Abstract 20581: Renin Angiotensin Aldosterone System Genotype in Adults Late After Fontan Palliation
Introduction: Adults with single ventricle physiology palliated with a Fontan circulation experience high morbidity and mortality due to circulatory failure. Renin-angiotensin-aldosterone (RAAS) genotype contributes to increased cardiovascular morbidity and mortality in various forms of acquired heart failure. This study evaluated the prognostic value of RAAS genotype evaluation and serum brain natriuretic peptide in a contemporary cohort of adults following Fontan palliation.
Hypothesis: RAAS genotype would be associated with increased ventricular mass late after the Fontan operation.
Methods: Single-centre prospective study of adults (n=106) with single ventricle physiology following Fontan palliation (median age 27±9 years). Patients were genotyped for 5 pro-hypertrophic RAAS gene polymorphisms. Serum BNP levels, ventricular mass and function, and clinical events were compared between those with ≥2 homozygous risk genotypes (‘high-risk”, n=31) versus those with ≤1 homozygous risk genotypes (‘low risk”, n=75).
Results: High-risk genotype was associated with impaired ventricular compliance and higher serum BNP levels. There was no association between RAAS genotype, ventricular mass, systolic function, and/or adverse cardiovascular events. Late Fontan failure occurred in 20% of patients. Serum BNP emerged as an independent predictor of late Fontan failure [HR 1.11 (CI 1.01 - 1.23) for 50 unit increase in BNP, p=0.04] and death [HR 1.25 (CI 1.07 - 1.47) for each 50 unit increase in BNP, p=0.006].
Conclusions: Late Fontan failure is common among adults with single ventricle physiology. Contrary to our hypothesis, RAAS genotype is not associated with increased ventricular mass late after the Fontan operation. However, high-risk genotype is associated with impaired ventricular compliance and neurohormonal activation with higher serum BNP being of prognostic significance in this population.
Author Disclosures: L.J. Burchill: None. A.N. Redington: None. C.K. Silversides: None. H.J. Ross: None. S. Mital: None. L. Jimenez-Juan: None. E.N. Oechslin: None. C. Slorach: None. L. Mertens: None. R.M. Wald: None.
- © 2014 by American Heart Association, Inc.