Normal pregnancy in primary alveolar hypoventilation treated with nocturnal nasal intermittent positive pressure ventilation

Abstract

Nocturnal nasal intermittent positive pressure ventilation (NIPPV) is increasingly used to treat chronic respiratory failure in a wide variety of conditions (myopathies, Ondine's curse, kyphoscoliosis, etc.) and allows a normal everyday life. Some of these diseases affect women of childbearing age. We report on a young woman suffering from primary alveolar hypoventilation, who presented with limitation of daytime activities, severe oxygen desaturation during sleep, polycythaemia and pulmonary hypertension. These abnormalities completely reversed after a few months of NIPPV applied through a nasal mask. Whilst under ventilatory assistance during sleep, she had an uneventful pregnancy and delivery of a normal baby. We suggest that in selected patients requiring NIPPV, pregnancy can be contemplated with a reasonable level of safety both for the mother and the child, provided that adequate mechanical ventilatory assistance during sleep is maintained throughout pregnancy.