The fetal cardiac rhabdomyoma

Introduction:

The prevalence of primary tumors of the heart is about 0.01%. The majority are benign. Rhabdomyomas are the most common of begnin pediatric cardiac tumors.

Methods:

We reported a case of multiple fetal cardiac rhabdomyoma, spontaneously regressed in post-natal period without complicating the pregnancy and the well-being of the neonate.

Results:

A 35 year old primigravid patient referred to our center at 25 weeks of gestation for intracardiac echogenic masse. Fetal echocardiography revealed homogenous, solid, echogenic masse in the left ventricle. The fetal heart rhythm was normal and there was no evidence of pericardial effusion or ascites. Fetal aortic and umbilical artery Doppler studies were appropriate for the gestational age. Close follow-up of the fetus performed until the delivery and serial echocardiografies revealed that the rhabdomyoma persisted, remaining the same size and there was any signs of cardiac disorder.

The patient was delivered by caesarean section at 38 weeks and during the postnatal period, masses tended to regress without any complications and close follow up is going on for the baby.

Discussion:

Cardiac rhabdomyoma is the most common primary pediatric tumor of the heart and is considered to be a hamartoma of developing cardiac myocytes. The in-utero sonographic diagnosis of cardiac rhabdomyoma has been well documented. The typical appearance of the rhabdomyoma is an intracardiac echogenic mass, most commonly arising in either ventricle or from the interventricular septum. Detection of intracardiac masses is facilitated by routine inclusion of the four-chamber heart view. Rhabdomyomas as described by Grebend and al are firm, white, well-circumscribed, lobulated nodules that occur in any location in the heart but are more common in the ventricles. They are myocardial (intramural) lesions. When multiple they can manifest as numerous miliary nodules measuring less than 1 mm in diameter (rhabdomyomatosis). Cardiac rhabdomyomas may have detrimental effects on the fetus or newborn from a cardiovascular standpoint as there is a risk of arrhythmias, outflow tract obstruction, and non-immune hydrops fetalis. Another potentially serious consequence is the high association of rhabdomyomas with tuberous sclerosis. Up to 50% of patients with a single cardiac rhabdomyoma have tuberous sclerosis and the incidence is even higher in patients with multiple rhabdomyomas.

Conclusion:

Cardiac rhabdomyomas are benign from the cardiovascular standpoint in most affected foetuses. As observed in postnatally diagnosed cardiac rhabdomyoma, TSC is diagnosed in most cases of fetal cardiac rhabdomyoma.