Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome.
We report on a patient with Marfan's syndrome, with coexistent obstructive sleep hypopnea (OSH) and restrictive lung disease, complicated by respiratory insufficiency, who was successfully treated with nasal intermittent positive airway pressure (NIPPV) and oxygen. NIPPV therapy turned out to be effective on arterial gas exchange and well tolerated. Moreover, progressive dilatation of the aortic root was attenuated during NIPPV, but could, however, not be reversed. We hypothetized that (at least partially) a decrease of nocturnal intrathoracic pressures could explain this.
Verbraecken
J
J
Department of Thoracic Medicine, University of Antwerp, Belgium.
Paelinck
B P
BP
Willemen
M
M
Van de Heyning
P
P
De Backer
W
W
D000328:Adult; D001011:Aorta / Q000473:pathology*; D004108:Dilatation, Pathologic; D005260:Female; D006801:Humans; D007385:Intermittent Positive-Pressure Ventilation; D008382:Marfan Syndrome / Q000150:complications / Q000473:pathology / Q000628:therapy*; D012131:Respiratory Insufficiency / Q000150:complications / Q000628:therapy*; D012891:Sleep Apnea Syndromes / Q000150:complications