TY - JOUR
T1 - Outcome of septal myectomy in patients with Fabry's disease
AU - Kunkala, Meghana R.
AU - Aubry, Marie Christine
AU - Ommen, Steve R.
AU - Gersh, Bernard J.
AU - Schaff, Hartzell V.
PY - 2013/1/1
Y1 - 2013/1/1
N2 - 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.
AB - 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.
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UR - http://www.scopus.com/inward/citedby.url?scp=84871893063&partnerID=8YFLogxK
U2 - 10.1016/j.athoracsur.2012.05.087
DO - 10.1016/j.athoracsur.2012.05.087
M3 - Article
C2 - 23272855
AN - SCOPUS:84871893063
SN - 0003-4975
VL - 95
SP - 335
EP - 337
JO - Annals of Thoracic Surgery
JF - Annals of Thoracic Surgery
IS - 1
ER -