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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.
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.
Abstract:
Alström syndrome is a rare, multisystemic genetic disorder, and dilated cardiomyopathy occurs in approximately two-thirds of patients with this condition. Because of donor organ shortage and unfavorable prognosis of multiple organ dysfunction, heart transplant is not the most desirable therapeutic option for patients with dilated cardiomyopathy with Alström syndrome. However, eliminating heart dysfunction elements at an appropriate time itself plays a pivotal role in preventing or even reversing other organ failures. Herein, we report the case of a 17-year-old boy who underwent successful isolated heart transplant despite severe liver dysfunction.
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