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Ventricular non-compaction cardiomyopathy: CMR and pathology findings
  1. S Pujadas,
  2. R Bordes,
  3. A Bayes-Genis

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A 39 year-old woman was diagnosed by non-invasive imaging techniques as having isolated ventricular non-compaction cardiomyopathy with severe left ventricular dysfunction. Cardiovascular magnetic resonance (CMR) study showed manifest apical trabeculation with deep intertrabecular recesses and thinning of the myocardial wall, characteristic of non-compaction cardiomyopathy (panel A). Contrast enhanced CMR images showed confluent areas of gadolinium enhancement in the interventricular septum, suggesting the presence of severe myocardial fibrosis in this territory (panel B). Six months later she underwent successful cardiac transplantation. Pathological findings identified abundant extracellular matrix within the myocardium and myocardial fibre disarray (panels C and D).

To our knowledge, this is the first report of a case of non-compaction cardiomyopathy which showed a correlation between myocardial late enhancement by CRM and histopathologically proven myocardial fibrosis.

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(A) Cardiovascular magnetic resonance (CMR) (balanced FFE sequence) showing apical trabeculation with deep intertrabecular recesses (arrows). (B) Contrast enhanced CMR showed confluent areas of gadolinium enhancement in interventricular septum (arrows). (C) Macroscopic examination of the heart revealed a yellowish area of interventricular septum suggestive of myocardial fibrosis, in agreement with gadolinium enhancement findings (arrows). (D) Microscopic examination (Masson’s trichrome) showed the characteristic findings of non-compaction cardiomyopathy: abundant extracellular matrix (asterisk) intermingled with myocardial fibre disarray and severe hyperplasia of vascular media (arrowheads). LV, left ventricle; RV, right ventricle.