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Long-Term Outcomes in Heart Transplanted Patients with Anderson-Fabry Disease: Effectiveness of a Combined Medical

Chiara Tessari1, Luigi Garufi1, Gino Gerosa1

  • 1Cardiac Surgery Unit, Department of Cardio-Thoracic-Vascular Sciences and Public Health, University of Padova, Padova, Italy.

Surgical Technology International
|August 3, 2022
PubMed

Insights

Heart transplantation combined with early enzyme-replacement therapy (ERT) offers Anderson-Fabry disease patients improved long-term outcomes. This approach should be considered for better quality of life in end-stage organ failure.

Area of Science:

  • Cardiology
  • Nephrology
  • Genetics

Background:

  • Anderson-Fabry disease is a genetic disorder leading to end-stage organ failure.
  • Organ transplantation for these patients remains controversial due to limited long-term outcome data.

Purpose of the Study:

  • To evaluate the long-term results of heart transplantation combined with early enzyme-replacement therapy (ERT) in Anderson-Fabry disease patients.
  • To provide evidence supporting transplantation with early ERT for improved patient prognosis.

Main Methods:

  • Retrospective analysis of patients with Anderson-Fabry disease who underwent heart transplantation.
  • Comparison of outcomes in patients receiving early ERT versus those with delayed or no ERT post-transplantation.

Main Results:

  • Patients receiving heart transplantation combined with early ERT demonstrated favorable long-term outcomes.
  • Early ERT initiation post-transplantation is associated with better prognosis compared to delayed or absent ERT.

Conclusions:

  • Heart transplantation combined with lifelong ERT should be considered a viable treatment option for end-stage Anderson-Fabry disease.
  • This combined therapeutic strategy offers patients a chance for improved long-term outcomes and quality of life.
Abstract

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