March

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.    

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.    

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.                

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.    

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.    

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.