Successful Heart Transplant in Dilated Cardiomyopathy Associated With Alström Syndrome: A Case Report

Jung Min Park1, Yu Rim Shin2, Jae Won Oh3

  • 1Department of Pediatrics, National Health Insurance Service Ilsan Hospital, Goyang, Korea.

Transplantation Proceedings
|November 12, 2022
PubMed

Insights

Alström syndrome patients with heart issues can undergo successful heart transplants, even with liver problems. Early intervention in heart dysfunction is key to preventing other organ failures in this rare genetic disorder.

Area of Science:

  • Cardiology
  • Genetics
  • Transplantation

Background:

  • Alström syndrome is a rare genetic disorder affecting multiple organs.
  • Dilated cardiomyopathy affects approximately two-thirds of Alström syndrome patients.
  • Heart transplantation is often not ideal due to organ shortages and multi-organ dysfunction.

Observation:

  • A 17-year-old boy with Alström syndrome presented with severe liver dysfunction.
  • The patient also had dilated cardiomyopathy, a common complication of Alström syndrome.

Findings:

  • The patient underwent a successful isolated heart transplant.
  • Despite severe pre-existing liver dysfunction, the transplant was successful.

Implications:

  • This case highlights the potential for successful isolated heart transplantation in Alström syndrome patients with co-existing organ dysfunction.
  • Timely management of cardiac dysfunction may prevent or reverse other organ failures in Alström syndrome.
  • This approach may offer a viable therapeutic option for select Alström syndrome patients.