Cardiopulmonary Insufficiency in Myotonic and Progressive Muscular Dystrophy

Abstract

DESPITE the frequent occurrence of ventilatory insufficiency and congestive heart failure in myotonic and progressive muscular dystrophy,1 2 3 the cardiac and pulmonary disturbances in these disorders have rarely been evaluated. In myotonic dystrophy particularly, symptoms of acrocyanosis, hypersomnolence and lethargy have been noted for many years,4 5 6 but no physiologic explanation has been offered. An appraisal of the cardiopulmonary functions in muscular dystrophy was stimulated by our observations on a young man with nondisabling myotonic dystrophy who showed marked somnolence, hypercapnia, hypoxemia and Cheyne–Stokes respiration.7 This report describes the ventilatory functions, blood gas concentrations, effects of carbon dioxide and oxygen inhalation and . . .