Nächtliche Heimbeatmung bei juvenilem Morbus Pompe mit pulmonalem Hypertonus und Rechtsherzinsuffizienz

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Abstract
A 17-year-old girl with type II glycogen storage disease (Pompe''s) developed severe right-heart failure as a result of pulmonary hypertension due to, predominantly nocturnal, hypoventilation. At night the partial pressure of oxygen was only 30-50 mmHg, pCO2 70-100 mmHg. After persistent nightly intermittent positive pressure ventilation blood gases as well as electrocardiographic and echocardiographic findings have now - after 12 months - become normal and the patient has been completely restored to a normal life. This case demonstrates that life expectancy and quality of patients with chronic forms of Pompe''s disease (and probably also with other chronic neuromuscular diseases) can be markedly improved by nightly artificial ventilation which reduces nocturnal hypoventilation and resulting pulmonary hypertension.

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