Outcome of septal myectomy in patients with Fabry's disease

Meghana R. Kunkala, Marie Christine Aubry, Steve R. Ommen, Bernard J. Gersh, Hartzell V Schaff

Research output: Contribution to journalArticle

4 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)335-337
Number of pages3
JournalAnnals of Thoracic Surgery
Volume95
Issue number1
DOIs
StatePublished - Jan 2013

Fingerprint

Fabry Disease
Enzyme Replacement Therapy
Ventricular Outflow Obstruction
Hypertrophic Cardiomyopathy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine

Cite this

Outcome of septal myectomy in patients with Fabry's disease. / Kunkala, Meghana R.; Aubry, Marie Christine; Ommen, Steve R.; Gersh, Bernard J.; Schaff, Hartzell V.

In: Annals of Thoracic Surgery, Vol. 95, No. 1, 01.2013, p. 335-337.

Research output: Contribution to journalArticle

Kunkala, Meghana R. ; Aubry, Marie Christine ; Ommen, Steve R. ; Gersh, Bernard J. ; Schaff, Hartzell V. / Outcome of septal myectomy in patients with Fabry's disease. In: Annals of Thoracic Surgery. 2013 ; Vol. 95, No. 1. pp. 335-337.
@article{b866c2adf89249f1ade1ffde2045169b,
title = "Outcome of septal myectomy in patients with Fabry's disease",
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.",
author = "Kunkala, {Meghana R.} and Aubry, {Marie Christine} and Ommen, {Steve R.} and Gersh, {Bernard J.} and Schaff, {Hartzell V}",
year = "2013",
month = "1",
doi = "10.1016/j.athoracsur.2012.05.087",
language = "English (US)",
volume = "95",
pages = "335--337",
journal = "Annals of Thoracic Surgery",
issn = "0003-4975",
publisher = "Elsevier USA",
number = "1",

}

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

Y1 - 2013/1

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.

UR - http://www.scopus.com/inward/record.url?scp=84871893063&partnerID=8YFLogxK

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

VL - 95

SP - 335

EP - 337

JO - Annals of Thoracic Surgery

JF - Annals of Thoracic Surgery

SN - 0003-4975

IS - 1

ER -