Left Ventricular Outflow Tract Obstruction Secondary to a Rhabdomyoma
A1-day-old infant was noted to have a grade II/VI systolic ejection murmur at the base of the heart. An echocardiogram was obtained, which revealed a pedunculated, mobile mass in the left ventricular outflow tract, affixed to the interventricular septum just below the aortic valve (Figures 1 and 2⇓). A 37-mm Hg peak Doppler gradient was detected between the left ventricle and ascending aorta. The patient was thought to be at risk for embolic events or sudden death. On the fifth day of life, the mass was resected through transaortic exposure using cardiopulmonary bypass and cardioplegic arrest (Figure 3). Histological examination showed the tumor to be a rhabdomyoma. The postoperative course was unremarkable, and a follow-up echocardiogram revealed no residual tumor or aortic valve dysfunction.
Although rhabdomyomas are the most common primary cardiac tumors in children, this case is unusual because only a single mass was present. The infant had no signs of tuberous sclerosis, but this condition may appear later in childhood.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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