Miocardiopatía no compactada: Una serie de 15 casos

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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 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.

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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

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