RT Book, Section
A1 Bianchi, Diana W.
A1 Crombleholme, Timothy M.
A1 D'Alton, Mary E.
A1 Malone, Fergal D.
SR Print(0)
ID 1106398479
T1 Intracardiac Tumors
T2 Fetology: Diagnosis and Management of the Fetal Patient, 2e
YR 2015
FD 2015
PB McGraw-Hill Education
PP New York, NY
SN 978-0-07-144201-5
LK obgyn.mhmedical.com/content.aspx?aid=1106398479
RD 2024/12/11
AB Key  PointsIntracardiac  tumors  are  extremely  rare  in  fetuses,  occurring  in  0.11%  to  0.14%  of  fetal  echocardiographic  studies.Most  fetal  intracardiac  tumors  (60%-80%)  are  due  to  rhabdomyoma.  Other  causes  (in  order  of  decreasing  frequency)  are  teratoma,  fibroma,  vascular  tumors,  and  myxoma.  If  multiple  tumors  are  present,  the  diagnosis  is  almost  always  rhabdomyoma.Sixty  to  ninety-five  percent  of  cases  of  rhabdomyoma  are  due  to  tuberous  sclerosis,  an  autosomal  dominant  disorder.Antenatal  management  includes  evaluation  of  fetal  cardiac  function  and  determining  if  dysrhythmias  are  present.Hydrops  and  dysrhythmias  are  associated  with  in  utero  demise.  Overall,  there  is  a  66%  survival  rate  for  fetuses  with  intracardiac  tumors.Genetic  consultation,  examination  of  parents  and  siblings,  fetal  brain  MRI,  and  level  II  sonography  are  all  indicated  to  rule  in  or  rule  out  tuberous  sclerosis.Delivery  should  occur  at  a  tertiary  center.Most  tumors  regress  postnatally,  but  if  surgical  removal  is  necessary,  the  postoperative  prognosis  is  excellent.Tuberous  sclerosis  is  associated  with  relatively  high  rates  of  new  mutation  and  gonadal  mosaicism,  so  the  affected  fetus/neonate  should  undergo  DNA  testing  to  both  confirm  diagnosis  and  provide  a  definitive  means  of  prenatal  diagnosis  in  future  pregnancies.