Non compaction cardiomyopathy: a series of 15 cases

• Published in: Revista medíca de Chile 2011-07, Vol.139 (7), p.864-871
• Main Authors: Enríquez R, Andrés, Baeza V, Ricardo, Gabrielli N, Luigi, Córdova A, Samuel, Castro G, Pablo
• Format: Article
• Language: Spanish
• Subjects: Adolescent; Adult; Aged; Echocardiography; Female; Follow-Up Studies; Humans; Isolated Noncompaction of the Ventricular Myocardium - complications; Isolated Noncompaction of the Ventricular Myocardium - diagnosis; Magnetic Resonance Imaging; Male; Middle Aged; Rare Diseases - diagnosis; Retrospective Studies; Young Adult
• ISSN: 0717-6163

Non compaction cardiomyopathy is a rare disorder caused by the arrest of myocardial compaction during embryogenesis, leading to a non compacted endocardial layer with marked hypertrabeculation and deep recesses. To report the clinical and echocardiographic characteristics of a series of 15 adult patients with non-compaction cardiomyopathy. We included a total of 15 patients aged 52 ± 17 years (40% males) diagnosed at our echocardiography laboratory between January 2001 and July 2010. The form of presentation was heart failure in 53% of subjects, syncope in 20%o, ventricular arrhythmias in 13%o and stroke in 7%>. Left ventricular end-diastolic diameter was 66 ± 11 mm and estimated ejection fraction was 27 ± 10%>. Apical and/or mid-ventricular segments of the left ventricle were involved in all the cases. Pulmonary hypertension was present in 40%o. 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%o presented sustained ventricular arrhythmias and 20%> had atrial fibrillation or flutter, whereas 13%o had cerebral embolic events. An automated internal cardioverter defibrillator was implanted in 47%o of patients. 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.