Abdominal paradox encountered in neuromuscular disease

A possible clue for cor pulmonale

Sung Il Im, Eung Ju Kim, Sun Won Kim

Research output: Contribution to journalArticle

Abstract

Oculopharyngeal muscular dystrophy (OPMD) is an uncommon autosomal dominant disorder that has been characterized by slow progression. Neuromuscular disease is one of several etiologies of pulmonary volume restriction from extrinsic or parenchymatous causes and can lead to pulmonary hypertension and right-sided heart failure, which is consistent with cor pulmonale. Here we describe a case of an OPMD patient with cor pulmonale that was reversed using mechanically-assisted ventilation < Learning objective: Although respiratory muscle weakness is an uncommon cause of respiratory failure and a rare cause of cor pulmonale, the specific physical signs for it could evoke those serious respiratory and cardiac conditions as well as underlying neuromuscular disease. Abdominal paradox is the most characteristic physical sign of dysfunction of the diaphragm, the principal muscle of respiration. However, it is often not easy to notice the sign, especially in the patients with neuromuscular disease>.

Original languageEnglish
JournalJournal of Cardiology Cases
Volume7
Issue number3
DOIs
Publication statusPublished - 2013 Mar 1

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Oculopharyngeal Muscular Dystrophy
Pulmonary Heart Disease
Neuromuscular Diseases
Pulmonary Hypertension
Ventilation
Heart Failure
Lung

Keywords

  • Cor pulmonale
  • Oculopharyngeal muscular dystrophy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Abdominal paradox encountered in neuromuscular disease : A possible clue for cor pulmonale. / Im, Sung Il; Kim, Eung Ju; Kim, Sun Won.

In: Journal of Cardiology Cases, Vol. 7, No. 3, 01.03.2013.

Research output: Contribution to journalArticle

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