Clinical and imaging characteristics of primary fetal cardiac tumors

  • Dr Maria Kiaffas, Onassis Cardiac Surgery Center, Greece
  • Dr Cleo Laskari, Onassis Cardiac Surgery Center, Greece
  • Dr Spyridon Rammos, Onassis Cardiac Surgery Center, Greece
  • Objective: Presentation of our experience with the clinical and imaging characteristics of fetal cardiac tumors

    Methods: Seven cases of fetal cardiac tumors, at the gestational age (GA) of 22-32 weeks (wks), were diagnosed echocardiographically, between September of 2003 and December of 2008.

    Results: Two of the fetuses had multiple tumors in both the right and left ventricles (RV & LV). Five had solitary tumors; in two the mass was located at the left ventricular outflow tract (LVOT) causing obstruction, in two it was located in the ventricular septum (VS) and in one in the left atrium (LA). Termination was elected in four pregnancies; the remaining three were carried to full-term. One of the tumors (located in the VS) was histologically proven to be a fibroma; the remaining are considered to be rhabdomyomas due to histology (two), association with tuberous sclerosis (one) multiple location (one) and regression of size (one). Association with tuberous sclerosis was identified in three of the cases. Follow-up ranges from 8 months to 3.5 years. (Table)

    Conclusions: Echocardiography is a vital diagnostic tool for fetal cardiac tumors identifying their number, location, size and echogenic characteristics usually indicative of possible histology. Rhabdomyomas are the most common tumors; they can be solitary or multiple and have a high association with TS. Fibromas, the second commonest, are usually massive with preference to the VS and often associated with myocardial dysfunction. Termination of pregnancy usually follows prenatal diagnosis of myocardial tumors, especially in an early GA and when TS is suspected

    Diagnosis Location Tumor type Associated problems Outcome
    22 wks Single - VS Rhabdomyoma - Termination
    23 wks Single - VS Fibroma Myocardial dysfunction/ Pericardial effusion Termination
    23 wks Single - LA Rhabdomyoma Tuberous sclerosis Termination
    29 wks Multiple - RV & LV Rhabdomyoma Tuberous sclerosis ? Term delivery / 8 mos TS ?
    31 wks Multiple - RV & LV Rhabdomyoma Tuberous scerosis Termination
    32 wks Single - LVOT Rhabdomyoma LVOT obstruction Term delivery/ 3.5 yrs healthy
    32 wks Single - LVOT Rhabdomyoma LVOT obstruction Term delivery/ 2 yrs Renal lesions - Arrhythmias