrial area and biventricular dysfunction in patients with ToF.                

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Lifetime cardiovascular management of patients with previous Kawasaki disease

Lifetime cardiovascular management of patients with previous Kawasaki disease Brogan P, Burns JC, Cornish J, Diwakar V, Eleftheriou D, Gordon JB, Gray HH, Johnson TW, Levin M, Malik I, MacCarthy P, McCormack R, Miller O, Tulloh RMR; Kawasaki Disease Writing Group, on behalf of the Royal College of Paediatrics and Child Health, and the British Cardiovascular Society. Heart. 2020 Mar;106(6):411-420. doi: 10.1136/heartjnl-2019-315925. Epub 2019 Dec 16. Review. PMID: 31843876 Free PMC Article Similar articles Select item 31806699   Take Home Points Kawasaki disease (KD) is an inflammatory disorder of young children, associated with vasculitis of the coronary arteries with subsequent aneurysm formation in up to one-third of untreated patients.. Ptients who develop coronary artery aneuryms (CAA) are at life-long risk of coronary thrombosis or the development of stenotic lesions, which may lead to myocardial ischaemia, infarction or death. Follow-up in a specialized Kawaski disease clinic with experts in Kawaski diseasee and intervnetional cardiology will help better coordinate care. An adult with a past history of KD and CAA, who presents with any symptoms or signs which could be due to aneurysm thrombosis or acute coronary syndrome, should be transferred promptly to a Heart Attack Centre (HAC). Every child or adult followed-up for CAA should have a person-specific protocol (PSP) written, detailing the pathway of care to be followed if a suspected acute coronary syndrome should occur. This consensus statement from the European Royal College of Paediatrics and Child Health, and the British Cardiovascular Society guidance on th llong-term management of patients who have vascula complications of KD and guidance on the emergency management of acute coronary complications.     Comment from Dr. Shaji Menon (Salt Lake City, Utah), Lead Section Editor of Pediatric Cardiology Journal Watch: Table 1 shows the imaging surveillence protocols for short-term and long-term cornary surveillance and evaluation of myocardial perfusion by level of patient risk. Patients with small or remodelled aneurysms may be seen less frequently, but those with giant aneurysms need regular imaging and assessment to detect developing thrombi within aneurysms, particularly in the early years after the acute KD illness, when the risk of thrombosis is greatest.   Table 2 suggests additional tests to be undertaken during a visit, and in the transition period from paediatric to adult care.    

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Identification of Risk Factors for Early Fontan Failure

Identification of Risk Factors for Early Fontan Failure Rochelson E, Richmond ME, LaPar DJ, Torres A, Anderson BR. Semin Thorac Cardiovasc Surg. 2020 Feb 19. pii: S1043-0679(20)30033-2. doi: 10.1053/j.semtcvs.2020.02.018. [Epub ahead of print] PMID: 32087242 Similar articles Select item 32145462   Take Home Points: Despite significant improvements in the perioperative care of single ventricle patients, the risk for lifelong morbidity and mortality following the Fontan procedure persists. Neonates undergoing balloon atrial septostomy are at significant risk for Fontan failure later in life. Other patient characteristics and perioperative events were not associated with Fontan failure in this cohort.     Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch: This is a single center retrospective study to evaluate all patients undergoing a Fontan procedure. The objective of this study was to identify characteristics throughout a patient’s lifespan that might predict early Fontan failure (death, Fontan takedown, heart transplant listing before hospital discharge or <30 days postoperatively).   Methods: Data collected included perioperative patient care (stage I, stage II and stage III), patient and operative characteristics, as well as outcomes. Patients were excluded if they underwent a hybrid stage I or if they underwent stage I and/or stage 2 surgery elsewhere.   Results: A total of 191 patients met inclusion criteria with the most common anatomy being HLHS followed by tricuspid atresia. Relevant stage 1 perioperative characteristics including 8% undergoing balloon atrial septostomy; 2 of which required RF perforation; 56% underwent the Norwood procedure with 29% undergoing isolated shunt placement. Relevant stage II preoperative characteristics were notable for moderate or severe AV valve regurgitation in 12% and moderate or severe systemic ventricular systolic dysfunction in 5%. Most patients underwent a unilateral or bilateral Glenn procedure with approximately half of stage II patients also requiring a pulmonary arterioplasty. Post stage II median chest tube duration was 4 days and medial hospital LOS was 6 days. Prior to stage III, 12% had moderate or severe AV valve regurgitation by echocardiogram. The degree of valvar regurgitation and ventricular dysfunction was highly associated with pre-Stage 2 AV valve regurgitation and ventricular dysfunction. Approximately 50% of pre-Fontan patients required an intervention during their pre-Fontan cath with the most common intervention being coiling of collaterals. Of the Fontans performed, 56% were extracardiac conduits while the remaining were lateral tunnel procedures; 48% were fenestrated. Outcomes included operative deaths (6 patients), Fontan takedown (2 patients) and no patients listed for cardiac transplantation before discharge/30 days post-op.   A neonatal balloon atrial septostomy (BAS) was the only characteristic associated with early Fontan failure at an odds ratio of 8.5. This was not associated with pre-Stage 2 or pre-Fontan cardiac catheterization hemodynamics. No other perioperative characteristic was associated with Fontan failure in this cohort.     Discussion: In this cohort, the incidence of Fontan failure was low and only a single patient characteristic (neonatal BAS) was associated with failure. It is likely that BAS in this cohort represents the physiology of a restrictive atrial septum which has been previously reported to be associated with poorer.   Limitations: There is potential selection bias in that patients who died prior to the Fontan procedure or those not deemed good Fontan candidates were excluded from this cohort. This study is also limited by the small number of Fontan failures making regression analyses difficult.   Next Steps: A multicenter study of a larger single ventricle cohort, including patients undergoing Fontan procedure as well as those who died or were felt to be poor Fontan candidates are needed to better determine patient and perioperative characteristics contributing to the inability to undergo a Fontan or subsequent Fontan failure.    

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Risk Factors for Seizures and Epilepsy in Children With Congenital Heart Disease

Risk Factors for Seizures and Epilepsy in Children With Congenital Heart Disease Ghosh S, Philip J, Patel N, Munoz-Pareja J, Lopez-Colon D, Bleiweis M, Winesett SP. J Child Neurol. 2020 Feb 27:883073820904912. doi: 10.1177/0883073820904912. [Epub ahead of print] PMID: 32103693 Similar articles Select item 32107587   Take Home Points: Neonates and infants <3 months with CHD undergoing cardiac surgery are at risk for seizures during the perioperative period and years after cardiac surgery. Children who were more likely to have seizures include those with brain injury, lower birth weight, higher STAT scores, high RACHS category, and genetic syndromes. Those children with CHD who went on to develop epilepsy were more likely to have had an ischemic or hemorrhagic stroke but not necessarily a history of perioperative seizures.     Commentary from Dr. Charlotte Van Dorn (Rochester, MN), section editor of Pediatric Cardiology Journal Watch: This is a single institution retrospective cohort study of neonates and infants <3 months of age with congenital heart disease undergoing cardiopulmonary bypass. The objective of this study was to identify potential risk factors for pre- and postoperative seizures and epilepsy in children with congenital heart disease.   The incidence of seizures in children with CHD during their hospitalization is estimated at 8% but increases to 11.5% in children assessed with 48 hour video EEG monitoring. The overall incidence of epilepsy in CHD, but operated and unoperated CHD, is 5% by 15 years of age. Seizure in CHD has been found to be associated with higher RACHS scores, delayed sternal closure, longer hospital stay, and use of ECMO; while epilepsy has been associated with ECMO use and longer hospital stay.   Methods included inclusion of all neonates and infants <3 months undergoing cardiopulmonary bypass. Seizures were identified as clinical with electrographic correlate or electrographic correlate only. All patients underwent imaging (brain MRI or head CT) prior to or after cardiac surgery. Patients were excluded if they did not complete the required postoperative follow-up visits.     Results: In those infants with seizures prior to surgery (n=6), none progressed to epilepsy during their follow up (mean follow up 4.1 years). Early post-operative seizures occurred in 4 patients and only 1 progressed to epilepsy (mean follow up 5.5 years). Children who were more likely to have seizures include those with brain injury, lower birth weight, higher STAT scores, high RACHS category, and genetic syndromes and were associated with delayed sternal closures and longer hospital stay. Epilepsy occurred in 5.3% of this cohort at a mean age of 1.53 years and only a single patient had a seizure during their initial ICU hospitalization. Of those children with epilepsy, 5 weaned off medications, 3 died due to cardiac complications, and 4 developed intractable epilepsy. Children with CHD who went on to develop epilepsy were more likely to have had an ischemic or hemorrhagic stroke.   Discussion: Children with CHD who also suffered a stroke (either ischemic or hemorrhagic) were more likely to develop epilepsy. Other risk factors for seizures include high risk surgery, low birth weight, presence of a genetic syndrome and delayed sternal closure. These findings support that seizures seen during the initial perioperative hospitalization may not lead to the diagnosis of epilepsy.   Limitations: this is a retrospective and single center study. The use of preoperative imaging (head CT and brain MRI) as well as EEG monitoring is not routinely used before or after cardiac surgery and may have contributed to preselection bias. Longer duration of follow up is needed to fully assess the risk for epilepsy in children with CHD undergoing cardiopulmonary bypass surgery.   Next steps: Neonates and infants <30 days with CHD undergoing cardiopulmonary bypass surgery are risk for developing seizures and epilepsy. This requires diligent monitoring with clinical examination and EEG assessment during the perioperative period and later in childhood in those children at higher risk.    

