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

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.