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欧洲心血管影像学会肥厚型心肌病调查

EACVI survey on hypertrophic cardiomyopathy.

作者信息

Podlesnikar Tomaz, Cardim Nuno, Ajmone Marsan Nina, D'Andrea Antonello, Cameli Matteo, Popescu Bogdan A, Schulz-Menger Jeanette, Stankovic Ivan, Toplisek Janez, Maurer Gerald, Haugaa Kristina H, Dweck Marc R

机构信息

Department of Cardiac Surgery, University Medical Centre Maribor, Ljubljanska ulica 5, 2000 Maribor, Slovenia.

Department of Cardiology, University Medical Centre Ljubljana, Zaloska cesta 2, 1000 Ljubljana, Slovenia.

出版信息

Eur Heart J Cardiovasc Imaging. 2022 Apr 18;23(5):590-597. doi: 10.1093/ehjci/jeab270.

Abstract

AIMS

The European Association of Cardiovascular Imaging (EACVI) Scientific Initiatives Committee performed a global survey to evaluate current practice for the assessment and management of patients with hypertrophic cardiomyopathy (HCM).

METHODS AND RESULTS

A total of 213 centres from 38 different countries (87% European) responded to the survey. One hundred twenty-one (57%) centres followed HCM patients in a general cardiology outpatient clinic and 85 (40%) centres in a specialized HCM/cardiomyopathy clinic. While echocardiography was the primary imaging modality, cardiovascular magnetic resonance (CMR) has become an important complementary tool. Cardiac anatomy, left ventricular (LV) systolic, and diastolic function were assessed according to current European guidelines and recommendations. To evaluate LV obstruction, 49% of the centres performed bedside provocation manoeuvres in every patient and 55% of the centres used exercise stress echocardiography. The majority of centres used the 5-year risk assessment of sudden cardiac death (SCD) calculated with the HCM Risk-SCD score. However, 34% of the centres also used extensive non-infarct late gadolinium enhancement on CMR and 27% the presence of LV apical aneurysm to help select patients for primary prevention implantable cardioverter-defibrillator therapy. Ninety-nine percent of the responding centres performed regular imaging follow-up of HCM patients.

CONCLUSION

Most centres followed European guidelines and recommendations for the diagnosis and management of patients with HCM. The importance of bedside provocation manoeuvres and exercise stress echocardiography to diagnose LV outflow obstruction requires emphasis. Additional risk markers for SCD are used in many centres and might indicate the need for an update of current European recommendations.

摘要

目的

欧洲心血管影像协会(EACVI)科学倡议委员会开展了一项全球调查,以评估肥厚型心肌病(HCM)患者评估与管理的当前实践情况。

方法与结果

来自38个不同国家的213个中心(87%来自欧洲)对调查做出了回应。121个(57%)中心在普通心脏病门诊随访HCM患者,85个(40%)中心在专门的HCM/心肌病门诊随访。虽然超声心动图是主要的成像方式,但心血管磁共振(CMR)已成为重要的辅助工具。根据当前欧洲指南和建议评估心脏解剖结构、左心室(LV)收缩和舒张功能。为评估LV梗阻,49%的中心对每位患者进行床边激发试验,55%的中心使用运动负荷超声心动图。大多数中心使用通过HCM风险-SCD评分计算的心脏性猝死(SCD)5年风险评估。然而,34%的中心还使用CMR上广泛的非梗死晚期钆增强,27%的中心使用LV心尖部室壁瘤的存在情况来帮助选择适合一级预防植入式心脏复律除颤器治疗的患者。99%的回应中心对HCM患者进行定期成像随访。

结论

大多数中心遵循欧洲关于HCM患者诊断和管理的指南及建议。需要强调床边激发试验和运动负荷超声心动图对诊断LV流出道梗阻的重要性。许多中心使用SCD的额外风险标志物,这可能表明需要更新当前的欧洲建议。

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