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The Combined Usefulness of the Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratios in Predicting Intravenous Immunoglobulin Resistance with Kawasaki Disease

The Combined Usefulness of the Neutrophil-to-Lymphocyte and Platelet-to-Lymphocyte Ratios in Predicting Intravenous Immunoglobulin Resistance with Kawasaki Disease.   Kawamura Y, Takeshita S, Kanai T, Yoshida Y, Nonoyama S.J Pediatr. 2016 Nov;178:281-284.e1. doi: 10.1016/j.jpeds.2016.07.035. Epub 2016 Aug 12.PMID: 27526622   Take-Home Points:   In patients with Kawasaki Disease, high Neutrophil to Lymphocyte ratio (NLR) and high Platelet to Lymphocyte ratio (PLR) before initiation of IVIG independently predicted IVIG resistance. The combination of NLR and ILR is a more reliable predictor than either of them used alone. The combination of these simple ratios calculated from the findings of standard blood test has the predictive ability of IVIG resistance almost equal to the traditional scoring systems     Commentary from Dr. Venugopal Amula (Salt Lake City UT), section editor of Pediatric Cardiology Journal Watch: In Kawasaki Disease, resistance to traditional therapy with IVIG and Aspirin occurs not infrequently and puts the patients at higher risk of developing coronary artery lesions.   Many scoring systems and laboratory biomarkers have been evaluated to predict IVIG resistance, as changes in treatment strategy may alter coronary artery involvement.   Kanai et al. report the utility of two blood cell type ratios, Neutrophil to Lymphocyte ratio( NLR) and Platelet to Lymphocyte Ratio, calculated before initiation of IVIG in predicting the ability of IVIG resistance in KD patients. The current report is an expansion of the same authors' pilot study that suggested the benefit of the combination of these ratios in predicting IVIG resistance. Small sample size limited the single-center pilot study.   . The current investigation is a retrospective, a multicenter cohort study of KD patients from seven hospitals in Japan admitted between March 2004 to Feb 2017. The datasets were divided into the development dataset (March 2004 to Mar 2014) and the validation cohort (April 2014 to Feb 2017).   Classic diagnostic criteria were applied, and IVIG resistance was defined as persistent fever lasting > 24 hrs. after IVIG completion or recrudescent fever after an afebrile period.   All patients were treated with a standard initial treatment regimen of 30mg/kg/d of Aspirin and 2g/kg/d of IVIG. Patients with incomplete KD, late diagnosis, coronary involvement before the initial treatment, and intensive nonstandard initial treatment regimen were excluded.   Receiver operating characteristic curves were constructed using the development dataset, and the most discriminating cut off values was used to assess sensitivity, specificity, PPV, and NPV when applied to NLR and PLR in predicting IVIG resistance. The predictive marker obtained was applied to the validation dataset to determine predictive accuracy.   A total of 852 patients were analyzed, with 520 representing the development dataset and the rest validation dataset. IN both the datasets, the NLR, PLR, cell count of neutrophils, ALT, AST, CRP Gunma score, and Kurume score were significantly higher in the IVIG resistant group than the active group. A ROC analysis showed the best cut off values of NLR and PLR were 4.11 and 119, respectively. Multiple regression analysis of the age at onset and other variables with a significant difference between the IVIG responsive and the resistant groups in the development dataset showed that both the NLR> or = 4.11 and PLR> or = 119 were independent predictors of IVIG resistance in KD with OR of 2.86 and 1.95 respectively. When combined the adjusted odds ratio was higher at 4.33 ( 95% CI 2.43-7.71; p<0.001).   The new predictive marker of the combination of NLR > 4.11 and PLR > 119 had a significant high adjusted OR of 2.6 in the validation set, suggesting it was as accurate in predicting IVIG resistance in a different dataset. The sensitivity and specificity of the new predictive in the validation data set were 0.54 and 0.72, almost the same in the development dataset. The marker also performed equally well, compared to the Gunma score and Kurume score in predicting IVIG resistance.   The authors present an easy-to-calculating predictive marker of a combination of NLR and PLR to predict IVIG resistance. Whether this novel and low-cost evaluation to predict IVIG resistance could be applied to different patient populations across the world is uncertain, but the authors opine that as these ratios simply focus on response to inflammation, they should work regardless of ethnicity.                                

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Treatment strategies for protein-losing enteropathy in Fontan-palliated patients

Treatment strategies for protein-losing enteropathy in Fontan-palliated patients. Schleiger A, Ovroutski S, Peters B, Schubert S, Photiadis J, Berger F, Kramer P. Cardiol Young. 2020 May;30(5):698-709. doi: 10.1017/S1047951120000864. Epub 2020 May 4.PMID: 32364090   Take-Home Points:   Protein-losing enteropathy (PLE) occurred in 30 out of 439 (6.8%) Fontan patients managed at a single institution during the study period of 1986-2019, and it developed at the median of 2.9 years after Fontan operation. Cardiopulmonary perturbations in the form of unilateral diaphragmatic paralysis or central PA stenosis or Fontan pathway obstruction or bradyarrhythmia were present at manifestation or during the disease in 25 of 30 patients (83%). The factors significantly associated with the development of PLE in this cohort were bradyarrhythmia, phrenic nerve palsy, and stenosis within the Fontan pathway. Patency of Fontan fenestration was associated with decreased odds of PLE. The treatment strategies were varied, including surgical management of phrenic palsy with diaphragm plication, pacemaker implantation for bradyarrhythmia, and percutaneous cath interventions aimed at relieving the Fontan pathway obstruction. Medical therapies were predominantly a combination of pulmonary vasodilator therapy and anti-inflammatory therapy with budesonide and diuretics and heart failure therapy. Between all the treatment strategies, stable remission, defined as regression of symptoms and elevated serum albumin levels >3.5g/dl over 12 months, was seen among 15 patients (50%) at the time of the study. The somatic growth and physical development was significantly delayed in Fontan patients associated with PLE than non-PLE cohort as compared by z-scores for height and body weight. The sexual development was also delayed but not statistically significant. The mortality in this cohort was 33.3% and occurred after a median of 7.2 years from the onset of the disease. The survival estimates declined from 96.1% at five years to 70.5 % at 10 and 50.1% at 20 years. Nonsurvivors had lower serum albumin and total protein levels, higher pulmonary artery pressure, and impaired ventricular function, at last follow-up compared to survivors. Lack of indications for surgical or cath-based interventions and failure to respond to medical treatment with no significant improvement or remission within 6-12 months should prompt cardiac transplantation evaluation.     Commentary from Dr. Venugopal Amula (Salt Lake City UT), section editor of Pediatric Cardiology Journal Watch: Protein Losing Enteropathy is an infrequent but critical complication that limits survival in Fontan patients – the exact pathophysiological mechanism of which remains unknown. This current report by A. Schleiger et al. is a retrospective, observational study of Fontan patients followed up at a single institution between 1986 and 2019. The authors identified a cohort of 439 patients, of which 327 were operated in their institution. They retrospectively applied a proposed universal diagnostic definition of protein-losing enteropathy in Fontan patients and identified 30 patients with the disease condition. PLE was defined by the criteria of the combination of persistent diarrhea or recurring edema or pleural effusions or ascites, decreased serum albumin levels, and total serum protein levels and confirmation of intestinal protein loss with increased fecal alpha antitrypsin levels.   Demographic, clinical, laboratory, and echocardiographic data were abstracted from institutional medical records. Surgical and hemodynamic data detailing the Fontan surgery was also retrieved. Standard descriptive statistics were applied survival, and freedom from PLE was assessed using Kaplan Meier Survival analysis.   The PLE patients had a long median follow up of 13.1 years. The long follow up helped them chronicle the anthropometric, clinical, hemodynamic, and laboratory parameters of these patients since the onset of PLE. Extracardiac total cavopulmonary connection was a common type of Fontan modification in 20 of 30 patients, and 60% had a single left ventricle. Patents who eventually developed PLE had significantly prolonged ICU and hospital lengths of stay, however ventricular morphology was unrelated to disease manifestation.   Around 80% of patients had cardiopulmonary perturbations that were heterogeneous, ranging from diaphragm paralysis, obstruction of Fontan pathway, bradyarrhythmia, and central PA stenosis. Significant risk factors associated with PLE were phrenic nerve palsy, bradyarrhythmia, and Fontan pathway obstruction.   The treatment strategies for PLE were analyzed in detail and involved surgical and catheter-based interventions for those indicated. Medical therapy was targeted towards pulmonary vasodilation, anti-inflammatory therapy with budesonide, diuretic, and heart failure therapy. Surgical indications were discrete such as phrenic nerve palsy and PM placement for bradyarrhythmias. A total of 64 percutaneous catheter-based procedures were performed in 25 patients directed towards relieving Fontan pathway obstruction.   Overall, 15 patients were in remission at the time of study with surgical or interventional or medical or combination of therapies.   During the follow up there was a progressive decline in ventricular function, although the degree of Av valve regurgitation did not change. One-third of the patients died after a median interval of 7.2 years, with an estimated ten-year survival of 70.5% and 20-year survival of 50.1%. Overall, four patients were listed for transplant with two patients receiving it while two died on the waiting list. Among those transplanted, one died in the postoperative period.   Although the authors evaluated a large cohort of patients with a reasonable long follow up period, the study is limited by being a single institutional experience. It may not apply to other centers with different populations and variations in surgical and postoperative management strategies. The extended study period lends itself to era effect on disease manifestation with improved current era surgical and anesthetic techniques. Significant advances in the understanding of the abdominal lymphatic system and drainage in the setting of central venous congestion have occurred recently and are paramount to the latest interventions of redirecting lymphatic drainage to prevent and treat PLE.            

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Retrospective Analysis of Clinical Genetic Testing in Pediatric Primary Dilated Cardiomyopathy: Testing Outcomes and the Effects of Variant Reclassification

Retrospective Analysis of Clinical Genetic Testing in Pediatric Primary Dilated Cardiomyopathy: Testing Outcomes and the Effects of Variant Reclassification.   Quiat D, Witkowski L, Zouk H, Daly KP, Roberts AE.J Am Heart Assoc. 2020 Jun 2;9(11):e016195. doi: 10.1161/JAHA.120.016195. Epub 2020 May 27.PMID: 32458740   Take Home Points:   Clinical genetic testing identifies about ⅓ of patients with a primary dilated cardiomyopathy (DCM) with many variants of unknown significance (VUS). Reevaluation/reclassification of prior genetic testing decreased the number of VUS but a large number still remained. Familial genetic testing may help with interpretation of the results.     Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: Only ~ 30-50% of DCM cases have an identifiable etiology, with neuromuscular and myocarditis being the most common. With the improvement in genetic testing, many of the “idiopathic” cases have shown pathogenic variants. However, the amount of VUS has been quite high, and in some regards, this could be considered almost as a “worst case scenario” since it remains unclear if this is the pathogenic etiology or a benign variant. This can markedly affect future family planning or determine prognosis in genotypic carriers. In 2015, the American College of Medical Genetics and Genomics established a 5-tier classification system for VUS (pathogenic, likely pathogenic, uncertain significance, likely benign, benign). This, along with an increase in testing panel content and availability of allele databases, people with a VUS on testing in the past may be able to be reclassified, allowing for improved diagnosis, prognosis, and family counseling.   In this paper, a cohort of 63 patients over a 10 year period (2008-2018) with DCM was evaluated. 18% had a family history of cardiomyopathy or sudden death. 30% had a disease causing variant identified with nearly half occurring de novo. 116 variants were found on initial gene testing, 8 classified as pathogenic, 11 likely pathogenic, 90 VUS, 3 likely benign, and 2 benign (and an additional 2 as unclassified). Reclassification was performed which resulted in the downgrading of 29% (26/90) of VUS to either likely benign or benign and an absolute decrease in the number of BUS from 60% to 52% and an increase in likely benign/benign from 14% to 24%. See table 2. In those with a positive family history (9), 3 variants were inherited and 6 were de novo mutations. In 6 patients with potentially disease causing VUS, familial testing showed 3 occurring de novo and 3 inherited. Interestingly though, they did not find a difference in rates of positive cardiomyopathy genetic testing in patients with (27%) or without (33%) a family history cardiomyopathy or sudden death. With regards to testing, larger gene panels (> 50) found 27% with pathogenic variants vs. 20% in smaller panels (< 50). Of their cohort, 29/63 (46%) underwent heart transplantation or died during the follow up period.   This study shows that periodic reclassification and familial cascade genetic testing can reduce the number of VUS to allow for better diagnosis and future screening for DCM. Unfortunately, the percentage of VUS remains high, so hopefully with further reevaluation as larger panels and other advances are made (or use of whole genome testing is done), this can be improved. Since there are no clear guidelines for how to undergo this process, the authors recommend a review of genetic testing any time there is a VUS, more than a year has passed, and the parents are planning to have more children.          

