[Left ventricular noncompaction: a cardiomyopathy in young individuals. Description of first cases in Africa]. / La non compaction du ventricule gauche, une cardiomyopathie du sujet jeune: premieres observations Africaines.

ABSTRACT

Left ventricular noncompaction (LVNC) is a recently identified and probably rare congenital cardiomyopathy characterized by changes in the structure of the myocardium secondary to incomplete embryogenesis. The purpose of this report is to describe three cases of LVNC involving African patients. To our knowledge these are the first cases described in Africa. All three patients in this series were men from sub-Saharan Africa ranging in age from 23 to 45 years. The first patient in whom cardiomegaly was recognized on a routine chest x-ray was asymptomatic. The second who presented with exertional dyspnea developed left bundle branch block. The third was admitted to the hospital for acute pulmonary edema. In all three cases transthoracic echocardiography suggested diagnosis. The left ventricle was dilated and hypokinetic and the myocardium exhibited a spongy aspect in association with the presence of prominent trabeculations separated by crypts located at the apex and lateral wall. Color Doppler demonstrated that intratrabecular recesses were filled by intraventricular blood flow. Magnetic resonance imaging (MRI) specifically confirmed this morphological feature. Sudden death due to arrhythmia, cardiac insufficiency, and systemic emboli are the main complications of LVNC. The incidence of LVNC, which is certainly underestimated, is highest in young adults but it can be diagnosed at any age. Echocardiography and MRI are effective tools for detection of the morphologic diagnostic criteria. Recent evidence suggests that LVNC is of genetic origin and the data reported here shows that the underlying mutations are present in sub-Saharan populations. Family screening in African populations is still difficult. Therapeutic management is currently based on symptomatic treatment of cardiac insufficiency and can require techniques not readily available in tropical settings.