PrenatDiagn 2008; 28 : 551–553.
Published online in Wiley InterScience
(www.interscience.wiley.com) DOI: 10.1002/pd.2014
RESEARCH LETTER: Right ventricular noncompaction associated with long QT in a fetus with right ventricular hypertrophy and cardiac arrhythmias
Ruben J Acherman, MD, William N Evans, MD, Joel K Schwartz, MD, Michelle Dombrowski, MD, Robert C Rollins, MD, William Castillo, MD, Sri Haltore, MD, Dean P Berthody, MD, FABR
ChildrenŐs Heart Center Nevada, 3006 S Maryland Pkwy, Ste 690, Las Vegas, Nevada 89109, USA. E-mail: IAcherman@aol.com.
Myocardial noncompaction, a disorder of the embryonic endomyocardial morphogenesis, is usually sporadic but may be familial (Koh et al., 2004). The prevalence in the general population is estimated at 0.05% (Espinola-Zavaleta et al., 2006). In up to 26% of cases, noncompaction is associated with structural congenital cardiac abnormalities. Studies in adult patients show noncompaction limited to the left ventricle in 62% and affecting both ventricles in 38% (Espinola-Zavaleta et al., 2006). Isolated right ventricular noncompaction is rare (Hruda et al., 2005; Espinola-Zavaleta et al., 2006) Myocardial noncompaction is best detected by echocardiography. Echocardiographic abnormalities include prominent ventricular trabeculae with deep intertrabecular recesses and the presence of blood flow filling the recesses visualized on color-Doppler imaging (Jenni et al., 2001). Systolic and diastolic functions may be abnormal. Therapy includes medical management of heart failure and arrhythmias, anticoagulation, and the use of pacemakers and implantable defibrillators. Cardiac transplantation is indicated for refractory congestive heart failure.