Fontan Failure Secondary to Charcot-Marie-Tooth–Induced Phrenic Neuropathy | Zendy

Temilola Y. Abdul | Zendy; Andrew E. Schneider | Zendy; Frank Cetta | Zendy; David J. Driscoll | Zendy

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Fontan Failure Secondary to Charcot-Marie-Tooth–Induced Phrenic Neuropathy

Author(s) -

Temilola Y. Abdul,

Andrew E. Schneider,

Frank Cetta,

David J. Driscoll

Publication year - 2018

Publication title -

texas heart institute journal

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.373

H-Index - 53

eISSN - 1526-6702

pISSN - 0730-2347

DOI - 10.14503/thij-17-6337

Subject(s) - medicine , paresis , surgery , peripheral neuropathy , tooth disease , diaphragmatic breathing , heart failure , disease , cardiology , pathology , alternative medicine , diabetes mellitus , endocrinology

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.

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