Echocardiographic Assessment in Patients with Vascular Ehlers-Danlos Syndrome: Insights from an Unexplored Field.

IF 3.1 Q2 PERIPHERAL VASCULAR DISEASE High Blood Pressure & Cardiovascular Prevention Pub Date : 2024-11-02 DOI:10.1007/s40292-024-00692-8
Giacomo Buso, Anna Paini, Claudia Agabiti-Rosei, Fabio Bertacchini, Deborah Stassaldi, Sara Capellini, Carlo Aggiusti, Massimo Salvetti, Carolina De Ciuceis, Marco Ritelli, Marina Venturini, Marina Colombi, Maria Lorenza Muiesan
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Abstract

Introduction: Vascular Ehlers-Danlos syndrome (vEDS) is an inherited connective tissue disorder characterized by arterial fragility. Celiprolol is a β1-adrenoceptor antagonist with partial β2 agonist activity that has been shown to reduce rates of vascular events in this setting, though the underlying mechanisms are not yet fully understood. Moreover, very few echocardiographic data are available in patients with vEDS.

Aim: To perform a comprehensive echocardiographic assessment of a cohort of patients with vEDS with or without celiprolol therapy compared with healthy subjects.

Methods: Twenty patients with genetically confirmed diagnosis of vEDS followed at our Institution (University Hospital of Brescia, Italy) were divided into two groups according to whether or not they were on celiprolol therapy at the maximum recommended dose (400 mg daily) for at least 12 months. Both groups were compared to 10 healthy individuals matched for sex, age, body mass index (BMI), and office blood pressure (BP) values. Each participant underwent transthoracic echocardiography with tissue Doppler analysis (TDI) for a comprehensive evaluation of cardiac structure and function.

Results: Mean age was 35 years and mean BMI was 21.6 kg/m2. Female sex was prevalent (60%). Left ventricular (LV) internal diameter values tended to be lower in patients with untreated vEDS than in healthy controls (4.33 vs 4.74 cm, respectively), though this difference was not statistically significant. Similar data were observed for LV mass index (56.9 vs 68.6 g/m2), stroke volume (56.6 vs 71.6 mL), and E/A ratio (1.26 vs 1.66), whereas an opposite trend was observed for e' lateral (13.2 vs 12.2 cm/s). No statistically significant difference was found between groups in terms of other parameters of LV mass, systolic and diastolic function. A normal LV geometry was found in all the cases. Indices of mechano-energetic efficiency and ventricular-arterial coupling were also similar between groups. No patient presented with aortic root dilation, mitral valve prolapse, valve insufficiency of more than mild degree, or valve stenosis of any degree.

Conclusion: Our study suggests that patients with vEDS have normal cardiac mass and geometry, as well as normal systolic and diastolic function. Celiprolol therapy does not seem to significantly influence such aspects. Compared with vascular imaging, less stringent follow-up with echocardiography seems reasonable in this setting. Future studies with prospective design should confirm these aspects.

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血管性 Ehlers-Danlos 综合征患者的超声心动图评估:未开发领域的启示。
简介血管性埃勒斯-丹洛斯综合征(vEDS)是一种以动脉脆性为特征的遗传性结缔组织疾病。塞利洛尔是一种具有部分β2受体激动活性的β1肾上腺素受体拮抗剂,已被证明可降低这种情况下的血管事件发生率,但其潜在机制尚未完全明了。此外,vEDS 患者的超声心动图数据很少。目的:与健康人相比,对接受或不接受塞利洛尔治疗的一组 vEDS 患者进行全面的超声心动图评估:在本院(意大利布雷西亚大学医院)接受随访的 20 名经基因确诊的 vEDS 患者根据是否按最大推荐剂量(每天 400 毫克)使用塞利洛尔治疗至少 12 个月分为两组。两组患者均与 10 名性别、年龄、体重指数 (BMI) 和办公室血压 (BP) 值相匹配的健康人进行了比较。每位参与者都接受了带有组织多普勒分析(TDI)的经胸超声心动图检查,以全面评估心脏结构和功能:平均年龄为 35 岁,平均体重指数为 21.6 kg/m2。女性占多数(60%)。未经治疗的vEDS患者的左心室(LV)内径值往往低于健康对照组(分别为4.33厘米和4.74厘米),但这一差异并无统计学意义。在左心室质量指数(56.9 vs 68.6 g/m2)、每搏量(56.6 vs 71.6 mL)和E/A比值(1.26 vs 1.66)方面也观察到类似的数据,而在e'侧(13.2 vs 12.2 cm/s)方面则观察到相反的趋势。在左心室质量、收缩和舒张功能的其他参数方面,组间差异无统计学意义。所有病例的左心室几何形状均正常。各组之间的机械能效率和心室-动脉耦合指数也相似。没有患者出现主动脉根部扩张、二尖瓣脱垂、轻度以上瓣膜功能不全或任何程度的瓣膜狭窄:我们的研究表明,vEDS 患者的心脏质量和几何形状正常,收缩和舒张功能也正常。塞利洛尔治疗似乎对这些方面没有明显影响。与血管造影相比,在这种情况下对超声心动图的随访不那么严格似乎是合理的。未来采用前瞻性设计的研究应能证实这些方面。
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来源期刊
CiteScore
5.70
自引率
3.30%
发文量
57
期刊介绍: High Blood Pressure & Cardiovascular Prevention promotes knowledge, update and discussion in the field of hypertension and cardiovascular disease prevention, by providing a regular programme of independent review articles covering key aspects of the management of hypertension and cardiovascular diseases. The journal includes:   Invited ''State of the Art'' reviews.  Expert commentaries on guidelines, major trials, technical advances.Presentation of new intervention trials design.''Pros and Cons'' or round tables on controversial issues.Statements on guidelines from hypertension and cardiovascular scientific societies.Socio-economic issues.Cost/benefit in prevention of cardiovascular diseases.Monitoring of healthcare systems.News and views from the Italian Society of Hypertension (including abstracts).All manuscripts are subject to peer review by international experts. Letters to the editor are welcomed and will be considered for publication.
期刊最新文献
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