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一个多学科主动脉病变诊所:麦吉尔大学的经验。

A Multidisciplinary Aortopathy Clinic: The McGill Experience.

作者信息

Kodaira Masaki, Lachapelle Kevin, Leask Richard L, Bates Kevin, Grossman Yoni, Guerrero-Chalela Carlos-Eduardo, Kennedy Lauren, Thanassoulis George, Steinmetz Oren K, MacKenzie Kent, Pressacco Josephine, Engert James C, Therrien Judith

机构信息

Division of Cardiology, McGill University, Montreal, Quebec, Canada.

Beth Raby Adult Congenital Heart Disease Clinic, Jewish General Hospital, Montréal, Quebec, Canada.

出版信息

CJC Open. 2025 Feb 3;7(7):921-927. doi: 10.1016/j.cjco.2025.01.021. eCollection 2025 Jul.

Abstract

BACKGROUND

Guidelines recommend that patients with thoracic aortic disease (TAD) be seen in a multidisciplinary aortopathy clinic, because of their complex and high-risk profile. However, reports on such clinics are limited.

METHODS

From September 2016 to May 2024, we evaluated 567 patients with TAD. They were seen by our multidisciplinary team, which is comprised of 1 cardiac surgeon, 2 vascular surgeons, 1 cardiologist, 1 cardiothoracic radiologist, a team of 4 engineering researchers, a dedicated member from the genetics department, and an administrative assistant. For patients who had computed tomography or magnetic resonance imaging performed outside our institution, image reanalysis was conducted by our cardiothoracic radiologist. Genetic testing was performed for patients with suspected hereditary TADs.

RESULTS

Reanalysis of external computed tomography and/or magnetic resonance imaging by our radiologist altered clinical decision-making in 5 of 51 cases (9.8%). Genetic testing that examined 25 genes associated with Marfan syndrome and related aortopathies was conducted on 250 patients, revealing a positive TAD gene in 32 (12.8%), a variant of unknown significance in 99 (39.6%), and a negative result in 119 (47.6%). Forty patients (7%) had surgery within a median of 2.7 months (quartile 1-quartile 3: 1.1-4.9) from their initial clinic visit.

CONCLUSIONS

Our 8-year experience at the aortopathy clinic of McGill University demonstrates that a multidisciplinary approach to TAD can deliver complete, precise, and timely care to this complex patient population.

摘要

背景

指南建议,由于胸主动脉疾病(TAD)患者情况复杂且风险高,应在多学科主动脉病变诊所就诊。然而,关于此类诊所的报告有限。

方法

2016年9月至2024年5月,我们评估了567例TAD患者。他们由我们的多学科团队诊治,该团队由1名心脏外科医生、2名血管外科医生、1名心脏病专家、1名心胸放射科医生、4名工程研究人员、遗传学部门的一名专职成员和一名行政助理组成。对于在我们机构外进行计算机断层扫描或磁共振成像的患者,由我们的心胸放射科医生进行图像重新分析。对疑似遗传性TAD患者进行基因检测。

结果

我们的放射科医生对外部计算机断层扫描和/或磁共振成像进行重新分析后,改变了51例中的5例(9.8%)的临床决策。对250例患者进行了检测25个与马凡综合征及相关主动脉病变相关基因的基因检测,结果显示32例(12.8%)TAD基因呈阳性,99例(39.6%)为意义不明的变异,119例(47.6%)为阴性。40例(7%)患者在首次就诊后的中位时间2.7个月(四分位数1 - 四分位数3:1.1 - 4.9)内接受了手术。

结论

我们在麦吉尔大学主动脉病变诊所的8年经验表明,对TAD采用多学科方法可为这一复杂患者群体提供全面、精确和及时的治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9b33/12277808/7f272b78cbbc/gr1.jpg

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