Severe right ventricular hypertrophy in hypertrophic cardiomyopathy: Serious symptoms, complex surgical procedures, and poor prognosis in Fuwai Hospital

Abstract

Objective: 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 those of 128 patients without SRVH. Results: Patients in the group with SRVH were younger than those in the group without SRVH (27.58±15.09 years vs 40.34±13.21 years, respectively; p<0.001). Patients with SRVH had more serious clinical symptoms and a higher New York Heart Association functional class than those without SRVH. Most patients in the group with SRVH 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 mm Hg vs 42.3±5.7 mm Hg, respectively; p=0.000) were significantly greater in patients with SRVH than in those without SRVH. A total of 30 patients in the group with SRVH underwent surgical correction. During a median follow-up period of 48 months, six patients with SRVH reached primary clinical endpoints (four sudden cardiac deaths, one heart failure-related death, and one heart transplantation), whereas only two deaths occurred in the patients without SRVH. Conclusion:We conclude that patients with HCM and SRVH exhibit serious symptoms and have complex surgical requirements and poor clinical outcomes.

New Genetic finding in a Combination of Long QT Syndrome and Hypertrophic Cardiomyopathy in a Single Patient

“…Hypertrophic cardiomyopathy is electrocardiographically characterised by left ventricular hypertrophy measured by SOKOLOW-LYON-index above 3.5 mV [2] or LEWIS-index above 1.6 mV [3], Cornell criteria above 2,0 mV for females and above 2,8 mV for males [4], Peguero-LoPresti ECG criteria above 2.3 mV for females and 2,8 mV for males [5], right ventricular SOKOLOW-LYON-index [6], signs of biventricular hypertrophy criteria [7] and pathologic Q-waves and T-wave inversions [8] Echocardiographically, HCM is characterised by asymmetrical left and right ventricular hypertrophy and usually with abnormalities of mitral valve with leaflet elongation, chordae and papillary muscle abnormalities enhancing left ventricular outflow tract obstruction as demonstrated in the three patients of this family.…”