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Surgery for Annulo-aortic ectasia in Patients with Marfan Syndrome during Perinatal Period
Kenji Minatoya, Hitoshi Matsuda, Hiroaki Sasaki, Hiroshi Tanaka, Tatsuya Oda, Yosuke Inoue, Naoki Kawamoto, Sayaka Kubota, Junjiro Kobayashi.
National Cerebral and Cardiovascular Center, Osaka, Japan.

Objectives: Pregnancy is a great risk of aortic complications in patients with Marfan syndrome (MFS). The purpose of this study is to retrospectively review our surgical results of aortic root repair in patients with MFS during perinatal period. Methods: Since 1998, 6 patients with MFS (32.0+2.7 year old) have undergone surgical treatment of aortic root during perinatal period. Three patients underwent emergent operations for Stanford type A acute aortic dissection. One had total arch replacement with valve sparing aortic root replacement, and 2 had hemiarch replacement with Composite graft replacement after delivery of the baby. Three patients underwent urgent operations for annulo-aortic ectasia during their pregnancy. They were at 12 weeks, 15weeks, and 18 weeks gestation, respectively. All had valve sparing aortic root replacement with fetus in utero. All operations with fetus in utero (Group U) were performed under normothermic high flow cardiopulmonary bypass. Results: In total, the average duration of cardiopulmonary bypass and cardiac ischemia was 235±89 min. and 161±41 min., respectively. In Group U, the average duration of cardiopulmonary bypass and cardiac ischemia was 191±11 min. and 148±2 min., respectively. During the operation, heartbeat of fetus was maintained at 120-140. No mortality regarding patients, baby, and fetus in the perioperative periods. All patients survived the operations without morbidity. All neonates were born without ill effects. Conclusions: Delivery of fetus whenever possible followed by aortic repair is the best solution for acute aortic dissection during pregnancy. Although emergent operations were safely performed for acute aortic dissection during pregnancy in this study, acute aortic dissection often leads catastrophic outcomes for both patient and fetus. To prevent the devastating situation, prophylactic valve sparing root replacement for dilatation of Valsalva sinus, even in pregnancy, could be one of the options for patients with MFS.

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