Berlin Heart EXCOR® pediatric ventricular assist device in a patient with Sotos syndrome: a case report

J Med Case Rep. 2019 Aug 31;13(1):286. doi: 10.1186/s13256-019-2190-9.

Abstract

Introduction: Berlin Heart EXCOR® pediatric ventricular assist device is a mechanical circulatory support device currently used in pediatric patients. Sotos syndrome is a well-described multiple anomaly syndrome characterized by overgrowth, distinctive craniofacial appearance, cardiac abnormalities, and variable learning disabilities.

Case presentation: We describe a 7-year-old female Caucasian child with classic Sotos syndrome features subjected to implantation of Berlin Heart EXCOR® pediatric biventricular assist device mechanical support. A heart transplant was carried out after a support time of 459 days. After 5 years of follow-up, our patient is clinically stable and the performance of the transplanted heart is excellent.

Conclusion: This case confirms that Berlin Heart EXCOR® pediatric ventricular assist device can provide satisfactory and safe circulatory support for children with end-stage heart diseases, even in those with Sotos syndrome. The syndrome is not a contraindication to implantation, since the complications are the same as those observed in patients without the syndrome and the prognosis is not affected by the disease.

Keywords: Berlin Heart EXCOR® pediatric ventricular assist device; NSD1 gene; Sotos syndrome.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Female
  • Heart Transplantation
  • Heart-Assist Devices*
  • Humans
  • Sotos Syndrome / therapy*