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Abdominal paradox encountered in neuromuscular disease: A possible clue for cor pulmonale.

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. .

SUBMITTER: Im SI

PROVIDER: S-EPMC6275299 | biostudies-literature | 2013 Mar

REPOSITORIES: biostudies-literature

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Abdominal paradox encountered in neuromuscular disease: A possible clue for cor pulmonale.

Im Sung Il SI Kim Eung Ju EJ Kim Sun Won SW

Journal of cardiology cases 20130201 3

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. <<b>Learning ...[more]

PMID: 30533126

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