Benign heart tumors present with a large spectrum of clinical features

  • Dr Susanne Navarini-Meury, Department of Pediatric Cardiology, University Hospital Berne, Switzerland
  • Mladen Pavlovic, Department of Pediatric Cardiology, University Hospital Berne, Switzerland
  • Alexander Kadner, Department of Heart Surgery, University Hospital Berne, Switzerland
  • Prof Thierry Carrel, Department of Heart Surgery, University Hospital Berne, Switzerland
  • Prof Jean-Pierre Pfammatter, Department of Pediatric Cardiology, University Hospital Berne, Switzerland

    • Objective
    Primary cardiac tumors are rare in the pediatric population and symptoms vary widely. We report on 5 children with a benign heart tumor presenting with different clinical symptoms.

    Methods
    We analysed retrospectively all patients presenting with a benign heart tumor at our pediatric cardiology department in the past 10 years.

    Results
    5 patients were seen in the past 10 years for benign heart tumors. 2 patients were diagnosed with rhabdomyoma, 2 with fibroma and 1 patient with myxoma. Patients age varied from newborns up to a 14-year-old boy. Two patients presented with arrhythmia, one with supraventricular and one with ventricular tachycardia. Both were found to have rhabdomyoma and diagnosis of tuberous sclerosis. One patient presented with impaired sportive performance, dyspnea, coughing and syncope due to tricuspidal stenosis caused by a myxoma. Another patient was seen due to unclear dysmorphic features. Echocardiography revealed pericardial fibroma and a large ventricular septal defect, with a genetic analysis resulting in Gorlin syndrome. The second patient with fibroma presented with failure to thrive as the only symptom.

    Conclusions
    Pediatric primary cardiac tumors are extremely rare, and most of them are histologically benign. Depending on size and location of the tumor, patients present with a great variety of clinical symptoms. Besides hemodynamic obstruction, arrhythmia is an important sign and the first episode can be a life-threatening arrhythmia or sudden death. This case series underlines the diversity of clinical features in children with benign cardiac tumors.