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Pediatric Cardiology Specialist’s Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease

Pediatric Cardiology Specialist's Opinions Toward the Acceptability of Comfort Care for Congenital Heart Disease. Swanson TM, Patel A, Baxter AJ, Morris SA, Maskatia SA, Lantos JD. Pediatr Cardiol. 2020 May 18. doi: 10.1007/s00246-020-02367-2. Online ahead of print. PMID: 32419096   Take Home Points: There is consensus amongst pediatric cardiologists and CV surgeons to seek legal action and mandate surgery for low surgical mortality risk lesions. There was less likelihood of seeking legal action for Shone complex, single ventricle lesions or CHD associated with trisomy 13 or 18.     Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: As survival has improved dramatically for CHD over the past 40 years, decisions regarding the acceptability of comfort care would seemingly change. Balancing short term and long term mortality data along with morbidities, whether cardiac or neurodevelopmental, is necessary for physicians to help counsel families appropriately. Given the complexity of CHD, it can be difficult for families to truly understand the risks, and defaulting to parental decision-making may not be the most ethical course of action. This paper describes an interesting and basic survey of pediatric cardiologists and CV surgeons at 4 sites to determine the acceptability of overriding parental refusal of surgery for various forms of isolated CHD or associated with various genetic syndromes. 4 choices were provided: (1) agree and support parents who choose not to pursue surgical intervention; (2) disagree but support parental decision; (3) disagree, would not support parental decision, but would not interfere with the decision; and (4) disagree and would seek legal action to pursue surgery. Opinions were cross referenced with STS data regarding surgical mortality for each lesion.   135 physicians were surveyed and there were no statistically significant differences between site location. Table 2 shows physician choice for each lesion. Table 3 shows the percentage of physicians who would seek legal action along with published mortality rates. Figure 1 shows a regression line to demonstrate the strength of correlation between surgical mortality and willingness to pursue legal action. For mortality less than 4.5%, most physicians would seek legal action. Associated genetic syndrome, especially trisomy 13 or 18 strongly decreased the likelihood of recommending surgery.   There are a few interesting points brought in the discussion. First, STS data alone is not always enough to make a decision regarding care. Especially for a single ventricle lesion, this does not capture survival to the following stages of palliation or include life expectancy and morbidity, which can strongly influence decision making. Additionally, ductal dependent lesions, in which non-intervention would lead to “quick” demise may be different than those that could lead to a shorter life expectancy but have profound and progressive future cardiac symptoms and medical problems. Additionally, genetic syndromes influence opinion, and interestingly, the presence of Down syndrome with an AVSD resulted in a lower number of physicians who would seek legal action, even though published mortality rates are lower than those without Down syndrome. The authors also conject that lesions such as Shone syndrome that have uncertainty with future outcomes could influence physician opinion. Fetal counseling or discussion regarding termination was not addressed in this survey, and could be confounded by politics or religious beliefs.          

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The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis

The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis. Kelly P, Hua N, Madriago EJ, Holmes KW, Shaughnessy R, Ronai C. Pediatr Cardiol. 2020 Jan;41(1):62-68. doi: 10.1007/s00246-019-02222-z. Epub 2019 Oct 31. PMID: 31673735 Similar articles Select item 31654097   Take Home Points: A single positive blood culture without other major or minor Modified Duke’s Criteria (MDC) and no prior history of congenital heart disease has a positive predictive value of 0 for infectious endocarditis (IE) Two positive cultures without other criteria also has a very low PPV MDC should be used to assess the clinical probability of IE; if low, a TTE is not recommended due to poor diagnostic yield Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  While sonographic findings are part of the major criteria for MDC, they are usually meant to supplement clinical judgment when there is a higher pretest probability of IE. The sensitivity and specificity in adults is not great for IE, and the ACC/AHA calls TTE an inadequate screening tool. This study was a retrospective chart review to determine the diagnostic yield of TTE in children with suspected IE and structurally normal hearts and no prior history of IE. 300 patients were included. Charts were reviewed to determine whether patients met any MDC prior to the echocardiogram. Clinical IE was determined if patients were treated with 4-6 weeks of IV antibiotics. Positive TTE findings were defined as mass, abscess, thrombus or new significant valvular regurgitation. Over the 10 year study period, 10/300 (3.3%) had positive TTE findings. Of those 10 patients, 8 were treated for IE with 2 false positives that the authors detail. Of the 290 with negative TTE, 3 were diagnosed with IE. These were all teenagers with poorer echo images and clinical features strongly suggestive of IE. See tables 3 and 4.  98 patients (33%) had 2 positive blood cultures; 7 were diagnosed with IE, with one likely having a false positive TTE and the other 6 having some MDC. The PPV of 2 positive cultures and no MDC risk factors was 0.071. 46 patients (15.3%) had only 1 positive blood culture. The PPV of 1 positive culture and no additional MDC risk factors was 0. The PPV of those that met MDC prior to TTE was 0.86. See table 5. There was pretty clear data that those with 1 or 2 positive blood cultures and no other MDC risk factors have a very low likelihood of IE and a positive echo. Misdiagnosis of IE obviously has increased morbidity (need for CVL and antibiotics) and TTE can have false positives. Therefore, use of MDC should be strongly recommended prior to obtaining a TTE. A few caveats/limitations include poor documentation of various vascular and immunologic findings in their chart review. Additionally, while presence of CHD is a risk factor, this study does not address the risk/PPV in this population.  

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New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders

New Screening Tool for Aortic Root Dilation in Children with Marfan Syndrome and Marfan-Like Disorders. Wozniak-Mielczarek L, Sabiniewicz R, Nowak R, Gilis-Malinowska N, Osowicka M, Mielczarek M. Pediatr Cardiol. 2020 Jan 31. doi: 10.1007/s00246-020-02307-0. [Epub ahead of print] PMID: 32006082 Similar articles Select item 20301322   Take Home Points: Aortic root ratio may be another helpful screening measurement for aortic root dilation in patients with suspected connective tissue disorders This study did not assess normal patients or patients with other etiologies for aortic root dilation (such as BAV) Whether cumulative changes such as rate of change or significant change would improve morbidity/mortality or be useful for surgical intervention remains to be seen Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  Similar to nearly all echo measurements in children, the aortic diameter is affected by a patient’s age, sex, and BSA. Z-scores are used to allow for the necessary adjustments in order to determine if something is within the normal range. There are currently 3 main z-score nomograms used. Based on a premise that z-score calculations are time consuming and impractical, this study aimed to introduce a simple screening method to identify aortic root dilation. The authors retrospectively analyzed 193 patients with Marfan Syndrome (MFS) or Marfan-like disorders (including Ehlers-Danlos, Loeys-Dietz, ectopia lentis, neonatal Marfan, MASS phenotype, and Marfanoid habitus). Marfanoid habitus was defined as a constellation of symptoms similar to MFS but did not fully meet Ghent criteria. The mean age was 12 years. The aortic root (sinuses of Valsalva only) was measured in the parasternal long axis view using both the leading edge in end-diastole and the inner edge in mid-systole. The aortic root ratio (ARr) was calculated as the aortic root diameter (mm) divided by the height (cm) multiplied by 100. This was compared to the 2 measurement techniques and z-scores using all 3 nomograms.   28-31 % of the patients had aortic root dilation as expressed by z-score (z > 2) depending on which nomogram was used. 11 patients (5.79%) had inconsistent results (dilated vs. non-dilated) when comparing z-score nomograms (Gautier vs. Cantinotti only). The mean value for ARr was 18.1 vs. 17.8 for the leading edge vs. inner edge measurements. The optimal cut-off for ARr was > 18.7 with resulted in a sensitivity of 88-100% and specificity of 94-98% (Table 7). The authors further assessed the subgroup in whom the ARr failed to identify aortic root dilation. They found the results were inconsistent using the 3 different nomograms. Notably, a detailed table shows that all of the z-scores of this group were either just below or just above 2. ROC curves showed an ARr > 18 yielded a sensitivity of 100% for the Petterson and Cantinotti nomograms and > 18.7 for the Gautier nomogram. Tables 10 and 11 show the high values of PPV, NPV and accuracy of ARr using the various cut-offs. The authors make a reasonable argument that extremely thin or obese patients may have inaccurate z-scores, and based on previous data that height is more important than BSA for aortic root diameter, the ARr may be useful. There are however a few major caveats to this study. First, ARr only assess the aortic root at the sinuses. It will therefore not be useful in those with significant annular dilation or ascending aorta dilation, and we know that surgical decision making often entails valve sparing vs. non-sparing aortic root replacement. Additionally, the Marfan-like subgroup was large and may not represent a normal population for which this measurement has not been validated. They also did not include a BAV/aortic root dilation group, or other groups with CHD such as TOF or d-TGA s/p ASO, which may have a different form of aortic root dilation than the genetic connective tissue disorder group. Finally, the claim that nomograms are difficult to use may be a bit spurious, given that many reporting software and measurement packages on the echo equipment may automatically calculate z-scores. It is interesting to note that there may be variation depending on the nomogram used, so echo readers should keep this in mind based on the specific software algorithms used. However, most of these patients would have routine follow up, so a “missed” diagnosis when the z-score is 1.9 is probably not too clinically relevant. Since this was a one-time measurement study, further research on the ARr over time would be necessary, since rate of change is also a key factor in decision making.  

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Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography

Imaging of the pulmonary vasculature in congenital heart disease without gadolinium contrast: Intraindividual comparison of a novel Compressed SENSE accelerated 3D modified REACT with 4D contrast-enhanced magnetic resonance angiography. Pennig L, Wagner A, Weiss K, Lennartz S, Grunz JP, Maintz D, Laukamp KR, Hickethier T, Naehle CP, Bunck AC, Doerner J. J Cardiovasc Magn Reson. 2020 Jan 23;22(1):8. doi: 10.1186/s12968-019-0591-y. PMID: 31969137 Free PMC Article Similar articles Select item 31974688   Take Home Points: A novel 3D non-contrast-enhanced magnetic resonance angiography (modified REACT-non-CE-MRA) is a good alternative for the visualization of the pulmonary arteries and pulmonary veins in congenital heart disease patients. Compared to 4D contrast-enhanced magnetic resonance angiography, modified REACT-non-CE-MRA offers a better image quality. Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: In this cardiovascular magnetic resonance study the authors applied a new 3D Relaxation-Enhanced Angiography without Contrast and Triggering (modified REACT-non-CE-MRA) to visualize the pulmonary arteries in congenital heart disease (CHD) patients and compared the novel technique with the standard non-ECG-triggered time-resolved 4D contrast-enhanced magnetic resonance angiography (4D CE-MRA). 25 patients with known or suspected CHD were included and images were analysed independently by two radiologists. Measurements were taken at seven distinct measurement points, namely main pulmonary artery, left and right pulmonary artery, right superior and inferior pulmonary vein as well as left superior and inferior pulmonary vein. Furthermore, image quality was assessed using a four-point scale including the parameters sharpness, presence of pulsation artifacts, and anatomic delineation. Out of 25 patients, 23 had a CHD including atrial septal and ventricular septal defect, transposition of the great arteries, tetralogy of Fallot and pulmonary atresia. Regarding the interobserver agreement, the modified REACT-non-CE-MRA had a higher agreement for the pulmonary veins compared to the 4D CE-MRA. For the pulmonary arteries interobserver agreement was comparable between the modified REACT-non-CE-MRA and the 4D CE-MRA. 4D CE-MRA showed larger diameters for all measurement points and this was demonstrated to be significant for the pulmonary arteries but not the pulmonary veins. 4D CE-MRA image quality scores wore lower compared to the scores for the modified REACT-non-CE-MRA (Figures 3 and 5). This study has nicely demonstrated that a novel non-contrast MRA technique, the so-called modified REACT-non-CE-MRA, has the potential to be a good alternative to CE-MRA techniques in CHD patients.

