多学科主动脉病变临床：麦吉尔经验

IF 2.5 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS

CJC Open Pub Date : 2025-07-01 DOI:10.1016/j.cjco.2025.01.021

Masaki Kodaira MD , Kevin Lachapelle MD, FRSC(C), FACS , Richard L. Leask PhD , Kevin Bates MSc , Yoni Grossman MD , Carlos-Eduardo Guerrero-Chalela MD , Lauren Kennedy MSc , George Thanassoulis MD, MSc, FRCPC , Oren K. Steinmetz MD, FRCS(C) , Kent MacKenzie MD , Josephine Pressacco MD, PhD , James C. Engert PhD , Judith Therrien MD, FRCPC

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引用次数: 0

摘要

背景：指南建议胸主动脉疾病（TAD）患者在多学科的主动脉疾病诊所就诊，因为他们的情况复杂且高风险。然而，关于此类诊所的报道是有限的。方法2016年9月至2024年5月，对567例TAD患者进行评估。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名工程研究人员组成的团队，遗传学部门的一名专职成员和一名行政助理组成。对于在我院以外进行计算机断层扫描或磁共振成像的患者，图像再分析由我们的心胸放射科医生进行。对疑似遗传性TADs患者进行基因检测。结果51例患者中有5例（9.8%）的影像学检查改变了临床决策。对250例患者进行基因检测，检测与马凡氏综合征及相关主动脉病变相关的25个基因，TAD基因阳性32例（12.8%），未知变异99例（39.6%），阴性119例（47.6%）。40名患者（7%）在首次就诊后的中位2.7个月内（四分位数1-四分位数3:1.1-4.9）接受了手术。结论：我在麦吉尔大学大动脉病变诊所8年的经验表明，多学科的TAD治疗方法可以为这一复杂的患者群体提供完整、精确和及时的治疗。

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A Multidisciplinary Aortopathy Clinic: The McGill Experience

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.

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