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Background Multicenter studies on infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) are lacking. We report the intermediate-term outcomes after ALCAPA repair in a multicenter cohort and identify risk factors for reintervention or death after discharge. Methods We retrospectively reviewed infants under 1 year of age who underwent ALCAPA repair from January 2009 to March 2018 at 21 US centers. The primary composite outcome was freedom from reintervention or death after discharge. We used the Kaplan-Meier survival analysis to examine freedom from reintervention or death and the Cox proportional hazard analysis to identify risk factors for this composite outcome. Results One hundred seventy-seven infants underwent ALCAPA repair; 170 (97%) survived to hospital discharge without transplantation. Twenty-three patients were lost to follow-up. The median duration of follow-up in the remaining 147 patients was 3.8 years (25%, 75%: 1.9 years, 6.0 years). Echocardiographic data were available at ∼3 years after discharge in 98 patients. Left ventricular function was normal in 96 patients (98%), whereas 26 patients (27%) had greater than mild mitral valve regurgitation. Sixteen patients (11%) underwent 20 reinterventions with 1 late death. Patients undergoing the Takeuchi procedure or atypical repairs (hazard ratio, 8.0; 95% confidence interval, 2.1-30.0) or with moderate or greater mitral regurgitation on discharge echocardiogram (hazard ratio, 3.4; 95% confidence interval, 1.2-9.1) were at increased risk for reintervention. Conclusions Intermediate-term outcomes after ALCAPA repair in infants are favorable. Persistent left ventricular dysfunction and reinterventions were uncommon, and mortality was rare. Patients who required atypical surgical repair or had moderate or greater mitral regurgitation at discharge warrant closer follow-up.

We review the etiologies of neurodevelopmental outcomes in patients with congenital heart disease. Developmental delays have been identified in patients with congenital heart disease (CHD). Though historically neurodevelopmental morbidity in patients with CHD was thought to be surgically related, recent advances in imaging technology and the ability to prospectively provide longitudinal follow-up indicate multiple complex etiologies of neurodevelopmental delay in this group. It has been suggested that alterations in cerebral perfusion is related to cerebral dysgenesis and immaturity. Additionally, while there has been significant improvement in surgical technique, the brain is still vulnerable to ischemia and injury, further placing the patient at risk for developmental delays. The neurodevelopmental and cognitive delays span many domains and may not be detected until adolescence when cognitive and neurobehavioral demands increase. Given these comorbidities, there is a need for early and frequent multidisciplinary assessments of the patient and early identification of delays to provide access to resources to improve these patients’ quality of life.

Truncal valve management in patients with truncus arteriosus is a clinical challenge, and indications for truncal valve intervention have not been defined. We sought to evaluate truncal valve dysfunction and primary valve intervention in patients with truncus arteriosus and determine risk factors for later truncal valve intervention. We conducted a retrospective cohort study of children who underwent truncus arteriosus repair at 15 centers between 2009 and 2016. Multivariable competing risk analysis was performed to determine risk factors for later truncal valve intervention. We reviewed 252 patients. Forty-two patients (17%) underwent truncal valve intervention during their initial surgery. Postoperative extracorporeal support, CPR, and operative mortality for patients who underwent truncal valve interventions were statistically similar to the rest of the cohort. Truncal valve interventions were performed in 5 of 64 patients with mild insufficiency; 5 of 16 patients with mild-to-moderate insufficiency; 17 of 35 patients with moderate insufficiency; 5 of 9 patients with moderate-to-severe insufficiency; and all 10 patients with severe insufficiency. Twenty patients (8%) underwent later truncal valve intervention, five of whom had no truncal valve intervention during initial surgical repair. Multivariable analysis revealed truncal valve intervention during initial repair (HR 11.5; 95% CI 2.5, 53.2) and moderate or greater truncal insufficiency prior to initial repair (HR 4.0; 95% CI 1.1, 14.5) to be independently associated with later truncal valve intervention. In conclusion, in a multicenter cohort of children with truncus arteriosus, 17% had truncal valve intervention during initial surgical repair. For patients in whom variable truncal valve insufficiency is present and primary intervention was not performed, late interventions were uncommon. Conservative surgical approach to truncal valve management may be justifiable.

