Department of Cardiology, Aalborg University Hospital, Hobrovej 18-22, 9000, Aalborg, Denmark.
Department of Clinical Genetics, Aarhus University Hospital, Palle Juul-Jensens Boulevard, 8200, Aarhus, Denmark.
Clin Res Cardiol. 2021 Jul;110(7):1106-1115. doi: 10.1007/s00392-021-01858-3. Epub 2021 Apr 22.
Studies indicate that other cardiovascular problems than aortic disease are a burden for patients with Marfan syndrome (MFS). The aim of the study was to assess the extent of this issue.
A registry-based population study of patients with a Ghent II verified MFS diagnosis. Each patient was matched with up to 100 controls on age and sex. From the Danish healthcare system, we identified 407 MFS patients (from 1977 to 2014) and their cardiovascular events and compared them with those in 40,700 controls. Total follow-up time was 16,439 person years.
Mitral valve disease was significantly more common in MFS [HR: 58.9 (CI 38.1-91.1)] and happened earlier and more often in women than men with MFS [age at first registration: 22 vs. 38 years, HR: 2.1 (CI 1.0-4.4)]. Heart failure/cardiomyopathy was also more common in MFS [HR: 8.7 (CI 5.7-13.4)] and men were more affected than women, and at younger age [39 vs. 64 years, HR: 0.18 (CI 0.06-0.55)]. In all cases, atrioventricular block [HR: 4.9 (1.5-15.6)] was related to heart surgery. Supraventricular [HR: 9.7 (CI 7.5-12.7)] and ventricular tachycardia [HR: 7.7 (CI 4.2-14.3)] also occurred more often than in the control group. The risk of sudden cardiac death was increased [HR: 8.3 (CI 3.8-18.0)] but the etiology was unclear due to lack of autopsies.
Non-aortic cardiovascular disease in patients with MFS is exceptionally prevalent and the range of diseases varies between women and men. Physicians caring for MFS patients must be aware of this large spectrum of cardiovascular diseases.
研究表明，马凡综合征（MFS）患者除了主动脉疾病外，还存在其他心血管问题。本研究旨在评估这一问题的严重程度。
对经根特 II 标准确诊的 MFS 患者进行基于登记的人群研究。每位患者按照年龄和性别与至多 100 名对照进行匹配。从丹麦医疗保健系统中，我们确定了 407 名 MFS 患者（1977 年至 2014 年）及其心血管事件，并与 40700 名对照进行了比较。总随访时间为 16439 人年。
二尖瓣疾病在 MFS 中更为常见[HR：58.9（CI 38.1-91.1）]，且女性患者比男性患者更早且更常发生[首次登记年龄：22 岁 vs. 38 岁，HR：2.1（CI 1.0-4.4）]。心力衰竭/心肌病在 MFS 中也更为常见[HR：8.7（CI 5.7-13.4）]，且男性比女性更易患病，且发病年龄更早[39 岁 vs. 64 岁，HR：0.18（CI 0.06-0.55）]。在所有情况下，房室传导阻滞[HR：4.9（1.5-15.6）]均与心脏手术相关。室上性心动过速[HR：9.7（CI 7.5-12.7）]和室性心动过速[HR：7.7（CI 4.2-14.3）]也比对照组更为常见。心脏性猝死的风险增加[HR：8.3（CI 3.8-18.0）]，但由于缺乏尸检，病因尚不清楚。
MFS 患者的非主动脉心血管疾病极为普遍，且疾病范围在女性和男性之间存在差异。治疗 MFS 患者的医生必须意识到这一大类心血管疾病。
