ISSN: 1305-385X
Hakkında: Özel sayılar şeklinde yayınlanır.
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Myocardial Noncompaction
Dr. Mehmet ERENa
aKardiyoloji Kliniği, Dr. Siyami Ersek Göğüs Kalp Damar Cerrahi Merkezi, İSTANBUL
Noncompaction of the ventricular myocardium is a rare cardiomyopathy thought to be caused by arrest of normal embryogenesis of the endocardium and myocardium. Both familial and sporadic forms of noncompaction have been reported. This abnormality is often associated with other congenital cardiac defects, but isolated form is also seen in the absence of other cardiac anomalies. Although the most common presentation is with congestive heart failure, clinical manifestations are highly variable. It ranges from no symptoms to disabling congestive heart failure, arrhythmias, and systemic thromboemboli. Echocardiography has been the diagnostic procedure of choice, but the correct diagnosis is often missed or delayed because of lack of knowledge about this uncommon disease and its similarity to other diseases of the myocardium. Antenatal detection is difficult due to the lack of associated cardiac anomalies. Its treatment is that of congestive heart failure, arrhythmias, and thromboembolism. The end-stage heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.Keywords: Myocardial noncompactionTurkiye Klinikleri J Int Med Sci 2007, 3(24):19-25
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