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Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents

Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents. Rathgeber SL, Guttman OR, Lee AF, Voss C, Hemphill NM, Schreiber RA, Harris KC. J Am Heart Assoc. 2020 Jan 7;9(1):e012529. doi: 10.1161/JAHA.119.012529. Epub 2020 Jan 4. PMID: 31902322 Free PMC Article Similar articles Select item 31852418 Select item 31130285   Take Home Points: Fontan-associated liver disease begins in childhood. Liver stiffness measured by transient elastography is associated with splenomegaly and time since Fontan operation. Comment from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch: Fontan-associated liver disease is an increasingly important complication and already starts in childhood. In this study the authors evaluated 76 pediatric Fontan patients with a median age of 11.7 years. In all patients an echocardiogram, laboratory studies, transient elastography and an abdominal ultrasound were performed. Furthermore, the aspartate transaminase (AST) to platelet ratio index (APRI) was calculated. 17 patients underwent additional liver biopsy. The authors found a significant correlation between the time since Fontan operation as well as splenomegaly and liver stiffness measured by transient elastography (see figure). In addition, the presence of splenomegaly was found to be associated with low platelets, AST and alkaline phosphatase. Liver biopsy did not demonstrate severe changes in this relatively young cohort, but mild-to-moderate histopathologic liver changes were found in all patients. There was no significant difference regarding liver stiffness between patients with a dominant left and right ventricle. The results are very interesting, but the study also clearly demonstrates that further research in Fontan-associated liver disease is needed.  

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Hepatic magnetic resonance T1-mapping and extracellular volume fraction compared to shear-wave elastography in pediatric Fontan-associated liver disease

Safety and efficacy of anticoagulant therapy in pediatric catheter-related venous thrombosis (EINSTEIN-Jr CVC-VTE). Thom K, Lensing AWA, Nurmeev I, Bajolle F, Bonnet D, Kenet G, Massicotte MP, Karakas Z, Palumbo JS, Saracco P, Amedro P, Chain J, Chan AK, Ikeyama T, Lam JCM, Gauger C, Pap ÁF, Majumder M, Kubitza D, Smith WT, Berkowitz SD, Prins MH, Monagle P, Young G, Male C. Blood Adv. 2020 Oct 13;4(19):4632-4639. doi: 10.1182/bloodadvances.2020002637   Take Home Points: Both rivaroxaban and standard anticoagulants (heparin and vitamin K antagonists) seem to be a safe treatment for children with central venous catheter related venous thromboembolism (CVC-VTE). Persistent need of the CVC and residual venous thromboembolism in children younger than 2 years were associated with continuation of anticoagulant therapy beyond the study period. Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric/Fetal Cardiology Journal Watch: The EINSTEIN-Jr study compared rivaroxaban to standard anticoagulants in 500 children of all ages for treatment of acute venous thromboembolism (VTE) of any type. Pediatric patients were randomized in a 2:1 ratio to rivaroxaban or comparator (heparin or vitamin K antagonist). In this subanalysis of the EINSTEIN-Jr study the authors analyzed the safety of anticoagulation and the clinical risk profile and characteristics of children with central venous catheter (CVC) related thromboembolism (CVC-VTE). They also evaluated if the risk factor profile affects the duration of anticoagulant treatment. Children with CVC-VTE were included if they were treated with unfractionated heparin, low-molecular weight heparin or fondaparinux. CVC-related, non–CVC-related recurrent deep VTE or other venous thrombosis as well as major or clinically relevant non-major bleeding were documented. In patients with no symptomatic recurrent VTE repeated imaging was performed and compared to baseline to assess vein recanalization. Overall, 126 patients with CVC-VTE were included (Figure 1). 90 patients received rivaroxaban and 36 were treated with standard anticoagulants. 76 patients were symptomatic, and 50 patients had asymptomatic CVC-VTE. Clinically significant venous thrombosis occurred in 2 children and 3 children on rivaroxaban were diagnosed with clinically relevant non-major bleeding (Table 2). 103 patients received a repeat imaging test. Complete vein recanalization was found in 55%, incomplete recanalization in 37%, no change in 6,8% and asymptomatic deterioration in 1% of the patients. Residual VTE on repeat imaging (limited to children younger than 2 years) and presence of a CVC at the end of the study were associated with continued anticoagulant therapy beyond the study period. Although the sample size was small, the authors were able to demonstrate the safety of rivaroxaban and standard anticoagulant medication in patients with CVC-VTE. However, the authors also mention that larger multicenter studies are needed.

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Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience

Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience. Kaplinski M, Ittenbach RF, Hunt ML, Stephan D, Natarajan SS, Ravishankar C, Giglia TM, Rychik J, Rome JJ, Mahle M, Kennedy AT, Steven JM, Fuller SM, Nicolson SC, Spray TL, Gaynor JW, Mascio CE. J Am Heart Assoc. 2020 Oct 20;9(19):e016889. doi: 10.1161/JAHA.120.016889. Epub 2020 Sep 23. PMID: 32964778 Free article.   Take Home Points: Risk of interstage mortality after the Norwood significantly decreased over the years. Performance of the superior cavo-pulmonary connection (SPVC) at an earlier age, increase in use of a right ventricle to pulmonary artery shunt, and an interstage monitoring program may be the factors that have contributed to this decrease. Further research will be necessary to determine the specific contribution of these interventions as well as the influence of other factors such as prenatal diagnosis, other changes in surgical practice including improved perfusion/bypass techniques, stem cell therapy, or checklists, towards decreasing interstage mortality. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric/Fetal Cardiology Journal Watch: Since the 3-stage approach for HLHS was established, there has been a progressive decline in overall mortality and long-term survival has improved. However, there has been increased focus on the interstage period between the Norwood and SPVC due to a high risk of mortality during that time. There have been multiple studies looking at an interstage monitoring program (ISMP), with somewhat conflicting data on whether this reduces mortality. Additionally, there has been a shift in some centers to use an RV to PA shunt instead of a BTT shunt for the Norwood, as well as an earlier stage 2 operation, in order to attempt to decrease mortality during that interstage period. The objectives of this study were to examine an overall incidence of interstage mortality during a 30-year period, evaluate interstage mortality across 4 predetermined eras based on changes in operative or medical management, and to analyze the impact of patient characteristics and operative factors on interstage mortality. Adjusted and non-adjusted logistic regression models were used to identify the risk factors for mortality. This was a retrospective chart review of 1111 patients that were available for interstage analysis (after excluding those that died or had SCPC before discharge, had biventricular conversion, or heart transplantation) over a 30-year period from 1984 - 2017. 33% had HLHS (MA/AA) and 30% had an HLHS variant. 12% had a suspected or confirmed genetic abnormality. The study period was divided into 4 eras; era 1: 1988-1994 (initial use of SCPC), era 2: 1995-2001 (change in surgical team), era 3: 2002-2010 (introduction of RV-PA shunt), and era 4: 2011-2017 (introduction of ISMP). Overall interstage mortality was 10.8% (120/1111) and decreasing significantly only for the most recent era (4.6%) (see figure 2 and table 2). While age at SCPC decreased over the 4 eras, there was no statistically significant difference for age of death. The authors do note that since the shortest length of time between the Norwood and SCPC was during the same period as the introduction of the ISMP, it was not possible to determine their individual relative contributions. Birth weight, gestational age, length of stay, genetic abnormality, and race were identified as risk factors using single covariate adjusted (era) logistic regression. A multiple logistic regression model indicated that only gestational age and race were associated with interstage mortality after adjusting for era (see table 4). A secondary analysis was conducted to estimate the relationship between shunt type after adjusting for era, gestational age, use of ECMO, and race, with an improvement in survival in those with RV-PA shunts compared to BTT shunts. Notably, the RV-PA shunt was first used in era 3 accounting for 27.4% of shunt type, and this increased to 50% in era 4. The risk of mortality significantly decreased by era 4 but it is unclear how much of each factor influenced that change. Is one more important than another or is there an “exponential” influence when all factors are present? Additionally, and especially in the last 5 years, there have been other changes in surgical practice, including improved bypass/perfusion techniques, stem cell therapy, and checklists post-bypass in some centers. Possibly even more importantly, this study did not discuss prenatal diagnosis as a factor that may also improve interstage (and overall) mortality. Future NPC-QIC prospective data may also delineate other factors that may be critical in improving mortality.    

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Racial Disparities in Hospital Mortality Among Pediatric Cardiomyopathy and Myocarditis Patients

Racial Disparities in Hospital Mortality Among Pediatric Cardiomyopathy and Myocarditis Patients. Olsen J, Tjoeng YL, Friedland-Little J, Chan T.Pediatr Cardiol. 2020 Oct 6. doi: 10.1007/s00246-020-02454-4. Online ahead of print.PMID: 33025028   Take Home Points Racial differences in health care outcomes exist for multiple illnesses in the adult population, this appears to also be true in the pediatric population with the diagnosis of myocarditis and cardiomyopathy. African American race and Hispanic ethnicity were independent risk factors for mortality. African American race was also associated with use of ECMO, mortality while on ECMO, and cardiac arrest. However, when adjusting the model for ECMO and arrest reduced the impact of African American race. Hispanic ethnicity was still associated with mortality even after controlling for variables. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: This was a retrospective cross-sectional study using the Kids’ Inpatient Database (KID). This is a national administrative database produced every 3 years with a random sampling of pediatric patients that have been discharged from a hospital. The goal of this study was to determine if any associations existed between race/ethnicity and hospital outcomes of pediatric patients diagnosed with cardiomyopathy or myocarditis. Patients < 18 years of with an ICD-9 code of cardiomyopathy or myocarditis with no other cardiac diagnosis were identified. Data from 2003, 2006, 2009, and 2009 were used. Race/ethnicity (non-Hispanic white, non-Hispanic African American, Hispanic, and other), demographic data, hospital procedures, and hospital mortality were collected. Associations between ethnicity/race and mortality were evaluated and subsequent models incorporated cardiac arrest, ECMO, VAD, or transplant in the analysis. Total of 34,617 patients were evaluated (white = 38.6%, African American = 20.4%, Hispanic = 15.4%, and other = 7.9%). Patients were mostly > 1 year of age (88.7%), male, 59.9%), had cardiomyopathy (70.8%), and were treated at non-pediatric hospitals (73.7%). Non-white patients were more likely to have the diagnosis of cardiomyopathy, use government insurance, live in lower income neighborhoods, and undergo fewer transplants. Rate of cardiac arrest, ECMO, and VAD did not differ between ethnicities. Overall mortality was 4.6% with white patients having significantly lower rates (4.1%) than African Americans (4.7%), Hispanic (5.4%), and other (5.8%). Other significant variables associated with mortality are presented in Table 3. Initial statistical multivariate model showed increased odds for mortality for African American (OR 1.25 [1.01 – 1.53]) and Hispanic (OR 1.29 [1.03 – 1.60]) patients. When ECMO, VAD, or transplant was included in the model, the mortality associations were no longer present for African Americans, but persisted for Hispanic patients. African American race was associated with use of ECMO (OR 1.46 [1.04 – 2.05]) and cardiac arrest (OR 1.23 [1.02 – 1.48]). African American and “other” race had higher mortality when ECMO was utilized. In patients that did not undergo ECMO, Hispanic race was associated with mortality. Adult studies have shown differences in health care outcomes based on race/ethnicity. This paper also documents decreased overall survival in African American and Hispanic patients compared to white patients with the diagnosis of cardiomyopathy or myocarditis. The reasons for these differences are multifactorial. By controlling for certain variables, the authors suggest that at least hospital selection and referral patterns are not the main reasons for these differences. They suggest pre-hospital and in-hospital factors or possibly intrinsic factors may be the key drivers. Limitations of the study include its retrospective nature. The lack of granular data and incomplete or incorrect data entry in the database are also shortcomings. Despite this, the large descriptive “n” allows for starting point to further evaluate why these differences exist.  

