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Cardiac surgery in patients with Fabry's disease: review of literature

Joss Fernandez1, Gardar Sigurdsson, Robert Saeid Farivar

  • 1University of Iowa Hospitals and Clinics, Carver College of Medicine, Iowa, USA.

Journal of Cardiac Surgery
|May 16, 2012
PubMed

Insights

Fabry disease, a rare genetic condition, can cause significant heart problems. This case study details mitral valve repair in a patient with Fabry disease, highlighting its impact on cardiac surgery.

Area of Science:

  • Cardiology
  • Genetics
  • Lysosomal Storage Diseases

Background:

  • Fabry disease is a rare X-linked lysosomal storage disease.
  • It can lead to progressive, multi-systemic complications, including cardiac involvement.
  • Cardiac manifestations of Fabry disease can mimic other structural heart diseases.

Observation:

  • A patient with known Fabry disease presented with symptoms requiring cardiac intervention.
  • The patient underwent successful mitral valve repair surgery.
  • This case highlights the importance of considering Fabry disease in cardiac evaluations.

Findings:

  • Mitral valve repair is a viable surgical option for patients with Fabry disease.
  • Early diagnosis and management of cardiac Fabry disease are crucial.
  • Fabry disease influences cardiac surgical decision-making and outcomes.

Implications:

  • This case underscores the need for increased awareness of cardiac manifestations in Fabry disease.
  • Surgical teams should be prepared to manage Fabry disease patients undergoing cardiac procedures.
  • Further research into the cardiac-specific management of Fabry disease is warranted.

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