Obstructive Hypertrophic Cardiomyopathy with Concomitant Mitral Regurgitation Treated with a Septal Myectomy and MV Repair: A Case Report

Case presentation

Hypertrophic cardiomyopathy (HCM) is the most common and very heterogeneous genetic cardiac disease with a different clinical presentation and prognosis. The overall prevalence of the disease is estimated between 0.05-0.2% of the population. Left ventricular outflow obstruction at rest is present in about 20% of patients. Most of the patients have a normal life expectancy, however high risk patients might develop heart failure, atrial fibrillation, ventricular arrhythmias and sudden cardiac death.

We present the case of 47-year-old Caucasian man who was hospitalized at our clinic with a history of chest pain and shortness of breath on physical activity in the last six months, which caused significant limitations of his life quality. Hypertrophic obstructive cardiomyopathy was diagnosed in 2011, when the patient was put on therapy with beta blocker. Transthoracic echocardiography revealed normal systolic function, presence of systolic anterior mitral valve motion (SAM) with moderate mitral regurgitation (MR). There was a significant concentric left ventricular hypertrophy predominantly located in the ventricular septum. The intraventricular gradient at rest was 77.8 mmHg. MRI of the heart confirmed significant LV hypertrophy with regions of fibrosis at the septum. The patient shortness of breath worsened progressively in the last month (NYHA III) despite optimized medical treatment with maximal beta blocker dose. Surgical approach with septal myectomy was performed with mitral valve repair. There were no operative complications, with excellent postoperative recovery and complete symptoms resolution. Control Doppler echocardiograms revealed LVOT rest gradient reduction to 34 mmHg. The good operative results were still present 9 months after the intervention.

Our case confirmed that septal myectomy with MV repair is an excellent treatment approach in young patient with obstructive hypertrophic cardiomyopathy and mitral valve involvement refractory to medical treatment.