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Early school-age cognitive performance post-pediatric heart transplantation

[et_pb_section fb_built="1" admin_label="section" _builder_version="3.22" custom_padding="||188px|||"][et_pb_row admin_label="row" _builder_version="3.25" background_size="initial" background_position="top_left" background_repeat="repeat" custom_margin="-101px|auto|-208px|auto||"][et_pb_column type="4_4" _builder_version="3.25" custom_padding="|||" custom_padding__hover="|||"][et_pb_text admin_label="Text" _builder_version="3.27.4" background_size="initial" background_position="top_left" background_repeat="repeat" min_height="1797px"]Early school-age cognitive performance post-pediatric heart transplantation Gold A, Bondi BC, Ashkanase J, Dipchand AI.Pediatr Transplant. 2020 Dec;24(8):e13832. doi: 10.1111/petr.13832. Epub 2020 Oct 26.PMID: 33105067   Take Home Points In this small (n= 25), but well controlled study of post-transplant pediatric patients, intellectual, academic, and perceptual-motor domain scores were low average while memory abilities were average. Clinical DSM-5 psychological diagnosis was present in >50% of the patients studied. Presence of a congenital heart disease and/or psychological diagnosis predicted worse neuropsychological domain scores. Close neuropsychological monitoring of these patients are needed to maximize long-term outcomes. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: Previous literature, using heterogeneous methodologies, has documented increased developmental delay, neuropsychological deficits in cognition, and decreased academic achievement in pediatric heart transplant patients with an incidence ranging from 10 – 50%. The goal of this study was to evaluate a non-biased patient sample under uniform conditions and time intervals to better understand the neuropsychological outcomes in these patients. Patients who underwent cardiac transplantation < 2 years of age were recruited. Multiple neuropsychological assessment tools (Table 1) were performed till entry into grade school. Scores were designated average (90 - 109), low average (80 – 89), well below average (70 – 79), and significantly poorer than age expectations (< 70). The study was performed from January 2014 to October 2018. Patients were excluded if they had been assessed before or could not follow up for testing. Baseline demographics as well as clinical data were recorded. Thirty-one patients underwent transplantation during the study period and 25 qualified for the study (18 females, 7 males). Median age at transplantation was 0.71 years and age at testing was 6.7 years. Fourteen patients had CHD and 11 had cardiomyopathy. 72% had neurological issues and 68% had sensory issues pre- or post-transplant. General intellect, academic, and perceptual motor indices were in the low normal range, whereas memory indices were in the normal range (Figure 1 – 3). Fourteen (56%) patients met DSM-5 criteria for a clinical diagnosis (intellectual disability mild 20%, learning disability 20%, language disorder 8%, and ADHD 12%). Another 8 (32%) patients were deemed at risk for developing issues. There was a negative correlation between prior neurological issues (stroke, seizure, microcephaly, or other) and full-scale intelligent quotient, verbal comprehension, working memory, visual learning, verbal story learning, and short delay verbal story memory. There was a negative correlation with CHD and full-scale intelligent quotient, working memory, and fluid reasoning. This study reinforces the need to closely follow up these patients from a neuropsychological standpoint so that early intervention can be performed to maximize long-term outcomes. Though not surprising, it also reinforces that patients with a congenital heart disease diagnosis or prior neurological issues are at increased risk for poorer neuropsychological outcomes. Though the study was small, from a single institution, and had a limited follow up time period, it has the benefits of being complete in its assessment of the patient population.           [/et_pb_text][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default" custom_margin="83px|auto||auto||"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture1.png" title_text="Picture1" _builder_version="4.7.4" _module_preset="default" min_height="729px"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture2.png" title_text="Picture2" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture3.png" title_text="Picture3" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture4.png" title_text="Picture4" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture5.png" title_text="Picture5" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][/et_pb_section]

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Kawasaki Disease and Clinical Outcome Disparities Among Black Children

Kawasaki Disease and Clinical Outcome Disparities Among Black Children View Article Luz A Padilla 1, Jacqueline L Collins 2, Adeniyi J Idigo 3, Yung Lau 2, Michael A Portman 4, Sadeep Shrestha 3 J Pediatr. 2020 Sep 24;S0022-3476(20)31244-0.   doi: 10.1016/j.jpeds.2020.09.052. Online ahead of print.  PMID: 32980379.  PMCID: PMC7513890. DOI: 10.1016/j.jpeds.2020.09.052 Take-Home Points : Black children with Kawasaki disease have a higher prevalence of Intravenous Immunoglobulin therapy refractoriness when compared to white children. Black children present with increased inflammatory markers but lower albumin, sodium when compared to white children even though no difference exists in time from fever to admission in between both the groups. Black children receive more ancillary drugs and stay longer in the hospital when compared to white children with Kawasaki disease and have higher persistence of coronary abnormalities on follow up echocardiograms. The disparate outcomes seem to be more related to biologic/genetic variation than health care access and delivery in this cohort of patients admitted to a tertiary level children’s hospital in the South Eastern United States. Commentary from Dr. Venu Amula (Salt Lake City, USA), section editor of Pediatric & Fetal Cardiology Journal Watch:  Race and ethnic variation in the incidence and outcomes of Kawasaki disease have been well reported worldwide. Though racial differences exist in health care access and delivery, it is unknown whether the Black race poses a significant risk for poor outcomes in Kawasaki Disease. Data show that Black children may be disproportionately affected by the Kawasaki disease and manifest altered response to treatment. The authors conducted a retrospective observational cohort study of Black and White children who met the American Heart Association Criteria for Kawasaki Disease and admitted to a tertiary level children’s hospital between January 2000 to 2015. The hospital serves proportionate Black and White populations and gave the investigators a unique opportunity to evaluate any differences in Kawasaki disease characteristics and clinical outcomes between the two communities. The groups were classified by race as assigned by the parent report.  Children who failed to meet AHA criteria, presented 36 hrs. after Intravenous Immunoglobulin treatment rendered at an outside hospital, and those with inadequate documentation at admission were excluded. Patients were identified using ICD codes from the hospital database. The baseline characteristics, hospital presentation, treatment, and echocardiography findings during Kawasaki disease hospitalization were compared between the two racial groups. The study resulted in a final cohort of 369 patients, comprising 192 Whites and 177 Blacks with no significant differences in age at admission or sex between the two racial groups. Mean hemoglobin and hematocrit levels were significantly lower in Black children at admission. Inflammatory markers at admission were higher, particularly C-reactive protein (CRP) and erythrocyte sedimentation rate. There was no difference in time to treatment (initiation of IVIG infusion) or healthcare delivery in both racial groups.  Ninety-four percent of the Black children and 89.7% of the White children received IVIG within ten days of fever onset, per the AHA guidelines. Among those treated, Black children had a lower IVIG response rate compared with whites (86.6% vs. 95.6%; P = .007). IVIG refractoriness was defined as persistent or recurrent fever within 36 hrs. of completing the treatment. More Black children than White children received alternative therapies (9.6% vs. 2.6%; P = .003. And also had a longer average length of hospital stay.  Black children are also noted to have a higher proportion of persistent coronary abnormalities upon follow-up echocardiograms. This critical study evaluates whether disparate outcomes exist in black children with Kawasaki disease than white children in a tertiary level children’s hospital.  Even though racial differences exist in response to the disease and IVIG therapy refractoriness, there is no difference in time to intervention suggesting uniform health care provision. The outcomes and sequelae of Kawasaki disease may be related to underlying biological and genetic variation. This study is limited by being a single-center study with a high likelihood of selection bias. Nevertheless, genetic differences in response to treatment cannot be underestimated.  

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Ascending Aortoplasty in Pediatric Patients Undergoing Aortic Valve Procedures.

