Abstract
BACKGROUND: Progressive systemic right ventricle (sRV) dysfunction is a significant challenge in adult congenital heart disease. Current guidelines do not specify effective heart failure medications for patients with sRV; however, previous studies have relied on semiquantitative assessments. The advancement of cardiac magnetic resonance (CMR) imaging as the gold-standard modality offers quantitatively accurate assessments even for complex cardiac anomalies. Therefore, we aimed to investigate prognostic factors associated with sRV dysfunction in patients on angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACE-I/ARB), using CMR-derived quantitative values. METHODS: We conducted a retrospective cohort study of 17 adult patients with sRV treated with ACE-I/ARB and performed logistic regression analysis, with the primary outcome defined as sRV ejection fraction (sRVEF) deterioration. RESULTS: Over an average follow-up period of 68.7 months, sRVEF deterioration occurred in 3 patients (17%). Logistic regression analysis identified tricuspid regurgitation (TR) as a potential independent prognostic factor for the primary outcome (odds ratio = 1.11; 95% confidence interval, 1.00-1.31). Furthermore, patients with mild TR (TR fraction ≤15%; N = 12) experienced improvements in sRVEF between the initial and last CMR assessments (from 49.1% ± 8.4% to 56.7% ± 8.0%, P = 0.0029), with increased stroke volume from 68.2 ± 18.6 to 79.5 ± 17.2 mL (P = 0.0029). In contrast, these changes were not observed in patients with moderate or severe TR (TR fraction >16%) (N = 5). CONCLUSIONS: Our CMR-based evaluation highlights the potential utility of TR stratification in predicting the changes in sRVEF among patients with sRV on ACE-I/ARB. Future randomized controlled trials that consider TR severity are required to elucidate the significance of ACE-I/ARB therapy.