DECOMPENSATED COR-PULMONALE AS THE FIRST MANIFESTATION OF ADULT-ONSETMYOPATHY

A 46-year-old white female was admitted for decompensated cor pulmonal e (CP), It had not interfered with her daily activities and she had no t experienced shortness of breath, fatigue or muscle weakness prior to the onset of right heart failure. A thorough investigation revealed s evere generalized muscle weakness with restrictive chest bellows disea se and secondary CP (mean pressure in the pulmonary artery 60 mm Hg), After having refused respiratory support the patient died a few days a fter admission. The muscle biopsy was consistent with adult-onset acid -maltase deficiency, This is a rare case of metabolic myopathy present ing as decompensated CP without previous symptoms of muscle weakness, This condition can easily be treated with nocturnal ventilatory suppor t, improving the quality and length of life.