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Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome
Author(s) -
Verbraecken J,
Paelinck BP,
Willemen M,
Van de Heyning P,
De Backer W
Publication year - 2003
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1046/j.0009-9163.2002.00002.x
Subject(s) - medicine , continuous positive airway pressure , positive airway pressure , marfan syndrome , anesthesia , cardiology , respiratory system , airway , positive pressure , obstructive sleep apnea
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.