Department of Cardiology, Complejo Hospitalario Universitario de A Universidad de A Coruña (UDC), Coruña, Spain.
Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, Spain.
Postgrad Med. 2022 May;134(4):420-428. doi: 10.1080/00325481.2022.2054174. Epub 2022 Mar 23.
We aimed to describe the clinical characteristics, underlying causes and outcomes of syncope in patients with transthyretin amyloid cardiomyopathy (ATTR-CM).
The clinical profile and underlying causes of syncopal episodes were reviewed in a cohort of 128 patients with ATTR-CM enrolled from January 2018 to June 2020 in a prospective multicentre registry in 7 hospitals of Galicia (Spain). After enrollment, patients were followed during a median period of 520 days. The effect of syncope on all-cause mortality was assessed by means of multivariate Cox´s regression.
Thirty (23.4%) patients had a history of previous syncope as a clinical antecedent before being enrolled in the prospective phase of the registry, and 4 (3.1%) experienced a first episode of syncope thereafter. The estimated incidence density rate of syncope during the prospective follow-up period after registry enrollment was 71.9 episodes per 1000 patients-year (95% Confidence Interval (CI) 32.8-111.1). The estimated overall prevalence of syncope was 26.6% (95% CI 18.9%-34.2%). Cardiac arrhythmias (n = 11, 32.3%), structural diseases of the heart or great vessels (n = 5, 14.7%), a neurally mediated reflex (n = 6, 17.6%), and orthostatic hypotension (n = 4, 11.8%) were identified as probable underlying causes of syncope; in 8 (23.6%) patients, syncope remained unexplained. Patients with syncope had increased non-adjusted all-cause mortality than patients without it (univariate hazard-ratio 3.37; 95% CI 1.43-7.94). When other independent predictors of survival were added to the survival model, this association was no longer statistically significant (multivariate hazard-ratio 1.81, 95% CI 0.67-4.84).
Syncope is frequent in patients with ATTR-CM. This study could not demonstrate an independent association between syncope and mortality in those individuals. ATTR-CM: Transthyretin amyloid cardiomyopathy; CI: Confidence Interval; HF: Heart Failure; HR: Hazard Ratio; IQR: Interquartile rank; LVEF: Left Ventricular Ejection Fraction; NTproBNP: N-terminal pro-brain natriuretic peptide; SD: Standard Deviation; Tc-DPD: technetium-99m-labeled 3,3-diphosphono-1,2-propanodicarboxylic acid.
本研究旨在描述转甲状腺素蛋白淀粉样心肌病（ATTR-CM）患者晕厥的临床特征、潜在病因和结局。
我们回顾了 2018 年 1 月至 2020 年 6 月期间，在加利西亚（西班牙）7 家医院的前瞻性多中心登记处纳入的 128 名 ATTR-CM 患者的临床特征和晕厥发作的潜在病因。登记后，对患者进行中位随访 520 天。通过多变量 Cox 回归评估晕厥对全因死亡率的影响。
30 名（23.4%）患者在登记进入登记处的前瞻性阶段之前有晕厥的既往病史，4 名（3.1%）此后出现首次晕厥发作。在登记后前瞻性随访期间，晕厥的估计发生率密度为 71.9 例/1000 患者年（95%置信区间 32.8-111.1）。总体晕厥患病率估计为 26.6%（95%置信区间 18.9%-34.2%）。心律失常（n=11，32.3%）、心脏或大血管结构疾病（n=5，14.7%）、神经介导反射（n=6，17.6%）和体位性低血压（n=4，11.8%）被认为是晕厥的可能潜在病因；在 8 名（23.6%）患者中，晕厥原因仍未明确。与无晕厥的患者相比，有晕厥的患者未校正的全因死亡率更高（单因素风险比 3.37；95%置信区间 1.43-7.94）。当将其他生存的独立预测因素添加到生存模型中时，这种关联不再具有统计学意义（多因素风险比 1.81，95%置信区间 0.67-4.84）。
晕厥在 ATTR-CM 患者中很常见。本研究未能证明晕厥与这些患者的死亡率之间存在独立关联。ATTR-CM：转甲状腺素蛋白淀粉样心肌病；CI：置信区间；HF：心力衰竭；HR：风险比；IQR：四分位间距；LVEF：左心室射血分数；NTproBNP：N 末端脑利钠肽前体；SD：标准差；Tc-DPD：锝-99m 标记 3,3-二膦酸-1,2-丙二醇。
