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Outcome of septal myectomy in patients with Fabry's disease

Meghana R Kunkala1, Marie Christine Aubry, Steve R Ommen

  • 1Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.

The Annals of Thoracic Surgery
|January 1, 2013
PubMed

Insights

Fabry's disease can cause cardiac issues mimicking hypertrophic cardiomyopathy. Extended septal myectomy may be a viable treatment for persistent left ventricular outflow tract obstruction despite enzyme replacement therapy.

Area of Science:

  • Cardiology
  • Genetics
  • Metabolic Disorders

Background:

  • Fabry's disease is a genetic disorder with cardiac manifestations that can resemble hypertrophic cardiomyopathy.
  • Left ventricular outflow tract (LVOT) obstruction is a significant cardiac complication in Fabry's disease.

Observation:

  • Distinguishing Fabry's disease from hypertrophic cardiomyopathy is crucial for appropriate treatment.
  • Enzyme replacement therapy (ERT) is the standard treatment for Fabry's disease.
  • Some patients with Fabry's disease experience persistent LVOT obstruction even after adequate ERT.

Findings:

  • This study presents two cases of extended septal myectomy in patients with Fabry's disease.
  • Septal myectomy was considered for patients with refractory LVOT obstruction secondary to Fabry's disease.

Implications:

  • Extended septal myectomy can be a therapeutic option for managing persistent LVOT obstruction in Fabry's disease.
  • These findings highlight the importance of considering surgical intervention when medical management fails.
  • Further research is needed to evaluate the long-term efficacy and safety of septal myectomy in Fabry's disease patients.

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