Perinatal diagnosis of cardiac tumors

As fetal cardiac tumors are a rare condition, we report the perinatal diagnosis and ultrasound findings of 12 cases.

In 10 cases the tumors were identified prenatally by fetal echocardiography; gestational age at detection ranged from 22 to 34 weeks. In two symptomatic infants cardiac tumors were diagnosed on the first day postpartum; prenatal ultrasound evaluation at 26 and 38 weeks of gestation did not reveal a cardiac lesion.

Six fetuses had singular tumors, in six fetuses they were multifocal. The left ventricle was most often affected. Termination of pregnancy was chosen in three cases, one in association with trisomy 21 and tuberous sclerosis. One intrauterine and three neonatal deaths due to cardiac failure occurred. Histopathologic examination revealed cardiac rhabdomyoma in six fetuses and fibroma in one case. In the five surviving fetuses the size of the tumors spontaneously decreased postpartum. Rhabdomyomata were associated with tuberous sclerosis in four out of 11 cases.

Cardiac tumors are detectable as early as 22 weeks of gestation. Presenting symptoms may be arrhythmia, dysfunction of the atrioventricular valves, pericardial effusion and fetal hydrops. The most common perinatal diagnosis is rhabdomyoma, which is often associated with tuberous sclerosis. Sequential examination in high risk patients should be considered as most tumors increase in size during pregnancy and may become evident in late second and third trimester of pregnancy. Postpartum, however, regression of tumor size is common.