Combination percutaneous and medical management of cardiac rhabdomyomas obstructing tricuspid valve inflow: Case report

Knadler, J.J., *Lawrence, E., Iacobas, I., Justino, H., Sheth, S. | JNPM 2021;


BACKGROUND:Cardiac rhabdomyomas can be prenatally diagnosed in patients with tuberous sclerosis complex. Many neonates require no intervention in early life other than close monitoring for regression of tumor over the period of months to years. In rare instances, cardiac rhabdomyomas can result in obstruction to blood flow or decreased ventricular function.

CASE REPORT:We describe the case of a neonate who was prenatally diagnosed with multiple large cardiac rhabdomyomas, one of which caused clinically significant obstruction to prograde blood flow across the tricuspid valve in the newborn period. To address the disturbance to prograde pulmonary blood flow, the patient underwent successful ductal stent placement in the neonatal period. A troponin elevation was noted shortly after birth, but no evidence of coronary compression or involvement was demonstrated by coronary angiography. The patient has subsequently been treated with sirolimus over a period of three months, with noted regression in tumors and improvement in tricuspid valve inflow.

CONCLUSIONS:A brief review of the literature regarding the diagnosis, treatment, and management of neonatal patients with cardiac rhabdomyomas is presented. A combined percutaneous and medical management approach may be of benefit in future cases of rhabdomyomas causing obstruction to pulmonary blood flow.

*Corresponding Author: 

 Joseph J. Knadler, MD, Lillie Frank Abercrombie Section of Cardiology, Department of Pediatrics, Texas Children’s Hospital, Baylor College of Medicine, 6651 Main Street, Legacy Tower, E1920, Houston, Texas 77030, USA. Tel.: +1 615 734 9430; fax: +1 832 826 4297 Email: