Fontan failure secondary to charcot-marie-tooth– induced phrenic neuropathy

Temilola Y. Abdul, Andrew E. Schneider, Frank Cetta, David J. Driscoll

Research output: Contribution to journalArticlepeer-review

1 Scopus citations


Charcot-Marie-Tooth disease comprises a vast array of defects in myelin integrity that causes progressive peripheral sensorimotor neuropathy. It is the most prevalent inherited peripheral neuropathy, and it can affect the management of coexisting medical conditions. We report the case of a 25-year-old woman who had undergone successful Fontan surgery during childhood, but her Fontan circulation failed as a result of diaphragmatic paresis caused by Charcot-Marie-Tooth disease type 1A. This diagnosis precluded cardiac transplantation.

Original languageEnglish (US)
Pages (from-to)270-272
Number of pages3
JournalTexas Heart Institute Journal
Issue number4
StatePublished - Aug 2018
Externally publishedYes


  • Charcot-marie-tooth disease/complications/ genetics/physiopathology
  • Diaphragm/innervation
  • Fontan procedure/adverse effects
  • Heart defects, Congenital/complications/ surgery
  • Muscular atrophy/ genetics
  • Muscular weakness/etiology
  • Phrenic nerve/ injuries
  • Postoperative complications/epidemiology
  • Respiratory paralysis/etiology
  • Time factors

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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