Patients with Single-Ventricle Physiology over the Age of 40 Years

• Main Authors: Claudia Pujol, Sandra Schiele, Susanne J. Maurer, Julia Hock, Celina Fritz, Alfred Hager, Peter Ewert, Oktay Tutarel
• Format: Article
• Language: English
• Published: MDPI AG 2020-12-01
• Series: Journal of Clinical Medicine
• Subjects: single-ventricle physiology; mortality; renal disease; adult congenital heart disease; Fontan
• Online Access: https://www.mdpi.com/2077-0383/9/12/4085

Background: Single-ventricle physiology (SVP) is associated with significant morbidity and mortality at a young age. However, survival prospects have improved and risk factors for a negative outcome are well described in younger cohorts. Data regarding older adults is scarce. Methods: In this study, SVP patients under active follow-up at our center who were ≥40 years of age at any point between January 2005 and December 2018 were included. Demographic data, as well as medical/surgical history were retrieved from hospital records. The primary end-point was all-cause mortality. Results: Altogether, 49 patients (19 female (38.8%), mean age 49.2 ± 6.4 years) were included. Median follow-up time was 4.9 years (interquartile range (IQR): 1.8–8.5). Of these patients, 40 (81.6%) had undergone at least one cardiac surgery. The most common extracardiac comorbidities were thyroid dysfunction (<i>n</i> = 27, 55.1%) and renal disease (<i>n</i> = 15, 30.6%). During follow-up, 10 patients (20.4%) died. On univariate analysis, renal disease and liver cirrhosis were predictors of all-cause mortality. On multivariate analysis, only renal disease (hazard ratio (HR): 12.5, 95% confidence interval (CI): 1.5–106.3, <i>p</i> = 0.021) remained as an independent predictor. Conclusions: SVP patients ≥40 years of age are burdened with significant morbidity and mortality. Renal disease is an independent predictor of all-cause mortality.