摘要:
Isolated noncompaction of the ventricular myocardium (INVM) is a rare congenital heart disease that results from an abnormal arrest in endomyocardial embryogenesis. It is characterized by the presence of prominent ventricular myocardial trabeculations and deep intertrabecular recesses, in the absence of other structural heart defects. INVM was initially described in the pediatric population (Chin et al, 1990), but it has also been recognized more recently in adults (Oechslin et al, 2000; Ritter et al, 1997). We present a case of a 35-year-old woman with clinical and echocardiographic features of INVM who underwent cardiac transplantation in our institution. The literature on cardiac embryogenesis and development, with an emphasis on the different genetic factors that lead to noncompaction of the ventricular myocardium, is also reviewed.