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(Circulation. 2007;115:e395-e397.)
© 2007 American Heart Association, Inc.

Images in Cardiovascular Medicine

Neonatal Tuberous Sclerosis and Multiple Cardiac Arrhythmias

Yaniv Bar-Cohen, MD; Michael J. Silka, MD; Mark S. Sklansky, MD

From the Division of Cardiology, Department of Pediatrics, Childrens Hospital Los Angeles, University of Southern California, Los Angeles.

Correspondence to Yaniv Bar-Cohen, MD, Childrens Hospital Los Angeles, 4650 Sunset Boulevard, Mail Stop #34, Los Angeles, CA 90027. E-mail ybarcohen@chla.usc.edu

An extract of the first 250 words of the full text is provided, because this article has no abstract.

An infant female was born at term with a presumptive diagnosis of tuberous sclerosis. Fetal echocardiograms had demonstrated multiple cardiac masses suggestive of rhabdomyomas (Figures 1 and 2), and a fetal magnetic resonance image suggested the presence of brain lesions. An urgent Cesarean section was performed at term due to an irregular tachycardia. Postnatal echocardiograms demonstrated multiple cardiac masses, with large lesions along the lateral wall of the right atrium, the right ventricular apex and at the atrioventricular septum (Figure 3). Intracavitary obstruction was not apparent, but a smaller rhabdomyoma seen under the pulmonary valve caused mild right ventricular outflow tract obstruction. A cranial ultrasound demonstrated multiple echogenic foci, confirming the diagnosis of tuberous sclerosis, and a subsequent computed tomography scan of the brain delineated these lesions more clearly (Figure 4).

View larger version (159K):   Figure 1. Echocardiographic fetal four-chamber view of the heart at 27 weeks gestation. Multiple homogenous lesions are seen, including a right atrial mass (1), a lesion at the ventricular septum (2), and a right ventricular apex mass (3). LA indicates left atrium; LV, left ventricle; RA, right atrium; and RV, right ventricle.

View larger version (49K):   Figure 2. Three-dimensional rendered echocardiographic image of the four-chamber view of the fetal heart at 20 weeks gestation demonstrating the same masses.

View larger version (97K):   Figure 3. Postnatal echocardiograms more clearly reveal the rhabdomyomas. This four chamber view was obtained on the 10th day of life and demonstrates the right atrial mass (1), measuring approximately 17 mm x 15 mm; the ventricular septal lesion . . . [Full Text of this Article]