周産期に大動脈解離を来したMarfan症候群の１例

概要

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Marfan syndrome is a heritable connective tissue disorder.The cardiovascular effects are life-threatening and life expectancy depends upon aortic dilatation,which results in dissection,rupture,or aortic regurgitation. We experienced a case of Marfan syndrome complicated by aortic dissection during pregnancy. A 28-year-old woman,who had been diagnosed with gestosis and imminent premature delivery in the 30th week of gestation by another obstetrician,visited our hospital because of general fatigue.The chest X-ray film showed cardiomegaly and congestion of the lung.Echocardiography showed enlargement of the aortic root and intimal flap,and severe aortic regurgitaton.We diagnosed this case as acute aortic dissection (DeBakey typeﾜ)with annuro-aortic ectasia. After transvaginal delivery, emergency operations for composite graft replacement with reimplantation of the coronary artery and aortic valve replacement were peformed.The post operative course was uneventful and the patient and her baby were discharged safely. Pregnancy,which is known to impose substantial cardiovascular stress, increases the risk of dissection especially in the patients with Marfan syndrome.We should recognize the risk in such cases,obtain the full understanding of the patient and her family, decide whether the pregnancy should go on, and have close cooperation with other relevant departments.
信州医学会の論文
1999-08-10