[et_pb_section][et_pb_row][et_pb_column type="4_4"][et_pb_text] Ascending Aortoplasty in Pediatric Patients Undergoing Aortic Valve Procedures. View Article Tan CW, Marathe SP, Kwon MH, Chavez M, Friedman KG, Staffa S, Del Nido P, Baird CW. Ann Thorac Surg. 2020 Sep 15:S0003-4975(20)31477-6. doi: 10.1016/j.athoracsur.2020.06.115. Online ahead of print. PMID: 32946842 Take Home Points: Ascending aortoplasty at the time of aortic valve surgery is safe and effective in reducing ascending aortic dimensions and recurrent aortic regurgitation in the short/intermediate term. Longer term follow up will be necessary to determine continued rate of growth. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch:  It is well known that hemodynamic and genetic factors can result in aortic root and ascending aorta dilation in patients with aortic valve disease. There are relatively clear guidelines in the adult population for when to intervene on the aorta during or independent of aortic valve surgery. However, the morbidity and mortality, the risk of dissection, and the impact on progressive aortic regurgitation in pediatric patients with ascending aorta dilation based on z-score is not well known. This results in a lack of guidelines within the pediatric population for intervention. Reduction aortoplasty can immediately reduce the size of the aorta, but it is unclear if this will prevent future growth and decrease risk of dissection/rupture or prevent future aortic regurgitation. The current general trend has been to accept higher aortic dimensions, but this may be result in the significant mortality. This was a retrospective study of patients between 2010-2018 who had an ascending aorta z-score of > +2 at the time of aortic valve surgery. Exclusions included patients with prior ascending aorta repair, single ventricle pathology, s/p ASO, heart transplant or known connective tissue disorder. 47 patients underwent aortoplasty with 39 having complete data points. They were compared with 39 matched controls. Echocardiograms pre-operatively, immediate post-operatively, and at latest follow up were reviewed for lateral ascending aorta dimensions in the PLAX and z-scores obtained based on BSA. The surgical technique was described in the paper and the goal was to reduce the ascending aorta size to a z-score between 0 and +2 or a decrease of at least 2 z-scores. A total of 39 subjects with a median age of 11 years and weight of ~41 kg were compared to 39 controls, with only BSA being dissimilar. No patients required a 2nd cross clamp due to inappropriate reduction of the aorta. Ozaki type reconstruction of the aortic valve was more common in the aortoplasty group. Table 2 shows the operative and post-operative details of each group. Within the study group, pre-operative mean ascending aorta z-score was 5.35+/-1.52 and reduced to 1.22+/-1.63 post-operatively. 29/47 patients had a z-score < +2. For the 39 patients with all-time points, median follow up was 12.5 months and ascending aorta z-score remained similar at 1.37+/-1.72 (Figure 2). Of the 12 patients with a post-operative z-score > +2, all remained with unchanged z-score (average change 0.14) at latest follow up. No differences in z-scores could be identified by aortic valve morphology, pathology or intervention, patient weight or BSA. In the control group, median follow up was 40.8 months. Pre-operative mean ascending z-score was 4.15+/-1.65 and was reduced to 3.26+/-2 at latest follow up that was deemed to be from greater somatic growth than aortic growth over time, resulting in a lower z-score. While the control group also had a significant reduction in average z-score, the reduction was significantly larger in the aortoplasty group (see Figure 3). The control group had 6.84 times the odds of moderate or greater aortic regurgitation (see Figure 4), and this was confirmed to not be related to type of aortic valve repair on secondary analysis. No patients in the aortoplasty group had any early post-operative complications. The authors note that rapid aneurysmal growth did not occur after aortoplasty. Additionally, there may be even greater benefit in those patients with residual aortic valve disease and persistent hemodynamic effect on the ascending aorta. Limitations of this study include the small sample size, retrospective design, and relatively short follow up duration. Additionally, it would have been nice if the authors would have included aortic root z-scores (sinuses and STJ) as well as the ascending aorta, and it is unclear why they were not. Hopefully, there will be longer term studies (greater than 10 years) and possibly including other imaging modalities (CT/MRI) looking at the absolute aortic dimensions and rate of change after aortoplasty. However, given the likely relatively low short and intermediate risk, as well as what seems to be a quite significant decrease in risk of progressive aortic regurgitation, it may be reasonable to consider aortoplasty in more patients.   [/et_pb_text][/et_pb_column][/et_pb_row][/et_pb_section]

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Genomic analyses implicate noncoding de novo variants in congenital heart disease

Genomic analyses implicate noncoding de novo variants in congenital heart disease View Article Richter F, Morton SU, Kim SW, Kitaygorodsky A, Wasson LK, Chen KM, Zhou J, Qi H, Patel N, DePalma SR, Parfenov M, Homsy J, Gorham JM, Manheimer KB, Velinder M, Farrell A, Marth G, Schadt EE, Kaltman JR, Newburger JW, Giardini A, Goldmuntz E, Brueckner M, Kim R, Porter GA Jr, Bernstein D, Chung WK, Srivastava D, Tristani-Firouzi M, Troyanskaya OG, Dickel DE, Shen Y, Seidman JG, Seidman CE, Gelb BD. Nat Genet. 2020 Aug;52(8):769-777. doi: 10.1038/s41588-020-0652-z. Epub 2020 Jun 29. PMID: 32601476 Take Home Points: A genetic etiology is identified for one-third of patients with congenital heart disease (CHD), with 8% of cases attributable to coding de novo variants (DNVs). In this National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Cardiac Genomics Consortium (PCGC) study of >13,000 patients with CHD utilizing whole-exome sequencing (WES) and chromosome microarrays rare, transmitted variants and DNVs in 8% of patients with sporadic CHD. CHD probands and their parents when compared to unaffected trios demonstrated a higher burden of DNVs in individuals with CHD. DNV burden was also observed in RNA-binding-protein regulatory sites. Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   In this landmark study National Heart, Lung, and Blood Institute (NHLBI)-funded Pediatric Cardiac Genomics Consortium (PCGC) recruited >13,000 patients and utilized whole-exome sequencing (WES) and chromosome microarrays to study CHD genetic architecture. The analyses identified damaging rare, transmitted variants and DNVs in 8% of patients with sporadic CHD (including 28% of syndromic and 3% of isolated CHD). Independent analyses of enhancers showed an excess of DNVs in associated genes (27 genes versus 3.7 expected, P = 1 × 10−5). CHD DNVs altered transcription levels in 5 of 31 enhancers assayed. The study also demonstrated DNV burden in RNA-binding-protein regulatory sites (OR = 1.13, 95% CI 1.1–1.2, P = 8.8 × 10−5).

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Protein-losing enteropathy and plastic bronchitis after the Fontan procedure

Protein-losing enteropathy and plastic bronchitis after the Fontan procedure View Article Varun J Sharma 1, Ajay J Iyengar 2, Diana Zannino 3, Thomas Gentles 4, Robert Justo 5, David S Celermajer 6, Andrew Bullock 6, David Winlaw 7, Gavin Wheaton 8, Luke Burchill 9, Rachael Cordina 10, Yves d'Udekem 11 J Thorac Cardiovasc Surg. 2020 Aug 12;S0022-5223(20)32359-X. doi: 10.1016/j.jtcvs.2020.07.107. Online ahead of print. PMID: 32928546;  DOI: 10.1016/j.jtcvs.2020.07.107 Take Home Points: Median time of onset of protein-losing enteropathy after Fontan is 5 years. Prevalence of protein-losing enteropathy and plastic bronchitis 30 years following after Fontan is 5%. Independent predictors for the risk of developing protein-losing enteropathy and plastic bronchitis were right ventricular morphology with HLHS, older age at Fontan, and prolonged pleural effusions after Fontan. Left ventricular morphology was protective. Freedom from death/transplantation after diagnosis of protein-losing enteropathy or plastic bronchitis at 5 and 10 years were 91% and 77% respectively. Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   This is a retrospective analysis of 1561 patient from the Australia and New Zealand Fontan registry. A total of 55 patients with protein losing enteropathy/plastic bronchitis were studied. Their median age at the Fontan was 5.7 years, and time to onset after the Fontan for protein-losing enteropathy was 5.0 years and plastic bronchitis was 1.7 years. Independent predictors for developing protein-losing enteropathy/plastic bronchitis were right-ventricular morphology with hypoplastic left-heart syndrome (hazard ratio, 2.30; confidence interval, 1.12-4.74), older age at Fontan (hazard ratio, 1.13; confidence interval, 1.03-1.23), and pleural effusions after Fontan (hazard ratio, 2.43; confidence interval, 1.09-5.41); left-ventricular morphology was protective (hazard ratio, 0.36; confidence interval, 0.18-0.70). In the protein-losing enteropathy/plastic bronchitis population, freedom from death or transplantation after protein-losing enteropathy/plastic bronchitis diagnosis at 5, 10, and 15 years was 70% (confidence interval, 58-85), 65% (confidence interval, 51-83), and 43% (confidence interval, 26-73), respectively; only older age (hazard ratio, 1.23; confidence interval, 1.01-1.52) was an independent predictor.  Protein-losing enteropathy and plastic bronchitis remain severe complications of Fontan circulation specially in patients with a dominant right ventricle.  No significant improvement in transplant-free survival was noted in the current era. A quarter of patients require heart transplantation within a few years of diagnosis.

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First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice

First-Degree Relatives Screening of Patients with Bicuspid Aortic Valve: Effectiveness and Feasibility in Pediatric Cardiology Daily Practice View Article Massardier C, Desroches F, Singbo N, Côté JM, Drolet C, Houde C, Vaujois L, Chetaille P. Pediatr Cardiol. 2020 Aug 26. doi: 10.1007/s00246-020-02423-x. Online ahead of print. PMID: 32851436 Take Home Points: Bicuspid aortic valve is the most common congenital heart disease with the prevalence of 1 to 2% in the general population and is now considered heritable. Echocardiographic screening of first-degree relatives of patients with bicuspid aortic valve is recommended. An autosomal dominant pattern of inheritance with incomplete penetrance is suspected. This study demonstrated a prevalence of 6% of bicuspid aortic valve in first-degree relatives (FDR). Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   In this retrospective single center study from Canada, a total of 713 first-degree relatives of 213 consecutive index cases with the median age of 11 years were studied.  Bicuspid aortic valve (BAV) was found in 6.6% of first-degree relatives and 5.4% had aortic valve dysfunction.  A total of 2.9% subjects had ascending aorta dilation.  One third of first-degree relatives didn’t perform the screening. Screening was done in 482 (67.6%), prescribed but not done in 134 (19%), not prescribed in 92 (13%) and declined in 5 (1%) FDR. BAV was more frequent in men (68.5%) and most of cases had BAV with raphe (87.2%), largely represented by BAV type L-R (57.8%). By univariate analysis, no significant associations were found between screening positivity in first degree relatives and aortic valve morphology, aortic valve dysfunction or ascending aorta dilatation severity in index cases. The prevalence of BAV in first-degree relative was similar to prospective adult studies and supports actual guidelines in pediatric cardiology practice. Ascending aorta dilatation was rare in our young population. Exhaustiveness and additional burden to implement current guidelines remain a challenge in daily practice.

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The Genetic Epidemiology of Pediatric Pulmonary Arterial Hypertension

The Genetic Epidemiology of Pediatric Pulmonary Arterial Hypertension. View Article Haarman MG, Kerstjens-Frederikse WS, Vissia-Kazemier TR, Breeman KTN, Timens W, Vos YJ, Roofthooft MTR, Hillege HL, Berger RMF. J Pediatr. 2020 Jun 2:S0022-3476(20)30689-2. doi: 10.1016/j.jpeds.2020.05.051. Online ahead of print. PMID: 32502478 Take Home Points: Prevalence of pulmonary arterial hypertension (PAH)-associated gene disorders and other genetic disorder was high in pediatric patients with PAH. In this study, 27% had a PAH-associated gene mutation/variant, 17% had a genetic disorder with an established association with PAH and in 23% genetic disorders without an established association with PAH were identified. Underlying genetic mutation impact survival and can be useful in risk stratification. Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   This study describes the prevalence of pulmonary arterial hypertension (PAH)-associated gene mutations, and other genetic characteristics in a national cohort of children with PAH from the Dutch National registry. The study also explores genotype-phenotype associations and outcomes. Of the 70 subjects in the study 19 (27%) had a PAH-associated gene mutation/variant: BMPR2 n = 7, TBX4 n = 8, ACVRL1 n = 1, KCNK3 n = 1, and EIF2AK4 n = 2. Twelve children (17%) had a genetic disorder with an established association with PAH (including trisomy 21 and cobalamin C deficiency). In another 16 children (23%), genetic disorders without an established association with PAH were identified (including Noonan syndrome, Beal’s syndrome, and various copy number variations). Of the 70 children tested, 40 children were diagnosed with isolated PAH. Transplant-free survival, unadjusted for clinical variables, varied significantly between groups of children with different genetic disorders. Children with CbIC deficiency and children with PVOD had the worst unadjusted outcome with a median transplant-free survival of <1 year, whereas pediatric TBX4 variant carriers showed the most favorable outcome.

