Isolated non-compaction of ventricular myocardium in a victim of the Wenchuan earthquake with crush syndrome and acute renal failure.

Liu F1, Gao FB, Fu P, Qiu HY, Hu HD, Tang H, Zhang L, Song B, Tang WX, Tao Y, Huang SM.

Author information

  • 1Division of Nephrology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

Abstract

Non—compaction of the ventricular myocardium NVM is a rare congenital genetic heart defect that was initially reported 17 years ago by means of autopsyfew cases have been published since thenIt has been classified as the primary inherited cardiomyopathy by the American Heart Association Scientific Statement in 2006 under contemporary definitions and classification of cardiomyopathies. It is caused by an arrested development of muscle fiber compactiona process that normally takes place during early embryogenesiswhich is characterized by numerous sinusoids or trabeculae that are excessive in number and abnormal in prominence and by deep intratrabecular recesses covered by endothelium that exhibits continuity with ventricular endocardiumIt usually involves one andor more segments of the left

Ventricle, while the clinical manifestations are highly variableranging from asymptomatic to congestive heart failurearrhythmiassystemic embolic eventsand sudden cardiac death. The most frequent symptom is heart failure, mainly of the left ventficular origin

 

Chin Med J Engl. 2009 Sep 20;12218:2196-8.

PMID: 19781312 [PubMed - indexed for MEDLINE]

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