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.

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.

Introduction: Disordered coagulation, clot formation and distal limb ischemia are complications of extracorporeal membrane oxygenation (ECMO) with significant morbidity and mortality. Medicinal leech therapy (hirudotherapy) has been attempted in plastic and orthopedic surgeries to improve venous congestion and salvage ischemic flaps. To our knowledge this has not been reported in pediatric cardiac surgery or during ECMO support. We present a complex neonate whose ECMO course was complicated by distal limb ischemia for whom leech therapy was attempted. Patient and Intervention: A 2 week-old 2.7 kg infant required ECMO support secondary to perioperative multiorgan system dysfunction following repair of critical coarctation and ventricular septal defect. Despite systemic anticoagulation, his clinical course was complicated by arterial thrombus, vasopressor-induced vascular spasm and bilateral distal limb ischemia. Medicinal leech therapy was tried after initially failing conventional measures. Result: Following the third leech application, this patient developed significant hemorrhage from the web space adjacent to the left great toe. An estimated 450 mL of blood loss occurred and more than 300 mL of blood product transfusions were required. He ultimately progressed to irreversible systemic end organ dysfunction and comfort care was provided. Conclusion: The use of medicinal leech therapy in pediatric cardiac surgery may be considered to minimize the consequences of advanced limb ischemia and venous congestion. However, this should be used with caution while patients are systemically anticoagulated during ECMO support. A directed review is presented here to assist in determining optimal application and potential course of therapy.

Completion extracardiac Fontan is the final stage palliation for functional single ventricle. It has been associated with less incidence of atrial arrhythmias and more laminar flow in the Fontan Pathway. Here, we present our technique of off-pump extracardiac Fontan.