Symptomatic Obstructive Hypertrophic Cardiomyopathy

Abstract

Hypertrophic cardiomyopathy (HCM) is a common, yet challenging condition for clinical management. The majority of these patients have evidence of left ventricular outflow tract (LVOT) obstruction that may be asymptomatic or associated with debilitating symptoms of heart failure. In this report, we discuss the clinical presentation, appropriate evaluation, and management of a patient with symptomatic obstructive HCM. A 65-year-old woman presents to the ambulatory clinic for evaluation and management of dyspnea. The patient has a lifelong history of a murmur but was never limited in her activities until ≈8 months ago. Her symptoms consist of dyspnea that occurs when climbing a flight of stairs and while gardening. These activities can also precipitate lightheadedness, although she has never had frank syncope. The patient’s medical history is significant for medically treated hypertension (metoprolol succinate, 100 mg/d); there is no history of coronary or peripheral atherosclerosis. She also has a 42-year-old son, who was recently diagnosed with obstructive HCM during a routine medical examination. On physical examination, the patient is relatively small in stature (5′7″; 55 kg). Her blood pressure is 120/70 mm Hg with a heart rate of 60 bpm. Her lungs are clear to auscultation. The jugular venous pulse is normal. Carotid upstroke is brisk and not bifid. Her left ventricular apex is localized and slightly sustained (1+). No murmur is present at rest. However, with squat-to-stand maneuver, there is a 2/6 systolic ejection murmur heard best at the left lower sternal border. The remainder of the physical examination is unremarkable. An ECG demonstrates normal sinus rhythm and voltage criteria for left ventricular hypertrophy. A chest radiograph is normal. The patient undergoes a transthoracic echocardiogram (Figure 1). Left ventricular size is normal, and systolic function is preserved (ejection fraction, 65%). There is moderate myocardial hypertrophy localized to the ventricular septum (maximal thickness, …