Heart and heart-liver transplantation in Amish patients with propionic acidemia
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View abstract on PubMed
Summary
This summary is machine-generated.Propionic acidemia (PA) in Amish patients can cause severe heart failure. Combined heart/liver transplantation may be a viable option for managing this rare metabolic disorder.
Area Of Science
- Genetics
- Metabolic Disorders
- Cardiology
Background
- Propionic acidemia (PA) is a genetic disorder affecting propionyl-CoA carboxylase activity.
- PA leads to toxic metabolite accumulation from odd-chain fatty acids and branched-chain amino acids.
- A specific PA variant in the Amish population often presents as dilated cardiomyopathy.
Purpose Of The Study
- To describe the clinical course and management of Amish patients with PA-associated dilated cardiomyopathy.
- To evaluate treatment strategies, including heart transplantation, for end-stage heart failure in this population.
Main Methods
- Case series reporting on three Amish patients with genetically confirmed PA and end-stage heart failure.
- Review of treatment outcomes, including heart transplantation and combined heart/liver transplantation.
Main Results
- One patient had a successful heart transplant without complications.
- Another patient experienced recurrent cardiogenic shock post-transplant due to metabolic decompensation.
- A third patient was treated with combined heart/liver transplantation.
Conclusions
- Managing PA-related metabolic cardiomyopathies presents unique challenges.
- A multidisciplinary approach is crucial for optimizing outcomes in these patients.
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