Francisco Buendía-Fuentes1, Blanca Gordon-Ramírez2, Laura Dos Subirà3, Pablo Merás4, Pastora Gallego5, Ana González4, Raquel Prieto-Arévalo6, Teresa Segura7, María J Rodríguez-Puras5, Silvia Montserrat8, Fernando Sarnago-Cebada7, Andrés Alonso-García6, José M Oliver6, Joaquín Rueda-Soriano1. 1. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitari i Politecnic La Fe, Instituto de Investigación Sanitaria La Fe, Centro de Investigación Biomédica en Red Cardiovascular, Valencia, Spain. 2. Unitat Integrada de Cardiopaties Congènites de l'Adolescent i l'Adult Vall d'Hebron-Sant Pau, Department of Cardiology, Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Barcelona, Spain. 3. Unitat Integrada de Cardiopaties Congènites de l'Adolescent i l'Adult Vall d'Hebron-Sant Pau, Department of Cardiology, Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Barcelona, Spain; Centro de Investigación Biomédica en Red Cardiovascular, Barcelona, Spain. Electronic address: laura_dos_subira@hotmail.com. 4. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario La Paz, Madrid, Spain. 5. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario Virgen del Rocio, Instituto de BioMedicina de Sevilla, Centro de Investigación Biomédica en Red Cardiovascular, Sevilla, Spain. 6. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital General Universitario Gregorio Marañon, Centro de Investigación Biomédica en Red Cardiovascular, Madrid, Spain. 7. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Universitario 12 Octubre, Madrid, Spain. 8. Adult Congenital Heart Disease Unit, Department of Cardiology, Hospital Clinic Barcelona, Institut Clinic Cardiovascular, Instituto de Investigaciones Biomédicas August Pi i Sunyer, Centro de Investigación Biomédica en Red Cardiovascular, Barcelona, Spain.
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 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points. RESULTS: A total of 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 atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 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 in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 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 atrioventricular 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.
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 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points. RESULTS: A total of 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 atrioventricular valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 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 in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 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 atrioventricular 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.