The prognostic role of right ventricular dysfunction in patients with hypertrophic cardiomyopathy.

Abstract

Hypertrophic cardiomyopathy (HCM) primarily affects the left ventricle (LV) sparing the right ventricle (RV) in vast majority of cases. However, several studies employing CMR have revealed that myocardial hypertrophy may also involve the RV. To assess RV size and function in a large prospectively cohort of HCM patients and to evaluate whether these parameters in association with other MR findings can predict cardiac events. Two participating centers prospectively included patients with known or suspected HCM between 2011 and 2017. CMR studies were performed with three different scanners. Outcome measures were a composite of ventricular arrhythmias, hospitalization for HF and cardiac death. Of 607 consecutive patients with known or suspected HCM, 315 had complete follow-up information (mean 65 ± 20 months). Among them, 115 patients developed major cardiac events (MACE) during follow-up. At CMR evaluation, patients with events had higher left atrium (LA) diameter (41.5 ± 8 mm vs. 37.17 ± 7.6 mm, p < 0.0001), LV mass (156.7 vs. 144 g, p = 0.005) and myocardial LGE (4.3% vs. 1.9%, p = 0.001). Similarly, patients with events had lower RV stroke volume index (42.7 vs. 47.0, p = 0.0003) and higher prevalence of both RV hypertrophy (16.4% vs. 4.7%, p = 0.0005) and reduced RV ejection fraction (12.2% vs. 4.4%, p = 0.006). In the multivariate analysis, LA diameter and RV stroke volume index were the strongest predictors of events (p < 0.001 and p = 0.0006, respectively). Anatomic and functional RV anomalies detected and characterized with CMR may have may have a major role in predicting the prognosis of HCM patients.