Author(s): ValBernal JF, Nistal JF, Martino M, Garijo MF
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Abstract Isolated left ventricular non-compaction is a rare unclassified cardiomyopathy characterized by arrest of normal embryogenesis that results in the persistence of intertrabecular recesses and the development of a spongy myocardium. The pathological diagnosis is often first established at autopsy. Described herein is the case of a 57-year-old man with isolated non-compaction of the left ventricle who had a 17 year history of worsening heart failure and was successfully treated with heart transplantation. To the authors' knowledge only seven adult patients with non-compaction cardiomyopathy have been reported to have undergone heart transplantation. Including the present case the mean age of the adult patients at transplantation was 39.5 years (range, 18-60 years). The male : female ratio was 3:1. Both ventricles were involved in 37.5\% of cases. The mean follow up was 6.3 years (range, 2 months-17 years). One patient died because of a malignant tumor 9 years after transplantation. The morphological pattern of the isolated ventricular non-compaction represents a pathological entity discernible from other cardiomyopathies and should be classified as a specific cardiomyopathy. It is likely that surgical pathologists will encounter this entity more frequently due to involvement in transplantation teams.
This article was published in Pathol Int
and referenced in Journal of Clinical Case Reports