Noncompaction of the ventricular myocardium: report of two cases with bicuspid aortic valve demonstrating poor prognosis and with prominent right ventricular involvement.
Noncompaction of the ventricular myocardium is a rare, unclassified cardiomyopathy due to an arrest of myocardial morphogenesis. The characteristic echocardiographic findings consist of multiple, prominent myocardial trabeculations and deep intertrabecular spaces communicating with the left ventricular (LV) cavity. The disease typically involves the LV myocardium, but right ventricular (RV) involvement is not uncommon. The clinical manifestations include heart failure (HF) signs, ventricular arrhythmias and cardioembolic events. Noncompacted myocardium may occur as an isolated cardiac lesion, as well as it can be in association with congenital anomalies. We describe two illustrative cases of noncompaction of the ventricular myocardium, a 19-year-old male with bicuspid aortic valve and progressive worsening of HF, and a 61-year-old male with marked RV involvement in addition to LV apical involvement, both with the typical clinical and echocardiographic features of the disease.
Cavusoglu
Yuksel
Y
Department of Cardiology, Faculty of Medicine, Osmangazi University, Eskisehir, Turkey. yukselc@ogu.edu.tr
Ata
Necmi
N
Timuralp
Bilgin
B
Gorenek
Bulent
B
Goktekin
Omer
O
Kudaiberdieva
Gulmira
G
Unalir
Ahmet
A
D000328:Adult; D001021:Aortic Valve / Q000002:abnormalities*; D009202:Cardiomyopathies / Q000000981:diagnostic imaging*; D004452:Echocardiography; D006352:Heart Ventricles / Q000000981:diagnostic imaging* / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis; D035583:Rare Diseases / Q000000981:diagnostic imaging; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000473:pathology*