title: Cardiac rhabdomyoma in intrauterine life: clinical features and natural history. A case series and review of published reports.
abstract: BACKGROUND
  Fetal cardiac rhabdomyoma is very rare; despite the fact that many cases and series have been reported, the clinical presentation, the natural history and the frequency with which this pathology is associated with tuberous sclerosis complex are not well determined. The aim of this investigation was to study the clinical features and the natural history of cardiac rhabdomyoma when diagnosed during prenatal life.
  
  
  METHODS
  Nine cases of cardiac rhabdomyoma detected among 5276 fetal echocardiograms recorded over a 10-year period in a single center were retrospectively reviewed. Medical records and echocardiograms were studied to determine the prenatal and postnatal course and outcome.
  
  
  RESULTS
  The incidence of cardiac rhabdomyoma in our center was 0.17%. The gestational age at diagnosis ranged from 27 to 36 weeks. The most common reason for fetal echocardiography was an abnormal obstetric ultrasound scan (6/9 cases). In no case was there a family history of tuberous sclerosis. In one case, the tumor was single whereas in 8 cases multiple tumors were diagnosed. During prenatal life the majority of tumors were clinically silent. One fetus died of hydrops and arrhythmia. Four children presented with arrhythmia postnatally and one required surgery. At a mean follow-up of 47 months, total or partial regression was observed in 7 patients. Seven patients developed postnatal clinical signs of tuberous sclerosis.
  
  
  CONCLUSIONS
  Fetal cardiac rhabdomyomas are often benign and have a tendency to regress, but their prognosis is guarded due to very frequent association with arrhythmias and tuberous sclerosis. During prenatal counseling, it is of utmost importance to inform the future parents of the virtually constant perspective of tuberous sclerosis complex.
authors.affiliation: Division of Cardiology, Hospital Casa del Sole, Palermo, Italy. spipiton@neomedia.it
authors.forename: Salvatore
authors.initials: S
authors.lastname: Pipitone
authors.forename: Maurizio
authors.initials: M
authors.lastname: Mongiovì
authors.forename: Roberto
authors.initials: R
authors.lastname: Grillo
authors.forename: Sergio
authors.initials: S
authors.lastname: Gagliano
authors.forename: Velio
authors.initials: V
authors.lastname: Sperandeo
mesh_terms: D001145:Arrhythmias, Cardiac / Q000150:complications; D004452:Echocardiography; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging* / Q000503:physiopathology; D005500:Follow-Up Studies; D006338:Heart Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D011256:Pregnancy Outcome; D012075:Remission, Spontaneous; D012189:Retrospective Studies; D012207:Rhabdomyoma / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D014402:Tuberous Sclerosis / Q000150:complications; D016216:Ultrasonography, Prenatal