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Multi-System Inflammatory Syndrome in Children in Association with COVID-19

Multi-System Inflammatory Syndrome in Children in Association with COVID-19. View Article Simpson JM, Newburger JW. Circulation. 2020 Jun 11. doi: 10.1161/CIRCULATIONAHA.120.048726. Online ahead of print. PMID: 32525700 Take Home Points: Multisystem inflammation in children (MIS-C) is a newly recognized syndrome mimicking Kawasaki disease in some aspects of clinical presentation and clinical course temporally associated with COVID-19 infection. Multisystem inflammatory syndrome in children shares similarities with atypical Kawasaki disease, however, clinical criteria of diagnosis of Kawasaki disease was not fulfilled. Coronary dilation including giant aneurysm can happen. Markers of inflammation and cardiac involvement—including troponin I, brain natriuretic peptide, D-dimer, C-reactive protein, and interleukin 6—were all elevated. Left ventricular systolic dysfunction requiring ionotropic support is common. Additional studies are needed to determine the longer-term impact of MIS-C on myocardial function and coronary arteries. The optimal treatment for MIS-C is uncertain. Patients are often treated with immunomodulatory therapy including intravenous immunoglobulin and steroids. In more severe cases e anakinra, infliximab, or tocilizumab has been used. Comment from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:  This article is an excellent commentary on the above discussed original article by Simpson and Newburger.

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Acute heart failure in multisystem inflammatory syndrome in children (MIS-C) in the context of global SARS-CoV-2 pandemic

Acute heart failure in multisystem inflammatory syndrome in children (MIS-C) in the context of global SARS-CoV-2 pandemic. View Article Belhadjer Z, Méot M, Bajolle F, Khraiche D, Legendre A, Abakka S, Auriau J, Grimaud M, Oualha M, Beghetti M, Wacker J, Ovaert C, Hascoet S, Selegny M, Malekzadeh-Milani S, Maltret A, Bosser G, Giroux N, Bonnemains L, Bordet J, Di Filippo S, Mauran P, Falcon-Eicher S, Thambo JB, Lefort B, Moceri P, Houyel L, Renolleau S, Bonnet D.Circulation. 2020 May 17. doi: 10.1161/CIRCULATIONAHA.120.048360. Online ahead of print.PMID: 32418446 Take Home Points: Multisystem inflammatory syndrome in children (MIS-C) is a new syndrome that is temporally associated with exposure to COVID-19. Multisystem inflammatory syndrome in children shares similarities with atypical Kawasaki disease, but many clinical signs are unique. MIS-C is characterized by patients with recent diagnosis of COVID-19 presenting with fever, laboratory evidence of inflammation, and evidence of clinically severe illness requiring hospitalization, with multisystem (≥2) organ involvement (cardiac, renal, respiratory, hematologic, gastrointestinal, dermatologic, or neurologic) in the absence of other plausible diagnoses. Additional studies are needed to determine the full spectrum of this illness and its long-term effects on cardiac structure and function. Commentary from Dr. Shaji Menon (Salt Lake City, Utah), section editor of Pediatric Cardiology Journal Watch:   This retrospective multicenter study from 12 hospitals in France and 1 hospital in Switzerland presents data for children with acute left ventricular systolic dysfunction or cardiogenic shock and associated multisystem inflammatory syndrome between March 22 and April 30, 2020.  The inclusion criteria were the presence of fever (>38.5°C), cardiogenic shock, or acute left ventricular dysfunction (left ventricular ejection fraction <50%) with inflammatory state (C-reactive protein >100 mg/mL). 35 patients fulfilling the inclusion criteria with febrile cardiogenic shock or left ventricular dysfunction and inflammatory state were included in the study. All children presented with fever (>38.5°C). Gastrointestinal symptoms including abdominal pain, vomiting, or diarrhea present in majority (80%). Two patients underwent emergency operation for suspected appendicitis that was ultimately diagnosed as mesenteric adenolymphitis. Although clinical signs mimicking Kawasaki disease were common, including skin rash, cheilitis, cervical adenopathy, and meningism none of the patients met criteria for classic Kawasaki disease (Table 1). Only 6 patients complained of chest pain. The ECG was not specific, with ST-segment elevation in only 1 patient (Table 2). In a large proportion of patients, the hemodynamic presentation at admission to the pediatric ICU was shock with low systemic blood pressure. The median duration between the first clinical symptoms and symptoms of heart failure was 6 days (interquartile range, 4.5–6 days). Ventricular systolic dysfunction with global hypokinesia was common. One patient manifested takotsubo syndrome presentation with akinesis of the apical segment. Segmental hypokinesia and pericardial effusion was seen in 3 patients. Right ventricular systolic function was preserved in all patients. Dilatation of the coronary arteries (Z score >2 adjusted for body temperature) was found in 6 patients (17%), including 5 patients with dilatation of the left main stem and 1 patient with dilatation of the right coronary artery. The majority of patients received intravenous immunoglobulin (25 of 35 of patients). Twelve patients received intravenous steroids, 3 children received anakinra because of persistent severe inflammatory state and 23 of 35 patients were treated with therapeutic-dose heparin. Complete recovery of left ventricular systolic function was observed in 71% of patients. Five patients had residual mild to moderate left ventricular systolic dysfunction. None had a thrombotic or embolic event. Median ICU stay was 7 days (interquartile range, 3.7–10 days), and median hospital stay was 10 days (interquartile range, 8–14 days). Unlike patients with Kawasaki disease, median age of patients with MIS-C were older (10 years) and left ventricular dysfunction was more common at presentation. Maculopapular rash in a 12-year-old girl.  

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Echocardiographic two-dimensional speckle tracking identifies acute regional myocardial edema and sub-acute fibrosis in pediatric focal myocarditis with normal ejection fraction: comparison with cardiac magnetic resonance

Echocardiographic two-dimensional speckle tracking identifies acute regional myocardial edema and sub-acute fibrosis in pediatric focal myocarditis with normal ejection fraction: comparison with cardiac magnetic resonance View Article Chinali M, Franceschini A, Ciancarella P, Lisignoli V, Curione D, Ciliberti P, Esposito C, Del Pasqua A, Rinelli G, Secinaro A. Sci Rep. 2020 Jul 9;10(1):11321. doi: 10.1038/s41598-020-68048-5. PMID: 32647322 Take Home Points: Speckle tracking echocardiography is helpful to systolic functional abnormalities in children with focal myocarditis and normal ejection fraction. Regions of reduced longitudinal strain on echocardiography corresponded to regions of myocardial edema detected by CMR imaging. Reduced global longitudinal strain was associated with a higher percentage of myocardial edema. Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This retrospective study assessed the ability of speckle tracking echocardiography (STE) in children and adolescents with focal myocarditis and normal ejection fraction (EF) to identify acute systolic functional abnormalities and persistent subclinical systolic dysfunction. 33 pediatric patients were included according to the study flow chart (Figure 1). Echocardiography was performed on admission and at discharge. Echocardiographic parameters assessed in this study included left ventricular (LV) diameters and wall thickness, LVEF as well as LV inflow velocities (E, peak early diastolic filling; A, late diastolic peak velocities). Tissue Doppler was performed to calculate E/e’ ratio. STE was used to measure global and regional longitudinal strain. CMR was performed on admission to measure LV function as well as to assess myocardial fibrosis and edema. In all patients, ejection fraction was normal on admission and none of the patients presented with LV hypertrophy or dilatation. There was a normal pattern of diastolic filling with no evidence of increased LV filling pressure. Global longitudinal strain was reduced in 58% of the patients with the most affected regions being the inferoseptal, inferior and inferolateral segments. CMR imaging revealed a similar regional distribution of myocardial edema. Reduction in global longitudinal strain was associated with a higher percentage of myocardial edema (Figure 2). Echocardiography at discharge demonstrated a full recovery in 13 and persistent subclinical dysfunction in 6 children. Patients with persistent dysfunction were found to have more myocardial fibrosis during a follow up CMR study. In summary, the authors were able to demonstrate the usefulness of STE in identifying areas of regional (subclinical) systolic dysfunction corresponding to areas of myocardial edema. This relatively small study motivates for further research in this field.

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Congenital Heart Disease After the Fukushima Nuclear Accident: The Japan Cardiovascular Surgery Database Study

Congenital Heart Disease After the Fukushima Nuclear Accident: The Japan Cardiovascular Surgery Database Study View Article Hirata Y, Shimizu H, Kumamaru H, Takamoto S, Motomura N, Miyata H, Okita Y. J Am Heart Assoc. 2020 Jul 7;9(13):e014787. doi: 10.1161/JAHA.119.014787. Epub 2020 Jul 2. PMID: 32613886 Take Home Points: No increase was observed in the number of first time congenital cardiovascular surgeries between January 2010 and December 2013 in all of Japan and the area of the nuclear accident. The increase of the total number of congenital cardiovascular surgeries between 2010 and 2013 might be explained by a reduction in mortality for the initial surgery. Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric Cardiology Journal Watch:  This important registry-based study assessed the effect of the Fukushima nuclear accident in March 2011 on the incidence of congenital heart disease. The authors hypothesized that the incidence of congenital heart disease did not increase after the accident. Data from 59 facilities within the Japan Cardiovascular Surgery Database was used for the analyses. Information about patients who underwent all types of congenital cardiovascular surgeries between 1st January 2010 and 31st December 2015 at the 59 facilities were extracted from the database. Patients who were older than 2 years at the time of surgery were excluded from the analysis.  The number of first congenital cardiovascular surgeries by the patients’ birth year–month in all 59 facilities and in the area of the nuclear accident (Tohoku region) was counted. Second, the percentage of live births in Japan that underwent their first congenital cardiovascular surgeries at the 59 facilities was calculated. Third, the total number of operations for complex diseases performed on patients one year old and younger per operation year, regardless of when the patients were born was counted. Between 1st January 2010 and 31st December 2015 26 251 patients underwent 44 818 surgeries. Out of them, 11 919 patients were born between 2010 and 2013, and were 2 years or younger at the time of surgery. The authors did not observe a monthly increase in the number first congenital cardiovascular surgeries in all of Japan (Figure 1A) and in the Tohoku area (Figure 1B). Also no increase in the percentage of live births that underwent congenital heart surgeries was found (Figure 2A and 2B). The total number of all congenital cardiovascular surgeries increased between 2010 and 2013 whereas mortality for the initial operation went down. In summary, this study gives new insights on the effects of the 2011 nuclear accident in Fukushima the incidence of congenital heart disease. It suggests that there was no obvious increase in the number of congenital heart disease patients.    

