LONG TERM OUTCOMES OF ADULTS WITH SINGLE VENTRICLE PHYSIOLOGY NOT UNDERGOING FONTAN REPAIR: A MULTICENTRE EXPERIENCE

Abstract Background To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology. Methods Multicentre, observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into three groups. (Eisenmenger - Restricted Pulmonary flow - Aortopulmonary shunt) Death was considered the main endpoint. Other clinical outcomes occurring during follow-up were considered as secondary endpoints. Results 146 patients, mean age 32.5±11.1 years were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality (at least moderate AV valve regurgitation, platelet count 120ms). 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent (atrial arrhythmias: 19.2%, stroke: 15.1%, pacemaker/ICD: 6.2%/2.7%). Conclusions Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate AV valve regurgitation, thrombocytopenia, renal dysfunction and QRS duration >120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality.