Isolated non-compaction of the left ventricular myocardium in an adult treated with heart transplantation.
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.
Val-Bernal
J Fernando
JF
Department of Anatomical Pathology, Marques de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain. apavbj@humv.es
Nistal
J Francisco
JF
Martino
María
M
Garijo
M Francisca
MF
D000328:Adult; D009202:Cardiomyopathies / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D005355:Fibrosis / Q000209:etiology / Q000473:pathology; D016027:Heart Transplantation; D006352:Heart Ventricles; D006801:Humans; D008297:Male; D016896:Treatment Outcome