Summer, 2015

Indication: Patient had an initial ultrasound performed at 18 weeks gestational age (GA) and an isolated echogenic intracardiac foci (EIF) was noted; otherwise the sonogram was normal. A repeat sonogram was ordered to evaluate fetal growth at 32 weeks GA. A heart abnormality was detected on ultrasound at that time. A fetal echocardiogram was ordered to further evaluate the abnormal heart findings.

Maternal/Familial History: 27 years old; Gravida 1 Para 0; Father of baby (FOB) has a history of seizures due to tuberous sclerosis

Sonographic Findings: Multiple echogenic masses were imaged within the ventricular chambers with the largest mass located at the apex. Cardiac rhythm was normal with FHR of 153 bpm. Fetal size is normal with an EFW of 48%. No evidence of hydrops detected.

Treatment/Antenatal Management: The patient was referred to Sanger Clinic in Charlotte for a fetal echocardiogram and post management evaluation by a pediatric cardiologist. Serial ultrasounds recommended to rule out hydrops and dysrhythmias. Delivery at a tertiary care center was recommended.

Other Diagnostic Methods: Fetal brain MRI and level II sonography are indicated to rule out lesions or abnormalities of the fetal kidneys and brain associated with tuberous sclerosis.

  • Intracardic tumors are a rare finding in fetuses and most that occur prenatally are rhabdomyomas (60%-80%), especially when multiple tumors are seen. Most rhabdomyomas are discovered in the late second and third trimesters and there have been no reported cases before 22 weeks GA.
  • There is a strong correlation of rhabdomyomas to tuberous sclerosis, an autosomal dominant disorder. A genetic consultation is indicated.
  • If tuberous sclerosis is suspected, ultrasound should rule out associated lesions or abnormalities of the fetal kidneys and intracranial structures.
  • The sonographic finding of hydrops and dysrhythmias may indicate a poor prognosis and are often associated with fetal demise.
  • Most rhabdomyomas regress postnatally, although surgical removal may be necessary if tumors affect hemodynamic function and rhythm. Postoperatively, the prognosis is excellent.

References: Bianchi, D., Crombleholme, T., D’Alton, M, Maloe, F. (2010). Fetology: Diagnosis and Management of the Fetal Patient. Intracardiac Tumors. 2 ed. (pgs. 402-408). McGraw Hill Professional.