학술논문
Aortic root diameter and nasal intermittent positive airway pressure treatment in Marfan's syndrome.
Document Type
Article
Source
Subject
*MARFAN syndrome treatment
*GENETIC disorder treatment
*MEDICAL genetics
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Language
ISSN
0009-9163
Abstract
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. [ABSTRACT FROM AUTHOR]