Lund J T, Jensen M B, Pettersson G
Rigshospitalet, thoraxkirurgisk afdeling RT, København.
Ugeskr Laeger. 1993 May 31;155(22):1716-9.
Thirteen patients with cardiovascular manifestations of Marfan's syndrome (MS) were admitted to the Department of Cardiothoracic Surgery, Rigshospitalet, Copenhagen, during an 11-year period (1981-92). One patient died preoperatively. Fifteen operations were performed in 12 patients. Four operations were elective, 11 were acute. Dilatation of the aorta (> 5 cm) accompanied by insufficiency of the aortic valve was the most frequent cardiovascular pathology, (13/13 (100%)). Aortic dissection was present in 8/13 (62%) patients. At the primary operation seven patients had the ascending aorta and the aortic valve replaced by a composite graft. Two of these also had the mitral valve replaced. Four patients had the aortic valve replaced and three patients underwent CABG (coronary artery bypass grafting) due to dissection into the coronary arteries. Three patients underwent a second operation. Two of these had composite grafts implanted, the last underwent CABG. The operative mortality at the primary operation was 17%, at re-operation it was 33%. This report illustrates the high risk of acute complications in patients with MS and emphasizes the importance of an early diagnosis of the syndrome, subsequent examination of the cardiovascular system and prophylactic elective surgery. Control of blood pressure and monitoring of aortic dilatation and valve function is recommended.
在11年期间（1981 - 1992年），13例患有马凡综合征（MS）心血管表现的患者被收治入哥本哈根里格霍斯医院心胸外科。1例患者术前死亡。12例患者共进行了15次手术。4次手术为择期手术，11次为急诊手术。主动脉扩张（>5 cm）伴主动脉瓣关闭不全是最常见的心血管病变（13/13，100%）。8/13（62%）的患者存在主动脉夹层。初次手术时，7例患者的升主动脉和主动脉瓣被复合移植物置换。其中2例还置换了二尖瓣。4例患者置换了主动脉瓣，3例患者因夹层累及冠状动脉而接受了冠状动脉旁路移植术（CABG）。3例患者接受了二次手术。其中2例植入了复合移植物，最后1例接受了CABG。初次手术的手术死亡率为17%，再次手术时为33%。本报告说明了MS患者急性并发症的高风险，并强调了该综合征早期诊断、随后对心血管系统进行检查以及预防性择期手术的重要性。建议控制血压并监测主动脉扩张和瓣膜功能。
