Abstract
Introduction: Adult Congenital Heart Disease (ACHD) patients have increased mortality and morbidity from heart failure. Angiotensin receptor-Neprilysin Inhibitors (ARNI) have emerged as a standard of therapy for adults with heart failure. These medicines have not been studied in ACHD patients. Hypothesis: ARNIs are associated with subjective and objective improvements in systemic right ventricle (SRV) patients. Methods: Eighteen SRV patients were prescribed ARNI at our institution in the last 3 years. Data including demographics, medical history, New York Heart Association functional class (NYHA FC), labs, 3D imaging, echocardiograms, cardiopulmonary stress test (CPET), and hospitalization were obtained by retrospective electronic chart review. Statistical analysis was performed using paired t and Wilcoxon rank sum tests. Results: Eighteen SRV patients (mean age 40 years, 72% male) were treated with ARNI (median 13 months) and conventional therapy (beta blocker (83%), loop diuretic (89%), antiarrhythmics (33%), and anticoagulation (50%)). Baseline data include: Mean blood pressure 118/75 mmHg, pulse 75 bpm, creatinine 0.86 mg/dL, potassium 4 meq/L, B-type natriuretic peptide (BNP) 322 pg/mL, and NT-Pro BNP 1091 pg/mL. Nine patients were NYHA FC 2, 7 FC 3, and 2 FC 4. High dose was achieved in 3 (17%) patients, and moderate in 3 (17%). CPET and imaging data were: VO2 18 mL/kg/min, VE/VCO2 38, RVEF 32%, FAC 21%, significant TR (12.5% moderate, and 12.5% severe) and TAPSE 9.4. There was no statistically significant difference pre vs post-ARNI in blood pressure, labs (creatinine, potassium, BNP and NT-ProBNP), or testing (VO2 18 vs 17, p=0.432; VEVCO2 38 vs 39, p=0.850; RVEF 32 vs 32%, p=0.648; FAC 18 vs 21%, p=0.489; TR p=0.317; and TAPSE 9.2 vs 9.1, p=0.906). There was a statistically significant improvement in median NYHA FC (2 vs 2.5, p=0.005), especially if baseline NYHA FC>2 (2 vs 3, p=0.011). There was a noted decrease in cardiac hospitalization (4 vs 9) that did not reach statistical significance (p=0.313). Conclusions: ARNIs are associated with noticeable improvement in functional class without impact on objective measures in SRV patients. The study findings are promising and highlight the need for larger, prospective, multicenter studies.
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