Introduction: Severe right ventricular hypertrophy (SRVH) in hypertrophic cardiomyopathy (HCM) is rare. We studied the clinical characteristics and prognosis of 36 patients with HCM and SRVH in a Chinese cohort.
Methods: Patients with HCM and SRVH were enrolled between 2013 and 2017. The clinical characteristics, treatment therapies, and clinical outcomes of the 36 patients were retrospectively studied and compared with 128 patients without SRVH.
Results: Patients in the SRVH group were younger compared with patients in the non-SRVH group (27.58 ± 15.09 years vs. 40.34 ± 13.21 years, respectively; p＜0.001). SRVH group had more serious clinical symptoms and a higher New York Heart Association (NYHA) functional class than non-SRVH group. Most patients in the SRVH group exhibited diffuse RV hypertrophy and 13 patients presented with biventricular outflow tract obstruction. Maximal left ventricular (LV) wall thickness (27.29 ± 7.95 mm vs. 24.33 ± 5.85 mm, respectively; p = 0.027) and LV outflow tract gradient (80.83 ± 24.41 mmHg vs. 42.3 ± 5.7 mmHg, respectively; p = 0.000) were significantly greater in the SRVH group compared with the non-SRVH group. A total of 30 patients in the SRVH group underwent surgical correction. During a median follow-up period of 48 months, 6 patients with SRVH reached primary clinical endpoints (4 sudden cardiac deaths, 1 heart failure-related death, and 1 heart transplantation), while only 2 deaths occurred in the non-SRVH group,.
Conclusion: We conclude that patients with HCM and SRVH exhibit serious symptoms and have complex surgical requirements and poor clinical outcomes.