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. <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 mo

SUBMITTER: Im SI 

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

REPOSITORIES: biostudies-literature