P4522Should we leave pulsatile forward flow after the bidirectional Glenn operation?

Abstract

950 Cardiomyopathy / Univentricular heart, heart failure and pulmonary hypertension in congenital heart diseases Methods: Family screening was offered to 2,372 consecutive unrelated patients; 10,000 subjects (43±7 years old, 52% men) were evaluated (mean 4.2 relatives/family), of which 32% were affected and 6% were possibly affected. Familial study was recommended because of an index case with hypertrophic cardiomyopathy (HCM) in 42%, idiopathic dilated cardiomyopathy (DCM) in 17%, arrhythmogenic right cardiomyopathy (ARCM) in 4%, arrhythmogenic left cardiomyopathy (ALCM) in 1%, left ventricular non-compaction (LVNC) in 4%, Brugada syndrome (BS) in 9%, long QT syndrome (LQTS) in 4% and other conditions in 18%. Index case and non-index cases were defined. Results: Familial disease was confirmed in 47%; 51% with HCM, 53% with DCM, 39% with ARCM, 92% with ALCM, 37% with LVNC, 28% with BS, and 35% with LQTS. 606 affected non-index patients were identified (46% meeting HCM, 19% DCM, 6% ARCM, 3% ALVC, 5% LVNC, 9% BS, and 6% LQTS criteria), in whom appropriate risk stratification and medication, if needed, were initiated. 23% had a family history of sudden death. 20% of all affected were athletes (29% of whom were non-index patients). Cardioverter defibrillator (ICD) implantation was indicated in 359 patients (64% for primary and 36% for secondary prevention). 20% of ICD implanted were in non-index cases. Conclusion:The prevalence of familial disease in inherited cardiac conditions is high. Systematic familial study identified a significant number of asymptomatic affected patients who could benefit from early treatment to prevent complications. Dedicated clinics and multidisciplinary teams are needed for proper screening programs.UNIVENTRICULAR HEART, HEART FAILURE AND PULMONARY HYPERTENSION IN CONGENITAL HEART DISEASES P4522 | BEDSIDE Should we leave pulsatile forward flow after the bidirectional Glenn operation?P. Walker-Smith, Q. Chen, H. Weaver, M. Caputo, S. Stoica, A. Parry, R. Tulloh. University Hospitals Bristol NHS Foundation Trust, Bristol Royal Hospital for Children, Bristol, United KingdomBackground: Children with functionally univentricular hearts undergo a treatment pathway to the Fontan operation. A lack of significant pulmonary artery pulse pressure could be deleterious for pulmonary compliance and long term health of the circuit. We wished to try to determine whether the presence of pulsatile flow at the time of the Fontan improved outcome after this operation. Methods: 148 children who underwent Fontan at our institution from 1999 to 2016 underwent cardiac catheterisation to determine pulse pressure, transpulmonary gradient and pulmonary vascular resistance, along with verification of anatomy and function pre-operatively. At Fontan 48 had undergone pulmonary artery band, 56 had previous systemic to pulmonary shunt and 44 had native pulmonary stenosis to protect their pulmonary vasculature. Comparison was made between those with forward flow (group1) at time of Fontan operation and those without (group...