Non compaction cardiomyopathy: a series of 15 cases

Abstract

Background: Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a noncompacted endocardial layer with marked hypertrabeculation and deep recesses. Aim: To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. Patients and Methods: We included a total of 15 patients aged 52 +/- 17 years (40% males) diagnosed at our echocardiography laboratory between January 2001 and July 2010. Results: The form of presentation was heart failure in 53% of subjects, syncope in 20%, ventricular arrhythmias in 13% and stroke in 7%. Left ventricular end-diastolic diameter was 66 +/- 11 mm and estimated ejection fraction was 27 +/- 10%. Apical and/or midventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40%. The average follow-up was 19 months and no patient died during this period. Sixty seven percent of the patients had manifestations of heart failure, 27% presented sustained ventricular arrhythmias and 20% had atrial fibrillation or flutter, whereas 13% had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47% of patients. Conclusions: Non-compaction cardiomyopathy is associated with high cardiovascular morbidity. The diagnosis is made in advanced stages of the disease, with significant dilation and ventricular dysfunction. (Rev Med Chile 2011; 139: 864-871).