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 fi brillation or fl utter, whereas 13% had cerebral embolic events. An automated internal cardioverter defi brillator 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 signifi cant dilation and ventricular dysfunction.
Andrés Enríquez, P., Ricardo Baeza, V., Luigi Gabrielli, N., Samuel Córdova, A., & Pablo Castro, G. (2011). Miocardiopatía no compactada: Una serie de 15 casos. Revista Medica de Chile, 139(7), 864–871. https://doi.org/10.4067/S0034-98872011000700006