Noncompaction of the Myocardium, A Rare Cardiomyopathy: A case report

Noncompaction of the myoc ardiuın (NM) is a rare cardiomyopathy due to an arrest of in trauterine ın yocardial morphogenesis. The c haracteris tic echocardiographic findings are multiple, proıninent myocardial trabeculations and deep intertrabecular recesses. The clinic manifestations ineJude heart failure, arrhythmias and embolic events. It has been deseribed in association with presence of some genetic abnorınal ities . We deseribe a case of NM with bicuspid aorta in a 19-year-old male with typical elinical and echocardiographic fea tures of the disease. Clinically, the patient had s igns of progressive worsening of heart failure. ECG demostrated left anterior hemiblock. Cardiomegaly was found in teleradiography. Echocardiography revealed a markedly dilated left ventricle with severely impaired systolic function (ejection fraction; 0.27), characteristic, multiple, prominent trabeculations in the left ventricular apex, bicuspid aorta and moderate degrees of aortic regurgitation. Despite aggressive medical treatment, patient died on the 14th day of hospitalization. This is the first reported case of NM in Turkey, and it is considered as cardiomyopathy.