Marfan症候群合併妊娠患者に対する妊娠継続状態でのBentall手術

Bentall Operation for Pregnant Women with Marfan’s Syndrome

Marfan症候群合併妊娠では，妊娠中に大動脈解離を発症する危険がある1，2）．また大動脈弁閉鎖不全症（AR）を合併している場合には，循環血液量の増加や心拍出量の増加に伴いARが悪化しやすい．われわれは，妊娠中期にStanford B型急性大動脈解離を発症し，後負荷の増大に伴うARの悪化による心不全および呼吸不全をきたした症例に対し，妊娠継続のまま体外循環下にBentall手術を行い，母子ともに救命しえた症例を経験したので若干の文献的考察を加え報告する．

A 35-years-old pregnant woman with Marfan’s syndrome visited the emergent department. She had sudden severe back pain. She was at the 20th week of gestation. An emergent chest computed tomo-graphy scan showed Stanford type B acute aortic dissection. After admission, strict blood pressure control was started. According to aortic valve insufficiency and fluid retention with pregnancy, acute heart and respiratory failure was getting worse. It seemed risky for both mother and the fetus to continue pregnancy. After sincere and detailed discussion between the patient and our multidisciplinary medical team, the patient decided to continue pregnancy. An urgent Bentall operation was performed. A careful attention was paid for the fetus during and after the surgery. Strict blood pressure control was also continued. The mother and the 30-week-gestation newborn recovered uneventfully. During four years of follow-up, thoracic and thoraco-abdominal aortic replacement was performed. The patient survived all of these procedures without any complication.