Abstract
Adults with congenital heart disease represent a rapidly growing patient group. Dysfunction of the right ventricle is often present, and right heart failure constitutes the main cause of death. Heart failure therapies used in acquired left heart failure are often initiated in adults with right heart failure due to congenital heart disease, but the right ventricle differs substantially from the left ventricle, and the clinical evidence for this treatment strategy is lacking. In this review, we identified existing clinical studies evaluating the effects of ACE inhibitors, angiotensin II receptor blockers and aldosterone antagonists in adults with congenital heart disease by a systematic literature search. From 13 identified studies no clear evidence of beneficial effects was found, but the design of the studies limits the validity of the results. The studies in general include low numbers of patients, have short follow-up periods and evaluate surrogate endpoints instead of hard clinical endpoints. Specific evaluation of symptomatic patients with a systemic right ventricle indicates that these patients may benefit from RAAS inhibitory treatments, but this requires further investigation. To conclude, existing studies do not support the use of RAAS inhibitory treatments in right heart failure due to congenital heart disease but contain important limitations. Hence, there is a need for new well-designed trials including higher numbers of patients and validated endpoints to optimize and guide future treatment of this patient group.