Abstract

MARTINEZ-MORALES, María Cecilia et al. Multiple intracardiac rhabdomyomas in fetal life mimicking tricuspid atresia at birth. Perinatol. Reprod. Hum. [online]. 2022, vol.36, n.2, pp.51-55.  Epub Dec 01, 2022. ISSN 2524-1710.  https://doi.org/10.24875/per.22000012.

Rhabdomyoma is the most common primary intracardiac tumor in children. We present the case of a patient diagnosed with multiple intracardiac rhabdomyomas during fetal life and with persistent cyanosis at birth. Echocardiographically, tumors were detected in the right atrium with protrusion towards the right ventricle, obstructing the flow of the tricuspid valve. The systemic flow depended on the foramen ovale (short circuit from right to left) and the pulmonary artery from the ductus arteriosus (short circuit from left to right), resembling tricuspid atresia. Treatment started with sirolimus and prostaglandins and an attempt to place a stent in the ductus arteriosus, without success. Resection of the rhabdomyoma obstructing the tricuspid valve was performed. During surgery, the patient presented supraventricular tachycardia and died of post-cardiotomy heart failure. Although most rhabdomyomas regress spontaneously or respond to management with mTOR inhibitors, hemodynamic compromise indicates surgical management.

Keywords : Cardiac rhabdomyoma; Newborn; Prenatal diagnosis; Tricuspid atresia.

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