Fetal cardiac rhabdomyoma: analysis of five cases - Revista Brasileira de Ginecologia e Obstetrícia

PURPOSE: to analyze the differential diagnosis, follow-up and therapeutic approach in five cases of primary cardiac tumors diagnosed during the prenatal period. METHODS: during the period from January 1997 to December 2008, 7989 pregnant women were submitted to morphological ultrasound due to the presence of risk factors for fetal malformations. Fetuses with hyperechogenic intracardiac masses larger than 1 mm diagnosed by ultrasound evaluation of the fetal heart, were selected for study. The differential diagnosis between the different tumor types was made on the basis of the ultrasound characteristics of the masses. RESULTS: five fetuses with hiperechogenic intracardiac masses were diagnosed, corresponding to a 0.06% prevalence rate. Gestational age ranged from 28 to 36 weeks (mean: 31), and maternal age ranged from 23 to 45 years (mean: 34,2). The most frequent location of the masses was the left ventricle (100%). Echographically, all masses were single or multiple, hyperechogenic, homogeneous and well delimited, compatible with a diagnosis of rhabomyoma. In cases in which the diameters of the masses were less than 20 mm, an expectant conduct was followed and no complications occurred during the prenatal period. One case with a huge tumor presented arrhythmia and cardiac insufficiency during the 35 gestational weeks, and the interruption of pregnancy was indicated. Tuberous sclerosis was associated in four cases (80%) and the diagnosis was confirmed during the postnatal follow-up. CONCLUSIONS: fetal morphological ultrasonography is the main form of early detection of primary cardiac tumors. The fetal cardiac evaluation is of fundamental importance for the differential morphological characterization of cardiac masses and for the evaluation of cardiac function. Rhabdomyomas are the most common type of fetal tumor. An expectant pre and postnatal conduct is followed, with a low risk of complications and with the possibility of spontaneous regression in most cases. Postnatal clinical follow-up is mandatory due to the high frequency of associated tuberous sclerosis.