妊娠を契機に大動脈解離を発症したMarfan症候群の1例

A Case of Marfan Syndrome Complicated with Aortic Dissection in Relation to Pregnancy

　妊娠を契機に大動脈解離を発症したMarfan症候群の1例を経験した．症例は第一子妊娠中の胸痛を既往に持つ21歳の女性で，第二子出産後の持続する胸痛を主訴に当院受診．精査の結果，大動脈弁逆流を伴う大動脈弁輪拡張症（AAE,AR）に併発した大動脈解離と診断し，大動脈基部全置換および上行大動脈人工血管置換術を施行した．術中所見からは，慢性解離を伴うAAE,ARの切迫破裂による症状と判断された．Marfan症候群は心・大血管系の異常を合併することが多いが，特に若年女性では妊娠に関連して大動脈解離を発症することが知られており，関連科の緊密な管理が必要である．また，人工弁が必要な場合には術後の妊娠の問題があり，可能ならば同種大動脈弁の使用が望ましいと考える．

A 21-year-old woman, who had once experienced chest pain in her first pregnancy with manifestations of Marfan syndrome, was admitted to our hospital due to chest pain after her second childbirth. Echocardiogra-phy showed enlargement of the ascending aorta, intimal flap, and aortic regurgitation (grade III/IV).

Composite graft replacement with reimplantation of the coronary artery was performed in an emergency operation under diagnosis of acute aortic dissection complicated with Marfan syndrome. However, intraoperative findings showed chronic aortic dissection with AAE, AR. Her post-operative course was unevent-ful and, currently, the patient is free of symptoms.

In general, Marfan syndrome introduces a high risk of aortic dissection. so it is necessary in the case of a young woman during pregnancy to have close cooperation with other relevant departments, and offer prophylactic surgery if necessary. In case of valve replacement, allograft is the graft of choice for such a patient, because of its requiring anticoagulation therapy.