Diagnosis and management of fetal cardiac tumors: multicenter experience and review of published reports

Objectives. We sought to determine the prevalence and natural history of cardiac tumors in patients referred for fetal echocardiography. Background. Cardiac tumors are rare; the prevalence, reported from autopsy studies of patients of all ages, varies from 0.0017% to 0.28%. Despite many case reports, the prevalence and natural history of fetal cardiac tumors are unclear. Methods. Fourteen thousand fetal echocardiograms recorded over an 8-year period in seven centers were available for retrospective review. Medical records and echocardiograms were studied to determine the reason for referral, family history of tuberous sclerosis, prenatal and postnatal course and tumor description and type. Results. Cardiac tumors were present in 19 pregnancies (0.14%). Gestational age at diagnosis ranged from 21 to 38 weeks. The most common indication for referral was mass on an obstetric ultrasound study. The tumors were singular in 10 patients and multiple in 9. Tumor size ranged from 0.4 × 0.4 to 3.5 × 4 cm, and the majority of tumors were not hemodynamically significant. There were 17 patients with rhabdomyomas, 1 with fibrom and 1 with an atrial hemangioma. Tuberous sclerosis complex was diagnosed in 10 patients. Partial or complete tumor regression was seen in eight patients; tumors were unchanged in five; and three required operation. Conclusions. Fetal cardiac tumors, rare condition, are often benign. The majority of tumors are rhabdomyomas, but not all fetuses with rhabdomyom have tuberous sclerosis.