Objective: The traditional outcomes of the Fontan Operation (FO) in Endocardial Cushion Defect (ECD) patients have been suboptimal. Previous studies have been limited by the smaller number of ECD patients, longer study period with an era effect and do not directly compare short-term outcomes of FO in ECD patients with non-ECD patients. Our study aims to address these shortcomings. Methods: A retrospective analysis of the Kids Inpatient Database (2009, 2012, and 2016) for the FO was done. The groups were divided into those who underwent FO with ECD as compared to non-ECD diagnosis. The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. Results: 3380 patients underwent the FO of which 360 patients (11%) were FO-ECD. ECD patients were more likely to have Down syndrome, Heterotaxy syndrome, transposition/DORV, and TAPVR as compared to non-ECD patients. FO-ECD had a higher discharge-mortality (2.84% vs. 0.45%, p=0.04). The length of stay (16 vs. 13 days, p=0.05) and total charges incurred ($ 283, 280 vs. 234, 106, p=0.03) for the admission were higher in the FO-ECD as compared to non-ECD patients. In multivariable analysis: ECD diagnosis, cardiac arrest, acute kidney injury, and post-operative hemorrhage were predictors of mortality. Conclusion: Contemporary outcomes for FO are excellent with very low overall operative mortality. However, the outcomes in ECD patients are inferior with higher operative mortality than non-ECD patients. Occurrence of post-operation complications, associated TAPVR and a diagnosis of ECD were predictive of a negative outcome.

The management of patients with transposition complex in combination with an interrupted aortic arch (IAA) presents a technical challenge to the surgeon to decide which is the best approach to correct both defects. This is a rare disorder and with significant variation in anatomic arrangements deciding on the ideal surgical repair. Over time a single-stage approach to repair has become standard.

Kawasaki Disease (KD) is a systemic vasculitis of small and medium arteries, preferably affecting coronary arteries. It is one of the most frequent causes of acquired heart disease in children. Despite being comprehensively studied, its etiopathogenesis is not totally explained. The surgical procedures usually become necessary during the late follow-up and may be coronary artery bypass grafting, cardiac defibrillator implantation with or without cardiac resynchronization therapy, or cardiac transplantation.

The Vasoactive-Inotropic Score at 48 hours is a good surrogate marker for adverse postoperative events in pediatric patients undergoing cardiac surgery with cardiopulmonary bypass and is limited by its ability to delineate the underlying cause for an unfavourable clinical course. Thus, other predictors such as the Technical Performance Score are likely to highlight the underlying cause and can be used to improve outcomes. However, patients with a high postoperative VIS score at 48 hours may benefit from closer longer-term follow for outcomes such as late survival, functional class, and need for reoperation.

The outcomes of the arterial switch operation have improved over a period of time with the elimination of coronary artery anatomy as a risk factor for operative mortality in some series. However, cumulatively, when all the series published so far are analyzed, two coronary variations, namely the single sinus coronary artery origin and intramural type, persist as risk factors for an adverse operative outcome.

This is a commentary on a single-institution retrospective review that describes the short-term outcomes of the pediatric patients who underwent re-operative surgery for residual or recurrent coarctation after a previous coarctation repair. There were 51 patients over 12 years.

Introduction: Opinion is divided about optimal early timing of the Fontan Operation (FO). While some studies have suggested 3 years-of-age, others have shown good outcomes below 2 years-of -age. We analyzed the impact of age ≤2-years as compared age >2-years on short-term outcome of the FO using a large national database. Methods: A retrospective analysis of the Kids Inpatient Database (2009-16) for the FO was done. The groups were divided into those who underwent FO at age ≤2-years (EF) as compared to age >2-years (LF). The data was abstracted for demographics, clinical characteristics, and operative outcomes. Standard statistical tests were used. Results: 3381 patients underwent FO during this period of which 1482 (44%) were EF. The mean ages of the EF and LF were 1.6 and 4.3, respectively (p< 0.001). LF were more likely to be non-White, female, and have Heterotaxy syndrome. HLHS was more common in EF. There was no difference in the discharge mortality, length of stay, disposition (majority went home), and mean total charges incurred. The overall discharge mortality was low at 0.7% (24/3381). In multivariate analysis: cardiac arrest, acute kidney injury, mechanical ventilation >96 hours, endocardial cushion defect and non-White ethnicity were predictors of a mortality and not age. Conclusion: Contemporary outcomes for FO are excellent with equivalent short-term outcomes in both the age groups. Occurrence of postoperative complications, non-white ethnicity and endocardial cushion defect diagnosis were predictive of a negative outcome.

The use of sutureless valves in infective endocarditis has been previously reported. Singh et al have reported a single-center successful surgical treated pulmonary endocarditis in 2 cases with a sutureless bioprosthesis.

Multiple ventricular septal defects (m-VSD), are a challenging clinical problem. m-VSD can be onerous to manage. Besides the inability to close all the defects in one operative setting due to inadequate visualization, previously undetected defects may become clinically apparent after the closure of the dominant defects, leading to inadequate ventricular septation. This increases the morbidity from the progression of pulmonary hypertension, persistence of congestive cardiac failure, higher incidence of postoperative heart block, and the need for reoperations.

WeChat and access to wireless communication may offer a continuum of care following medical and surgical intervention. This cardiac surgery research study evaluates the process of parental education and social support following pediatric cardiac surgery utilizing standard of care compared to telehealth.

Multi-disciplinary teams present the framework in the increasingly challenging care provided for patients. Wang et al. present a 37 year old female who is 35 weeks pregnant when a rare cardiac angiosarcoma is diagnosed.

Bas et al. report the clinical measurement, using High Performance Liquid Chromatography (HPLC), of asymmetric dimethyarginine in two groups of patients undergoing aortic coarctation repair.