[A case of progressive muscular dystrophy with pulmonary hypertension].

A case of progressive muscular dystrophy of the limb girdle type is reported. The patient, a 37-year-old man, showed severe hypoxemia upon blood gas analysis, which had been predicted by pulmonary function tests, together with elevated pulmonary arterial pressure revealed by cardiac catheterization. He showed abnormal symptoms of respiration during the night, and so a sleep study was performed. The results revealed central type apnea not only during REM sleep but also frequently during NREM sleep. Acidosis and hypoxemia induced by sleep apnea caused vasoconstriction of the pulmonary artery and long-term repetition of this had caused pulmonary hypertension.