Heart failure in adult with congenital heart disease is an important cause of morbi-mortality. Sacubitril/Valsartan has been validated for the treatment of heart failure in adults with left ventricular dysfunction.

The aim of this study was to assess safety and efficacy of Sacubitril/Valsartan in symptomatic adults with systemic right ventricle dysfunction.

This is a retrospective and single center study (Quebec Heart and Lung Institute, Quebec City, Canada).

Eight patients (mean age 41±9.2years, 5 males) with a significant systemic right ventricle dysfunction in New York functional class II–III despite optimal heart failure medication were included. Sacubitril/Valsartan was up titrated at the maximal dosage tolerated. The mean dosage reached was 85±22mg/90±24mg. During a median follow-up of 248 (213–430) days, systolic (121±13 vs. 116±13mmHg, P=0.238) and diastolic (76±6mmHg vs. 72±5mmHg, P=0.105) blood pressure, potassium (4.1±0.4mmol/L vs. 3.9±0.2mmol/L, P=0.111) and creatinine (71.7±14.5μmol/L vs. 68.9±14.7μmol/L, P=0.516) levels remained stable. No discontinuation, reduction in dose or side effects were noted. A significant improvement in functional status was observed (P=0.03): 6 patients had a New York Heart Association class improvement and 2 remained in the same class (Fig. 1). Maximal oxygen uptake, NTproBNP levels and echocardiographic data did not reach significant improvement.

Sacubitril/Valsartan titration in a population of patients with congenital heart disease and significant right ventricle dysfunction was well tolerated and associated with significant improvement in functional status class. Whether Sacubitril/Valsartan improved right ventricle remodeling or decrease heart failure hospitalization or mortality in that population remained to determined.