Clinical Presentation and Echocardiographic Characteristics of Patients with Left Ventricular Noncompaction

BackgroundLeft ventricular noncompaction is a primary genetic cardiomyopathy caused by arrestof normal embryogenesis of the endocardium and myocardium. This anomaly isfrequently associated with arrhythmias, heart failure and thromboembolic events.ObjetivesThe goal of the present study was to describe the clinical presentation and the electrocardiographicand echocardiographic characteristics of patients with this cardiomyopathy.MethodsTwenty-two patients with left ventricular noncompaction detected by echocardiographybetween July 2004 and April 2009 were analyzed. Electrocardiogram andtransthoracic Doppler echocardiography was performed to all the patients, and 12patients underwent 24-hour Holter monitoring. Weight, height and body mass indexwere calculated and compared with 66 patients distributed into three groups of 22patients each: 1) control group without heart disease, 2) hypertrophic cardiomyopathy;and 3) idiopathic dilated cardiomyopathy.Results and ConclusionsIn the population with left ventricular noncompaction, female gender prevailed,lower weight and body mass index was observed and dyspnea was the most commonsymptom. The electrocardiogram showed sinus rhythm, conduction disturbancesand repetitive ventricular arrhythmia. Transthoracic echocardiography showed differentdegrees of systolic and diastolic left ventricular dysfunction with areas of noncompactionin the mid and apical inferior, posterior and lateral segments, in somecases complicated with intraventricular thrombi and occasionally associated withcoronary artery fistulas.