Erschienen in:
01.11.2019 | Case Report
Spontaneous ascending aortic rupture in a pregnant woman with neurofibromatosis type 1
verfasst von:
Atsushi Tateishi, Masahiro Okada, Mikizo Nakai, Yutaka Yokota, Yosuke Miyamoto
Erschienen in:
General Thoracic and Cardiovascular Surgery
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Ausgabe 11/2019
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Abstract
Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that affects 1 in 3000 individuals. Vascular involvement in NF-1 is a well-recognized, but rare, feature of this disease. In pregnant women, the risk of aortic dissection or rupture is elevated during pregnancy and the postpartum period. We report a pregnant woman who had a history of NF-1 with a spontaneous ascending aortic rupture. This rupture was successfully treated by emergent surgery. The mother and the 28-week-gestation newborn recovered uneventfully. During 7 years of follow-up, aorta of the patient shows no significant change. A review of the literature regarding the pathogenesis of this condition is also presented.