Background The increasing complexity of congenital cardiac surgery has resulted in the increased use of extracorporeal membrane oxygenation (ECMO) support for children who cannot be weaned from cardiopulmonary bypass. The purpose of this research was to assess the mortality and morbidity in children requiring ECMO support after the repair of congenital heart defects (CHDs). Methods The hospital records of all patients with CHD who required ECMO after a cardiac surgical procedure between January 2001 and December 2016 were retrospectively reviewed. Various outcomes were reported and tested for any association with hospital death. Results A total of 113 children required ECMO for cardiopulmonary support after congenital cardiac surgery; 88 (77.9%) were placed on ECMO in the operating room. Median age of the patients was 3 months (range, 4 days–15 years) and median weight was 3.5 kg (range, 2.2–42.5). Forty-two (37.2%) survived to hospital discharge. In children with single-ventricle physiology, survival to discharge was 37.3% (19/51 patients) and for biventricular physiology, it was 37.1% (23/62 patients). Univariate analysis revealed number of days on ECMO support, renal failure, and stroke as risk factors for hospital mortality, while age and cross-clamp time were found to be statistically nonsignificant. Conclusion Satisfactory results can be achieved in pediatric patients by using ECMO support for postoperative cardiac and pulmonary failure refractory to medical management. Prolonged ECMO support, renal failure, and stroke are risk of mortality.

BackgroundJunctional ectopic tachycardia is a serious arrhythmia that frequently occurs after tetralogy of Fallot repair. Arrhythmia prophylaxis is not feasible for all pediatric cardiac surgery patients and identification of high risk patients is required. The objectives of this study were to characterize patients with JET, identify its predictors and subsequent complications and the effect of various treatment strategies on the outcomes in selected TOF patients undergoing total repair before 2 years of age.MethodsFrom 2003 to 2017, 609 patients had Tetralogy of Fallot repair, 322 were included in our study. We excluded patients above 2 years and patients with preoperative arrhythmia. 29.8% of the patients (n = 96) had postoperative JET.ResultsJET patients were younger and had higher preoperative heart rate. Independent predictors of JET were younger age, higher preoperative heart rate, cyanotic spells, non-use of B-blockers and low Mg and Ca (p = 0.011, 0.018, 0.024, 0.001, 0.004 and 0.001; respectively). JET didn’t affect the duration of mechanical ventilation nor hospital stay (p = 0.12 and 0.2 respectively) but prolonged the ICU stay (p = 0.011). JET resolved in 39.5% (n = 38) of patients responding to conventional measures. Amiodarone was used in 31.25% (n = 30) of patients and its use was associated with longer ICU stay (p = 0.017). Ventricular pacing was required in 4 patients (5.2%). Median duration of JET was 30.5 h and 5 patients had recurrent JET episode. Timing of JET onset didn’t affect ICU (p = 0.43) or hospital stay (p = 0.14) however, long duration of JET increased ICU and hospital stay (p = 0.02 and 0.009; respectively).ConclusionJET increases ICU stay after TOF repair. Preoperative B-blockers significantly reduced JET. Patients with preoperative risk factors could benefit from preoperative arrhythmia prophylaxis and aggressive management of postoperative electrolyte disturbance is essential.

Background: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival. Methods: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded. Results: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01. Conclusion: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.

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Background Currently, non‐valved conduits are preferred for extracardiac total cavo‐pulmonary connection (TCPC). However, previous work has failed to provide objective data comparing the postoperative outcome between non‐valved TCPCs and bovine jugular vein valved xenograft (BJV) TCPCs. Hence, the objective of this study is to compare the postoperative outcomes in extracardiac TCPC patients who received BJV vs synthetic non‐valved conduits and evaluate the effect of BJV on liver fibrosis. Methods Of 206 patients who had extracardiac TCPC from 2002 to 2017 were divided into three groups. Group A (n = 66) received BJV, group B (n = 37) received PET conduits and group C (n = 103) received polytetrafluoroethylene (PTFE) tube. Study endpoints were hospital outcomes, conduits thrombosis, reinterventions, and survival. Liver stiffness and fibrosis were assessed in eight patients with BJV. Results Preoperative parameters were comparable among groups. Thrombosis was significantly lower in group C (P < .0003) but no difference between groups A and B (P = .951). Reinterventions did not differ significantly among groups (Log‐rank P = .598). Hospital deaths occurred in seven patients (3.4%). There was no difference in survival between groups (Log‐rank P = .221). The median liver stiffness score was 18.65 kPa and the eight patients had advanced liver fibrosis (grade F3‐4) in group A. Conclusion PTFE is the recommended conduit for TCPC with a lower risk of thrombosis compared to BJV and PET. BJV conduits in TCPC circuits may not protect against liver fibrosis. BJV should not be considered as an option for TCPC.

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BackgroundIsolated right Superior Vena Cava drainage into the left atrium in the absence of other cardiac anomalies is an extremely rare condition. The vein of Galen aneurysmal malformation is a congenital vascular malformation. It comprises 1% of all pediatric congenital anomalies. The association vein of Galen aneurysmal malformation, with congenital heart disease has been described.Case presentationWe describe a 16-months old toddler presenting at 7-months of age with respiratory distress and cyanosis. CT brain showed Vein of Galen aneurysmal malformations. Echocardiography showed partial anomalous systemic venous drainage in the form of right superior vena cava drained into left atrium. Four sessions of Endovascular embolization were performed. Surgical repair of partial anomalous systemic venous drainage was done successfully.ConclusionsThe superior vena cava in our case overrides the atrial septum promoting direct drainage of venous return into the LA, thus causing dilated left ventricle instead of dilatation of right ventricle which is the usual presentation of VAGMs.

Background  Factor X deficiency (also known as Stuart–Prower factor deficiency) is an autosomal recessive extremely rare hereditary hematologic disorder, affecting around 1:1,000,000 of the general population. Case Presentation  This case report describes a patient with hypoplastic left heart syndrome and severe factor X deficiency, who underwent staged surgical palliation. From stage 1 Norwood palliation, through superior cavopulmonary anastomosis and ending with total cavopulmonary connection with satisfactory hemostasis and no significant perioperative bleeding complication. Conclusion  The need to maintain hemostasis while aiming to prevent intracardiac thrombosis requires multidisciplinary team approach including hematologist, cardiac surgeon, pediatric cardiac intensivist, and anesthesiologist along with meticulous hemostasis during surgery and careful monitoring of coagulation profile in the postoperative period.