Cardiopulmonary function in fibrodysplasia ossificans progressiva.
Abstract
Cardiopulmonary function was evaluated in 21 patients with fibrodysplasia ossificans progressiva. Neither cardiac enlargement nor failure was observed, but six patients had abnormal electrocardiograms. All had marked restrictive spirometry because of chest wall fixation and depended upon diaphragmatic respiration. The severity of chest restriction was independent of sex, age, duration of disease, and extnet of other physical disability. Progression to chronic respiratory failure was not observed. Chest infection in the presence of diminished pulmonary reserve is the major hazard of life in this rare disease and prophylactic measures should be considered.
