January

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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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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Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi: 10.1007/s00246-020-02293-3. [Epub ahead of print] PMID: 31980851 Take Home Points: • Routine cardiac catheterization is a common part of Fontan surveillance algorithms. • Despite appearing to be clinically stable, a large proportion of Fontan patients in this cohort required transcatheter interventions; required initiation of pulmonary vasodilator therapy; and were identified to have hepatic fibrosis. Commentary from Dr. Konstantin Averin (Edmonton), catheterization section editor of Pediatric Cardiology Journal Watch: As survival of patients with single ventricle physiology palliated with a Fontan circulation improves it is becoming increasingly recognized that they are prone to developing significant end organ pathology. Various monitoring strategies have been proposed but all usually involve a surveillance catheterization 5-10 years after the Fontan surgery. The value of this assessment has not been fully explored. The authors sought to explore the utility of surveillance cardiac catheterization in stable Fontan patients at a single center. Sixty-three Fontan patients (mean age 14.6 ± 3.0 and mean time from Fontan surgery 11.3 ± 3.1 years) underwent cardiac catheterization over a 5-year period. The pre-catheterization echocardiograms (within a year) were unremarkable – 80% had normal ventricular function and less than mild AVV insufficiency. Baseline hemodynamics were mostly reassuring with 4 patients having a Fontan pressure > 15 mmHg and 19 having a PVRi > 2.0 iWu. Vasoreactivity testing with iNO was performed in 53 and half had a reduction in the PVRi of at least 20%. Fifty-seven patients underwent a trans-jugular liver biopsy at the time of procedure with more than half (32/57) demonstrating higher grades of congestive hepatic fibrosis. Forty percent of patients had an intervention at the time of the procedure: pulmonary artery stent (16), existing stent dilation (5), SVC angioplasty (4), Fontan stent (3), and others (7). There were no major complications. Despite the limitations of this work, the authors demonstrate that routine invasive assessment of Fontan patients can provide valuable information and allow for optimization of the Fontan circulation. Given the high morbidity experienced by this patient population it is important for providers to be pro-active in longitudinal Fontan assessment management as a stable Fontan is not necessarily a good Fontan

Routine Surveillance Catheterization is Useful in Guiding Management of Stable Fontan Patients. Patel ND, Sullivan PM, Sabati A, Hill A, Maedler-Kron C, Zhou S, Shillingford N, Williams R, Takao C, Badran S. Pediatr Cardiol. 2020 Jan 24. doi:...

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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,...

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