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Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California

Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California View Article Peyvandi S, Baer RJ, Chambers CD, Norton ME, Rajagopal S, Ryckman KK, Moon-Grady A, Jelliffe-Pawlowski LL, Steurer MA. J Am Heart Assoc. 2020 Apr 21;9(8):e015255. doi: 10.1161/JAHA.119.015255. Epub 2020 Apr 19. Take Home Points Adverse social and environmental factors at the neighborhood level may play an important role in the development of congenital heart disease (CHD). Worse social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) quartiles had higher odds of CHD. Maternal comorbidities explain some, but not all, of this socio-environmental relationship with development of CHD. Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   The California Office of Statewide Health Planning and Development database was used to obtain data on newborn patients born between 2007 – 2012.  Maternal and infant data are linked in this database and ICD-9 codes were used for diagnostic classification.  Significant congenital heart disease (CHD) was defined as a defect that would require surgery within the 1st year of life.  A social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) were determined at the neighborhood level.  Z-scores for both indexes were obtained for each patient and scores were categorized into 4 quartiles, with 1st quartile being the most ideal situation and 4th quartile being the least ideal situation. Hierarchical logistic regression was used to determine the association between the main predictors and the primary outcome after adjustment for maternal factors and age of cohort.  Sensitivity analysis was conducted to determine the relationship after excluding single ventricle diagnosis in regions with high prenatal diagnosis.  A mediation analysis was used to test if maternal comorbidities may be in the causal pathway of SDI and EEI. During the time period studied, over 2 million live births were included in the study.  All individual sociodemographic and environmental factor Z-scores were worse for in the CHD group (7698 infants with CHD studied) compared to the controls.  The odds of live-born CHD were significantly higher among those with increasing SDI and EEI quartile.  Odds were also significantly higher in mothers with comorbidities to have a child with CHD.  These odds still were significant when excluding infants with known chromosomal abnormalities/syndromes (6120 infants with CHD studied).  Odds were also still significant when excluding single ventricle patients to take into account possible bias when evaluating just live-born births.  Odds were 1.48 (1.32 – 1.66) higher for having a child with CHD for those in the 4th quartile versus those in the 1st quartile.  Causal mediation analysis showed that 13% (95% CI, 10 – 20%) of the total effect of SDI/EEI on the incidence of CHD is mediated through the presence of maternal comorbidities with race/ethnicity as confounders in the relationship between maternal conditions and incidence of CHD. As the authors stated, this study is limited by the use of an administrative database where errors in entry may occur, ~20% of data were excluded due to incomplete data, only live-born infants were evaluated thus true incidence of CHD may be under-reported due to fetal demise/termination, misclassification of pollutants, and no data on timing of exposure to pollutants. Despite these limitations, this is a large database study that provides intriguing data suggesting how multifactorial variables may play a role in the development of CHD.  Improving the socioeconomic welfare and decreasing pollutant exposure may be another method to decrease the incidence of CHD in the overall population.

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Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy

Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy View Article El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH. J Am Soc Echocardiogr. 2020 Apr 9. pii: S0894-7317(20)30067-5. doi: 10.1016/j.echo.2020.01.020. [Epub ahead of print] PMID: 32279939 Take Home Points Exercise stress echocardiography (ESE) is a safe and feasible modality in children with hypertrophic cardiomyopathy. In children without rest LVOT gradient 44% develop gradients > 30 mm Hg with exercise. ESE-derived rest and exercise gradients correlated with risk of cardiac outcome. Children with LVOT gradients <30 mm Hg have lowest risk of CV events. Children with LVOT gradients ≥30 mm Hg have 5 times the risk of CV events. Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:   This is a single center retrospective chart review of all pediatric hypertrophic cardiomyopathy patients who underwent exercise stress echocardiography to determine if exercise stress echocardiography can be useful in risk stratifying hypertrophic cardiomyopathy subgroups and if LVOT obstruction occurs in the patients with nonobstructive hypertrophic cardiomyopathy. Methods:  Data was collected on all pediatric hypertrophic cardiomyopathy patient who underwent exercise stress echocardiography from 2007-2018 at Boston Children’s Hospital.  Subjects were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. The composite adverse endpoints on follow-up included heart transplant, aborted cardiac arrest, and sudden cardiac death. Results:  A total of 91 (61% male), median age 12 years (6-24 years) with hypertrophic cardiomyopathy underwent exercise stress echocardiography; baseline patient characteristics are described in table 1.  Median left ventricle wall thickness was 20 mm and median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33). Exercise stress echocardiography data; table 2.  Of the 40 patients in group 2 (resting LVOT <30 mmHg, exercise LVOT gradient ³ 30 mmHg) 20 had obstruction due to septal hypertrophy/systolic anterior motion, 13 patients mid cavitary obstruction and other 7 patients had a mixed obstruction etiology. Of the total cohort, 73 (80%) subjects  were free of cardiac events prior to the first exercise stress echocardiogram study.  In the 3 year follow up data 26 patients had 31 cardiac events. Discussion: In this cohort, 90 (99%) patients had an event-free exercise stress echocardiogram with one group 3 patient experiencing a fast-polymorphic ventricular fibrillation arrest requiring resuscitation during exercise stress test.  This data showed pediatric exercise stress echocardiogram for hypertrophic cardiomyopathy can be performed safely with low risk to the patients.

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Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California.

[et_pb_section fb_built="1" admin_label="section" _builder_version="3.22"][et_pb_row admin_label="row" _builder_version="3.25" background_size="initial" background_position="top_left" background_repeat="repeat"][et_pb_column type="4_4" _builder_version="3.25" custom_padding="|||" custom_padding__hover="|||"][et_pb_text admin_label="Text" _builder_version="4.7.4" background_size="initial" background_position="top_left" background_repeat="repeat" hover_enabled="0" sticky_enabled="0"]Environmental and Socioeconomic Factors Influence the Live-Born Incidence of Congenital Heart Disease: A Population-Based Study in California. Peyvandi S, Baer RJ, Chambers CD, Norton ME, Rajagopal S, Ryckman KK, Moon-Grady A, Jelliffe-Pawlowski LL, Steurer MA. J Am Heart Assoc. 2020 Apr 21;9(8):e015255. doi: 10.1161/JAHA.119.015255. Epub 2020 Apr 19. Take Home Points Adverse social and environmental factors at the neighborhood level may play an important role in the development of congenital heart disease (CHD). Worse social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) quartiles had higher odds of CHD. Maternal comorbidities explain some, but not all, of this socio-environmental relationship with development of CHD. Commentary from Dr. Clifford Cua (Columbus, OH), Section Editor of Pediatric Cardiology Journal Watch:   The California Office of Statewide Health Planning and Development database was used to obtain data on newborn patients born between 2007 – 2012.  Maternal and infant data are linked in this database and ICD-9 codes were used for diagnostic classification.  Significant congenital heart disease (CHD) was defined as a defect that would require surgery within the 1st year of life.  A social deprivation index (SDI) (6 measures of wealth and income) and environmental exposure index (EEI) (levels of exposure to pollutants) were determined at the neighborhood level.  Z-scores for both indexes were obtained for each patient and scores were categorized into 4 quartiles, with 1st quartile being the most ideal situation and 4th quartile being the least ideal situation. Hierarchical logistic regression was used to determine the association between the main predictors and the primary outcome after adjustment for maternal factors and age of cohort.  Sensitivity analysis was conducted to determine the relationship after excluding single ventricle diagnosis in regions with high prenatal diagnosis.  A mediation analysis was used to test if maternal comorbidities may be in the causal pathway of SDI and EEI. During the time period studied, over 2 million live births were included in the study.  All individual sociodemographic and environmental factor Z-scores were worse for in the CHD group (7698 infants with CHD studied) compared to the controls.  The odds of live-born CHD were significantly higher among those with increasing SDI and EEI quartile.  Odds were also significantly higher in mothers with comorbidities to have a child with CHD.  These odds still were significant when excluding infants with known chromosomal abnormalities/syndromes (6120 infants with CHD studied).  Odds were also still significant when excluding single ventricle patients to take into account possible bias when evaluating just live-born births.  Odds were 1.48 (1.32 – 1.66) higher for having a child with CHD for those in the 4th quartile versus those in the 1st quartile.  Causal mediation analysis showed that 13% (95% CI, 10 – 20%) of the total effect of SDI/EEI on the incidence of CHD is mediated through the presence of maternal comorbidities with race/ethnicity as confounders in the relationship between maternal conditions and incidence of CHD. As the authors stated, this study is limited by the use of an administrative database where errors in entry may occur, ~20% of data were excluded due to incomplete data, only live-born infants were evaluated thus true incidence of CHD may be under-reported due to fetal demise/termination, misclassification of pollutants, and no data on timing of exposure to pollutants. Despite these limitations, this is a large database study that provides intriguing data suggesting how multifactorial variables may play a role in the development of CHD.  Improving the socioeconomic welfare and decreasing pollutant exposure may be another method to decrease the incidence of CHD in the overall population.       [/et_pb_text][/et_pb_column][/et_pb_row][/et_pb_section]

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Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy.

Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy. El Assaad I, Gauvreau K, Rizwan R, Margossian R, Colan S, Chen MH. J Am Soc Echocardiogr. 2020 Apr 9. pii: S0894-7317(20)30067-5. doi: 10.1016/j.echo.2020.01.020. [Epub ahead of print] PMID: 32279939 Similar articles Select item 32271829   Take Home Points Exercise stress echocardiography (ESE) is a safe and feasible modality in children with hypertrophic cardiomyopathy. In children without rest LVOT gradient 44% develop gradients > 30 mm Hg with exercise. ESE-derived rest and exercise gradients correlated with risk of cardiac outcome. Children with LVOT gradients <30 mm Hg have lowest risk of CV events. Children with LVOT gradients ≥30 mm Hg have 5 times the risk of CV events.   Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch:   This is a single center retrospective chart review of all pediatric hypertrophic cardiomyopathy patients who underwent exercise stress echocardiography to determine if exercise stress echocardiography can be useful in risk stratifying hypertrophic cardiomyopathy subgroups and if LVOT obstruction occurs in the patients with nonobstructive hypertrophic cardiomyopathy. Methods:  Data was collected on all pediatric hypertrophic cardiomyopathy patient who underwent exercise stress echocardiography from 2007-2018 at Boston Children’s Hospital.  Subjects were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. The composite adverse endpoints on follow-up included heart transplant, aborted cardiac arrest, and sudden cardiac death. Results:  A total of 91 (61% male), median age 12 years (6-24 years) with hypertrophic cardiomyopathy underwent exercise stress echocardiography; baseline patient characteristics are described in table 1.  Median left ventricle wall thickness was 20 mm and median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33). Exercise stress echocardiography data; table 2.  Of the 40 patients in group 2 (resting LVOT <30 mmHg, exercise LVOT gradient ³ 30 mmHg) 20 had obstruction due to septal hypertrophy/systolic anterior motion, 13 patients mid cavitary obstruction and other 7 patients had a mixed obstruction etiology. Of the total cohort, 73 (80%) subjects  were free of cardiac events prior to the first exercise stress echocardiogram study.  In the 3 year follow up data 26 patients had 31 cardiac events. Discussion: In this cohort, 90 (99%) patients had an event-free exercise stress echocardiogram with one group 3 patient experiencing a fast-polymorphic ventricular fibrillation arrest requiring resuscitation during exercise stress test.  This data showed pediatric exercise stress echocardiogram for hypertrophic cardiomyopathy can be performed safely with low risk to the patients.        

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Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy

Septal Flash-like Motion of the Earlier Activated Ventricular Wall Represents the Pathophysiology of Mechanical Dyssynchrony in Single-Ventricle Anatomy Hayama Y, Miyazaki A, Ohuchi H, Miike H, Negishi J, Sakaguchi H, Kurosaki K, Shimizu S, Kawada T, Sugimachi M. J Am...

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Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis

Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis O'Connor MJ, Lorts A, Davies RR, Fynn-Thompson F, Joong A, Maeda K, Mascio CE, McConnell...

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