Implantation of a left ventricular assist device to provide long-term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.

Original languageEnglish (US)
Pages (from-to)379-383
Number of pages5
JournalESC heart failure
Volume4
Issue number3
DOIs
StatePublished - Jan 1 2017

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Heart-Assist Devices
Duchenne Muscular Dystrophy
Cardiomyopathies
Respiratory Muscles
Muscle Strength
Diaphragm
Therapeutics
Heart Failure

Keywords

  • DMD-associated cardiomyopathy
  • Heart failure
  • LVAD support and multidisciplinary approach

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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title = "Implantation of a left ventricular assist device to provide long-term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy",
abstract = "A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.",
keywords = "DMD-associated cardiomyopathy, Heart failure, LVAD support and multidisciplinary approach",
author = "Douglas Stoller and Faris Araj and Alpesh Amin and Catherine Fitzsimmons and Robert Morlend and Thibodeau, {Jennifer T.} and Claudio Ramaciotti and Drazner, {Mark H.} and Meyer, {Dan M.} and Mammen, {Pradeep P.A.}",
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T1 - Implantation of a left ventricular assist device to provide long-term support for end-stage Duchenne muscular dystrophy-associated cardiomyopathy

AU - Stoller, Douglas

AU - Araj, Faris

AU - Amin, Alpesh

AU - Fitzsimmons, Catherine

AU - Morlend, Robert

AU - Thibodeau, Jennifer T.

AU - Ramaciotti, Claudio

AU - Drazner, Mark H.

AU - Meyer, Dan M.

AU - Mammen, Pradeep P.A.

PY - 2017/1/1

Y1 - 2017/1/1

N2 - A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.

AB - A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation. A critical factor contributing to the long-term success of this patient was the decision to select an LVAD that would not disrupt the diaphragm and thus preserve the respiratory muscle strength. This case demonstrates that permanent mechanical LVADs should be considered for appropriately selected Duchenne muscular dystrophy patients with medically refractory end-stage cardiomyopathy.

KW - DMD-associated cardiomyopathy

KW - Heart failure

KW - LVAD support and multidisciplinary approach

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