Outcome of septal myectomy in patients with Fabry's disease.
case_series · Level IV
Where this comes from
- Record sourced from PubMed, PMID 23272855.
- Also identified by DOI 10.1016/j.athoracsur.2012.05.087.
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Abstract
Fabry's disease has cardiac manifestations that may mimic hypertrophic cardiomyopathy, including left ventricular outflow tract (LVOT) obstruction. It is important to distinguish hypertrophic cardiomyopathy from Fabry's disease, which can be treated with enzyme replacement therapy (ERT). However, after adequate ERT, some patients have no resolution of cardiac symptoms; thus, septal myectomy might be considered. We present 2 cases of extended septal myectomy in patients with LVOT obstruction secondary to Fabry's disease.
Medical subject headings
- Cardiac Surgical Procedures
- Fabry Disease
- Heart Septum