Analysis of heart-rate variability during angioedema attacks in patients with hereditary c1-inhibitor deficiency

Database from the article Perego F, De Maria B, Bova M, Petraroli A, Marcelli Cesoni A, De Grazia V, Zingale LC, Porta A, Spadaro G, Dalla Vecchia LA. Analysis of Heart-Rate Variability during Angioedema Attacks in Patients with Hereditary C1-Inhibitor Deficiency. Int J Environ Res Public Health. 2021 Mar 12;18(6):2900. doi: 10.3390/ijerph18062900. PMID: 33809031; PMCID: PMC8002127.

Abstract

C1-inhibitor hereditary angioedema (C1-INH-HAE) is a rare disease characterized by self-limiting edema associated with localized vasodilation due to increased levels of circulating bradykinin. C1-INH-HAE directly influences patients' everyday lives, as attacks are unpredictable in frequency, severity, and the involved anatomical site. The autonomic nervous system could be involved in remission. The cardiac autonomic profile has not yet been evaluated during the attack or prodromal phases. In this study, a multiday continuous electrocardiogram was obtained in four C1-INH-HAE patients until attack occurrence. Power spectral heart rate variability (HRV) indices were computed over the 4 h preceding the attack and during the first 4 h of the attack in three patients. Increased vagal modulation of the sinus node was detected in the prodromal phase. This finding may reflect localized vasodilation mediated by the release of bradykinin. HRV analysis may furnish early markers of an impending angioedema attack, thereby helping to identify patients at higher risk of attack recurrence. In this perspective, it could assist in the timing, titration, and optimization of prophylactic therapy, and thus improve patients' quality of life.

本数据集源自论文Perego F、De Maria B、Bova M、Petraroli A、Marcelli Cesoni A、De Grazia V、Zingale LC、Porta A、Spadaro G、Dalla Vecchia LA的研究成果：《遗传性C1酯酶抑制剂缺乏症患者血管性水肿发作期间的心率变异性分析》，刊载于《国际环境研究与公共卫生杂志》（International Journal of Environmental Research and Public Health），2021年3月12日出版，第18卷第6期，文章编号2900，DOI: 10.3390/ijerph18062900，PMID: 33809031；PMCID: PMC8002127。

### 摘要
C1酯酶抑制剂遗传性血管性水肿（C1-INH-HAE）是一种罕见疾病，以循环缓激肽水平升高引发局部血管舒张，进而出现自限性水肿为典型特征。该病发作的频率、严重程度及受累解剖部位均难以预判，直接严重影响患者的日常生活。目前已有研究推测自主神经系统可能参与疾病的缓解过程，但此前尚未在发作期或前驱期对心脏自主神经特征展开系统评估。本研究对4名C1-INH-HAE患者实施多日持续心电图（ECG）监测，直至其出现血管性水肿发作。针对其中3名患者，研究人员计算了发作前4小时与发作最初4小时内的功率谱心率变异性（HRV）指标。结果在患者的前驱期检测到窦房结迷走神经调控增强，这一发现或可反映缓激肽释放介导的局部血管舒张反应。心率变异性分析可作为血管性水肿即将发作的早期标志物，助力识别发作复发风险更高的患者，进而辅助优化预防性治疗的时机、剂量与方案，最终显著改善患者的生活质量。