Background Literature describing morbidity and mortality after truncus arteriosus repair is predominated by single-center reports. We created and analyzed a multicenter dataset to identify risk factors for late mortality and right ventricle-to-pulmonary artery (RV-PA) conduit reintervention for this patient population. Methods We retrospectively collected data on children who underwent repair of truncus arteriosus without concomitant arch obstruction at 15 centers between 2009 and 2016. Cox regression survival analysis was conducted to determine risk factors for late mortality, defined as death occurring after hospital discharge and greater than 30 days after operation. Probability of any RV-PA conduit reintervention was analyzed over time using Fine-Gray modeling. Results We reviewed 216 patients with median follow-up of 2.9 years (range, 0.1 to 8.8). Operative mortality occurred in 15 patients (7%). Of the 201 survivors there were 14 (7%) late deaths. DiGeorge syndrome (hazard ratio [HR], 5.4; 95% confidence interval [CI], 1.6 to 17.8) and need for postoperative tracheostomy (HR, 5.9; 95% CI, 1.8 to 19.4) were identified as independent risk factors for late mortality. At least one RV-PA conduit catheterization or surgical reintervention was performed in 109 patients (median time to reintervention, 23 months; range, 0.3 to 93). Risk factors for reintervention included use of pulmonary or aortic homografts versus Contegra (Medtronic, Inc, Minneapolis, MN) bovine jugular vein conduits (HR, 1.9; 95% CI, 1.2 to 3.1) and smaller conduit size (HR per mm/m2, 1.05; 95% CI, 1.03 to 1.08). Conclusions In a multicenter dataset DiGeorge syndrome and need for tracheostomy postoperatively were found to be independent risk factors for late mortality after repair of truncus arteriosus, whereas risk of conduit reintervention was independently influenced by both initial conduit type and size.

Post-pericardiotomy syndrome (PPS) is an inflammatory process involving the pleura, pericardium, or both and occurs after cardiothoracic surgery. Surgical atrial septal defect (ASD) closure is associated with higher incidence of PPS post-operatively as compared to other operations. Reported incidence of PPS varies from 1 to 40%. NSAIDs are often used to treat PPS and in our center, some practitioners have prescribed ibuprofen prophylactically. This study sought to investigate the impact of prophylactic treatment with ibuprofen on the development and severity of PPS following surgical ASD closure, with particular attention to secundum-type ASDs. We retrospectively reviewed clinical and operative data of all surgical ASD repairs in our center from 1/2007 to 7/2017. ASDs were grouped by subtype. PPS was considered positive if the primary cardiologist diagnosed and documented clinical signs of PPS on post-operative outpatient follow-up. Records were reviewed to confirm documented diagnosis of PPS. A total of 245 cases were reviewed with 207 having sufficient data. Median age was 2 years (range 4 months-27 years), female 57%. Overall incidence of PPS was 10%. There was no difference in incidence of PPS in those prescribed ibuprofen as compared to those who were not. This was true for both the entire cohort and the subgroup analysis (P = 1.0). Four patients overall required pericardiocentesis, none of whom received prophylactic ibuprofen. Prophylactic ibuprofen prescription following surgical ASD repair did not reduce the rate of PPS in our cohort.

The purpose of this paper is to review the presentation, mechanism and acute management of pediatric tachyarrhythmias and to discuss current updates in the literature. In 2015, the AHA updated the PALS guidelines to include lidocaine as a first line agent alongside amiodarone in the management of ventricular tachycardia. A recent study re-addressed the safety of the use of adenosine on heart transplant patients with supraventricular tachycardia (SVT). Also, there have been recent studies which explored alternative prevention modalities for postoperative junctional ectopic tachycardia such as the use of dexmedetomidine and higher magnesium levels. This review discusses the mechanism, presentation, and management of pediatric tachyarrhythmias. It focuses on SVT, as it is the most common cardiac arrhythmia in children requiring intervention, but also discusses other life-threatening arrhythmias including ventricular arrhythmias and Wolff-Parkinson-White syndrome. It reviews the current updates in acute management and discusses future directions of prophylactic management.

Post pericardiotomy syndrome (PPS) is an inflammatory process involving the pleura, pericardium or both and occurs after cardiothoracic surgery. Surgical ASD closure is associated with a higher rate of PPS compared to other forms of CHD. Reported incidence of PPS varies from 1-40%. NSAIDs are often