Abstract 12644: Clinical Features and Outcomes by Systemic Ventricle Morphology in Adult Fontan Patients

Abstract

Introduction: Single ventricle physiology is commonly encountered in patients after Fontan palliation. However, limited data exists on the differences in clinical characteristics and long-term outcomes in adults with systemic left ventricular morphology (SLV) compared to systemic right ventricular morphology (SRV). Methods: Retrospective review of medical records from a tertiary medical center for 2010-2018 identified 191 Fontan patients ≥ 18 years of age for whom ventricular morphology data was available. Results: Of 191 patients, 62 (32%) had SRV and 129 (68%) had SLV. Mean age was 28.9 ± 9.1 years and mean age at the time of Fontan palliation was 6.4 ± 6.7 years. Patients with SLV were older (30.9 ± 9.6 years vs. 24.7 ± 6.0 years) and more likely to have classic atriopulmonary connection (17% vs. 2%). Differences in adverse outcomes by ventricular morphology are shown in the figure. After adjusting for age, patients with SRV were more likely to have atrioventricular valve regurgitation (adjusted odds ratio 3.42, 95% confidence interval 1.56-7.52, p=0.002). There were no significant age-adjusted differences between SRV and SLV patients for NYHA class, arrhythmia/pacemaker placement/implantable cardioverter-defibrillator (ICD) placement, heart failure hospitalization, thromboembolic complications, cirrhosis, protein-losing enteropathy, Fontan revision, or death. Data from cardiac catheterization, cardiopulmonary exercise testing, and liver function tests showed no significant differences. Conclusion: Adverse outcomes among adult recipients of the Fontan procedure did not differ significantly based on ventricular morphology. Further multicenter studies will be necessary to understand finer differences in SRV and SLV pathophysiology and eventually the impact of ventricular modeling on long term outcomes as the adult Fontan population continues to age.