Right ventricular dysfunction in hypertrophic cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is one of the primary diseases affecting the cardiac muscle and is characterized by heterogeneous genetic, morphological, functional and clinical features. It is the most common genetic heart disease, with an estimated prevalence at 0.2%. Structural abnormalities in the RV are present in a great proportion of patient with HCM. With advancements of echocardiography, and improved diagnosis the percentage of RV hypertrophy (RVH) in HCM is augmented to 53%. The main common finding in this situation is the mild ventricular obstruction. There are no specific symptoms related to the RV dysfunction in HCM. There is no demonstrated correlation between the sites and the severity of the LV obstruction and the clinical symptoms. Therefore, the development of the heart failure signs and symptoms, ventricular and/or supraventricular arrhythmias or sudden deaths are most often impossible to predict. There is no specific medical treatment for RV dysfunction in HCM. In absence of the significant gradient in the LV outflow, the patients are treated with "normal" therapy of HCM, consisting in beta-blockers and calcium-channel blockers. In case of significant RV gradient, surgical intervention has been reported. The standard surgical approach consist in myectomy (Morrow procedure) and relief of the RV gradient by resection and patch enlargement. The results are very good, symptoms are improved at intermediate-term follow- up and sudden deaths are rare.