Pediatric Cardiology

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.