Bhudia Sunil K, Troughton Richard, Lam Buu-Khanh, Rajeswaran Jeevanantham, Mills William R, Gillinov A Marc, Griffin Brian P, Blackstone Eugene H, Lytle Bruce W, Svensson Lars G
Marfan Syndrome and Connective Tissue Disorder Clinic, Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Ann Thorac Surg. 2006 Mar;81(3):843-8. doi: 10.1016/j.athoracsur.2005.08.055.
Because mitral valve dysfunction in adults with Marfan syndrome is poorly characterized, this study compares mitral valve pathophysiology and morphology with that of myxomatous mitral disease, documents types of mitral valve operations, and assesses long-term survival and durability of mitral valve surgery in Marfan patients.
From May 1975 to June 2000, 27 adults with Marfan syndrome underwent mitral valve surgery. Their valve pathophysiology and morphology was compared with that of 119 patients with myxomatous mitral disease undergoing surgery from September 1995 to March 1999. Survival and repair durability were assessed at follow-up.
Compared with myxomatous disease patients, Marfan patients had less posterior leaflet prolapse (44% versus 70%, p = 0.01), more bileaflet (44% versus 28%, p = 0.09) and anterior leaflet prolapse (11% versus 3%, p = 0.07), and presented earlier for surgery (age 41 +/- 12 years versus 57 +/- 13, p < 0.0001). Marfan patients had longer and thinner leaflets. Mitral valve repair was performed less frequently in Marfan (16 of 27, 59%) than myxomatous disease patients (112 of 119, 94%). There were no hospital deaths; at 10 years, survival was 80% and freedom from reoperation 96%, with only 1 reoperation among the 16 repairs.
Mitral valve pathophysiology and morphology differ between Marfan and myxomatous mitral valve diseases. Valve repair in Marfan patients is durable and gives acceptable long-term results, even in adults who present with advanced mitral valve pathology. With increasing use of the modified David reimplantation operation and sparing of the aortic valve, mitral valve repair is a greater imperative, particularly since we have not had to reoperate on any Marfan patients with reimplantations.
由于马方综合征成年患者的二尖瓣功能障碍特征尚不明确，本研究比较了二尖瓣的病理生理学和形态学与黏液瘤样二尖瓣疾病的差异，记录了二尖瓣手术的类型，并评估了马方综合征患者二尖瓣手术的长期生存率和耐久性。
1975年5月至2000年6月，27例成年马方综合征患者接受了二尖瓣手术。将他们的瓣膜病理生理学和形态学与1995年9月至1999年3月接受手术的119例黏液瘤样二尖瓣疾病患者进行比较。随访时评估生存率和修复耐久性。
与黏液瘤样疾病患者相比，马方综合征患者后叶脱垂较少（44%对70%，p = 0.01），双叶脱垂较多（44%对28%，p = 0.09），前叶脱垂较多（11%对3%，p = 0.07），且手术时间较早（年龄41±12岁对57±13岁，p < 0.0001）。马方综合征患者的瓣叶更长更薄。马方综合征患者二尖瓣修复的频率（27例中的16例，59%）低于黏液瘤样疾病患者（119例中的112例，94%）。无院内死亡；10年时，生存率为80%，再次手术率为96%，16例修复中仅1例再次手术。
马方综合征与黏液瘤样二尖瓣疾病的二尖瓣病理生理学和形态学不同。马方综合征患者的瓣膜修复是持久的，能给出可接受的长期结果，即使是患有晚期二尖瓣病变的成年人。随着改良大卫再植入手术的应用增加以及主动脉瓣的保留，二尖瓣修复变得更加必要，特别是因为我们尚未对任何接受再植入手术的马方综合征患者进行再次手术。
