Marfan症候群に対する大動脈基部置換,僧帽弁置換,上行弓部下行大動脈置換同時手術の1治験例  [in Japanese] A Case Report of Aortic Root Replacement, Mitral Valve Replacement and Extended Thoracic Aorta Replacement for a Patient with Marfan's Syndrome  [in Japanese]

Marfan症候群では異時的ばかりでなく,同時に心血管病変が多発し,拡大手術,多期手術が必要になることも多い.全身状態と再手術を考慮して症例に合わせた必要十分な手術術式を選択,工夫することが重要である.今回,われわれは大動脈弁閉鎖不全を伴う大動脈基部拡大,僧帽弁閉鎖不全,Stanford B型慢性大動脈解離を合併した39歳,女性のMarfan症候群の症例に対し,Carrel patch法による大動脈基部置換,僧帽弁置換,上行弓部胸部下行大動脈置換を一期的に行い良好な結果を得た.待機例での2弁同時置換に上行弓部下行大動脈置換を行った報告はわれわれが知るかぎりでは本症例が初めてであるが,若く,合併症のない症例であったので,脳保護法,心筋保護法などの発達した現在では許容しうる手術侵襲であった.手術時の工夫を若干の文献的考察を加えて報告した.

A 39-year-old woman with Marfan's syndrome was referred with a symptom of exertional dyspnea, had mitral valve regurgitation, annuloaortic ectasia with aortic valve regurgitation and Stanford B type chronic aortic dissection. She was successfully treated with a one-stage operation, consisting of aortic root replacement with the Carrel patch method, mitral valve replacement and extended replacement of the thoracic aorta (ascending, arch and thoracic descending aorta), through median sternotomy and left antero-axillary thoracotomy. This operation was performed under hypothermic circulatory arrest with continuous retrograde cerebral perfusion. The postoperative course was uneventful. Although the operation may include complicated procedures, it is important to perform a sufficient operation corresponding to the patient's condition and lesions, employing the most advanced surgical techniques, such as circulatory arrest, myocardial protection and so on.