Cardiomyopathy in Marfan syndrome
Cardiomyopathy in Marfan syndrome
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DOI：
10.1093/ejcts/ezv073
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发表时间：
2016-02-01
影响因子：
3.4
通讯作者：
Walter, Eva Maria Delmo
中科院分区：
文献类型：
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作者：
Hetzer, Roland;Siegel, Guenter;Walter, Eva Maria Delmo
本报告旨在评估马凡氏综合征（MFS）患者的原发性和继发性心肌病的存在，这些患者接受了这种遗传疾病的原发性心血管后遗症的手术治疗。同样，我们的目的是确定MFS的心肌是否易受缺血影响，而不依赖于手术期间使用的心肌保护。1986年4月至2012年5月期间，421例MFS患者因心血管症状接受了手术治疗。其中47例（平均年龄：39.45±12.64岁，中位数：36岁，范围：19-66岁）最终因心肌病接受手术治疗。他们被分为A组：随后发生缺血性心肌病并最终因冠状动脉疾病接受冠状动脉血运重建术的患者（n = 11）；B：随后发展为终末期心肌病，植入机械循环支持装置支持衰竭心脏的患者（n = 13）； C：随后发展为终末期心肌病的患者（n = 23），其中21例进行了原发心脏移植，2例仍在等待供体心脏。对47例患者的病历进行回顾性分析，结果显示：A组3例（27.2%）患者在首次各类心血管手术前已存在缺血性心肌病，8例（72.7%）患者术后出现缺血性心肌病。既往手术与心肌病发生的时间间隔平均为8.0±07年。B组5例（38.4%）术前存在原发性心肌病，8例（61.5%）术后出现终末期心肌病。既往手术与心肌病发生的时间间隔平均为9.0±4个月。在C组中，5例（21.7%）在心血管手术前被诊断为心肌病，18例（78.2%）在术后发展为终末期心肌病。既往手术与心肌病发生的平均间隔时间为3±0.9年。平均随访9.4±1.37年，总生存率为51.8%。根据心肌病手术治疗情况分类，A、B、C组生存率分别为54.5%（平均随访9.35 +/- 1.8年）、40.1%（平均随访7.01 +/- 2.8年）和70%（平均随访10.5 +/- 2.0年）。各组间生存率差异无统计学意义（P = 0.56）。心肌保护类型和缺血时间对心肌病的发生无显著影响（P < 0.78）。我们的发现支持了一部分MFS患者存在心肌病。马凡氏心肌病似乎与心肌保护类型和缺血持续时间无关。
This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery.Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 +/- 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts.Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) patients had already existing ischaemic cardiomyopathy before the first various cardiovascular surgeries, while ischaemic cardiomyopathy in the other 8 (72.7%) developed postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 8.0 +/- 07 years. In Group B, 5 (38.4%) had existing primary cardiomyopathy prior to surgery, while 8 (61.5%) developed end-stage cardiomyopathy postoperatively. The interval between previous surgery and development of cardiomyopathy was a mean of 9.0 +/- 4 months. In Group C, 5 (21.7%) had been diagnosed with cardiomyopathy prior to the cardiovascular surgery, while 18 (78.2%) developed end-stage cardiomyopathy postoperatively. The mean interval between previous surgery and development of cardiomyopathy was 3 +/- 0.9 years. At a mean follow-up of 9.4 +/- 1.37 years, the overall survival rate is 51.8%. Categorized based on the surgical treatment done for cardiomyopathy, survival rates of 54.5% (the mean follow-up of 9.35 +/- 1.8 years), 40.1% (mean follow-up of 7.01 +/- 2.8 years) and 70% (mean follow-up of 10.5 +/- 2.0 years) were seen in Groups A, B and C, respectively. There is no significant difference in survival rates (P = 0.56) among groups. Likewise, the type of myocardial protection and duration of ischaemic times were not significant (P > 0.78) to the development of cardiomyopathy.Our finding supports the existence of cardiomyopathy in a subset of patients with MFS. Marfan cardiomyopathy appears to be independent of the type of myocardial protection and duration of ischaemia.