OBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY: A CASE REPORT
Birry Karim1, Ika Prasetya Wijaya1
1. Cardiovascular Division, Internal Medicine Department, Faculty of Medicine University of Indonesia / Cipto Mangunkusumo National Hospital
Introduction: Hypertrophic cardiomyopathy (HCM) is the most common genetic disease
of the heart. Its prevalence is estimated in 1 per 500 individuals. The disorder has a variable presentation and carries a high incidence of sudden death in young athletes. Case Illustration: A 28-year-old male patient was referred to our outpatient clinic with a previous diagnosis of coronary arterial disease suspicion. He reported no significant symptom or history. Repeated electrocardiographs had been performed and showed T wave inversion in lead I, aVL, V2-V6. At our clinic, we found no abnormality from the physical examination. An echocardiography was performed and demonstrated asymmetric hypertrophy of the interventricular septum, with systolic anterior motion of the mitral valve and severe diastolic dysfunction, indicating a significant left ventricular outflow tract obstruction. A cor angiography showed only myocardial bridging of the left anterior descending artery and there was no stenosis detected. Discussion: HCM is inherited in an autosomal dominant manner. Many patients with hypertrophic cardiomyopathy are asymptomatic or mildly symptomatic. Our patient has no abnormal sign and symptom and no history of sudden cardiac death in the family. Common ECG findings include ST-T wave abnormalities, left atrial enlargement, and LV hypertrophy. Obstructive hypertrophic cardiomyopathy is due to mid systolic obstruction of flow through the LV outflow tract. The hallmarks of the obstructive HCM consist of systolic anterior motion of the anterior mitral valve leaflet, septal wall thickness of > 15 mm, and asymmetrical septal hypertrophy with a ratio of septal wall thickness to posterior wall thickness of greater than 1.4 : 1. Continuous wave Doppler reveals an elevated flow velocity across the LV outflow tract. A cardiac catheterization is useful to determine the degree of outflow obstruction, cardiac hemodynamics, the diastolic characteristics of the LV and LV anatomy, and coronary anatomy. Transcatheter septal alcohol ablation is performed to relieve the LV outflow obstruction by intentional infarction of a portion of the interventricular septum. Conclusion: HCM may have variable presentation and may be asymptomatic. The obstruction of LV outflow tract is a prevalent characteristic which may influence clinical outcome patterns and mortality rates. Although it detectable in the routine evaluation, it requires detailed, static and dynamic investigation by imaging methods in order to improve the diagnosis and the management. Key words: Obstructive hypertrophic cardiomyopathy