Cardiac Rhabdomyoma in a Neonate.

Abstract

Tuberous sclerosis complex has an autosomal dominant pattern of inheritance. Hamartomas are seen in various organs in tuberous sclerosis. Tuberous sclerosis is linked to cardiac rhabdomyomas, which is one of the common heart tumors in children. We present a full-term neonate who was suspected to have the congenital disease due to the presence of pansystolic murmur on examination. Echocardiography revealed multiple rhabdomyomas in the right ventricular (RV) and LV wall and interventricular septum of varying sizes and shapes. The largest rhabdomyoma arising from the lateral wall of RV measuring 16 mm × 18 mm. There was 6 mm × 8 mm rhabdomyoma protruding in the left ventricular outflow tract in nonobstructive manner. There was no inflow or outflow obstruction. Small apical muscular ventricular septal defect noted. Mother had numerous papulonodular lesions on both cheeks, nose, and forehead (adenoma sebaceum). No other neurocutaneous markers were present. Mother had no systemic involvement. In babies born to mothers with suspected tuberous sclerosis, a high degree of suspicion is required to rule out cardiac tumors.