Univentricular hearts not undergoing Fontan: the type of palliation matters

• Published in: Revista española de cardiología (English ed.) 2024-01, Vol.77 (1), p.6-16
• Main Authors: Gordon, Blanca, Buendía-Fuentes, Francisco, Rueda-Soriano, Joaquín, Merás Colunga, Pablo, Gallego, Pastora, González García, Ana Elvira, Prieto-Arévalo, Raquel, Segura de la Cal, Teresa, Rodríguez-Puras, María J., Montserrat, Silvia, Sarnago-Cebada, Fernando, Alonso-García, Andrés, Oliver, José M., Dos-Subirà, Laura
• Format: Article
• Language: English
• Subjects: Adult; Aortopulmonary shunts; Atrial Fibrillation; Atrial Flutter; Cardiopatías congénitas; Cavopulmonary shunts; Congenital heart defects; Corazón univentricular; Fontan Procedure; Heart Defects, Congenital - surgery; Heart Ventricles - surgery; Humans; Procedimiento de Fontan; Pulmonary Artery - surgery; Retrospective Studies; Shunt aortopulmonar; Shunt cavopulmonar; Single ventricle; Treatment Outcome; Univentricular Heart - surgery
• ISSN: 1885-5857; 1885-5857
• DOI: 10.1016/j.rec.2023.03.001

There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation. SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death. We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037). The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality. La información sobre pacientes con fisiología univentricular (FU) y flujo pulmonar restrictivo no sometidos a cirugía de Fontan es escasa. El objetivo de este estudio es comparar la supervivencia y los eventos cardiovasculares en estos pacientes según el tipo de paliación. Los datos de pacientes con FU se obtuvieron de las bases de datos de 7 centros con unidades de cardiopatías congénitas del adulto. Se excluyó a los pacientes que completaron la circulación de Fontan o desarrollaron un síndrome de Eisenmenger. Se crearon 3 grupos según la fuente de flujo pulmonar: G1, flujo anterógrado pulmonar restrictivo; G2, shunt cavopulmonar, y G3, shunt aortopulmonar±shunt cavopulmonar. El objetivo principal fue la muerte. Se identificó a 120 pacientes. La media de edad en la primera visita fue 32,2 años. El seguimiento med