Abstract
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 (n = 27, 55.1%) and renal disease (n = 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, p = 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.
Highlights
Life expectancy for those born with congenital heart disease (CHD) has increased over the last few decades [1]
The number of adults with congenital heart disease (ACHD) is increasing and patients are reaching older age [2]; the median age of ACHD patients has increased to 40 years [1]
While extracardiac comorbidities were common, the presence of renal disease was an independent predictor of all-cause mortality
Summary
Life expectancy for those born with congenital heart disease (CHD) has increased over the last few decades [1]. The number of adults with congenital heart disease (ACHD) is increasing and patients are reaching older age [2]; the median age of ACHD patients has increased to 40 years [1]. While the outcome in these younger patients with SVP has been extensively studied, data regarding older patients (above 40 years of age) are scarce [5]. This is true for patients with SVP who have not had the Fontan operation [6,7]. Renal disease is an independent predictor of all-cause mortality
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