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

Francesca Perego, Beatrice De Maria, Maria Bova, Angelica Petraroli, Azzurra Marcelli Cesoni, Valeria De Grazia, Lorenza Chiara Zingale, Alberto Porta, Giuseppe Spadaro, Laura Adelaide Dalla Vecchia

Research output: Contribution to journalArticlepeer-review


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 brady-kinin. C1-INH-HAE directly influences patients’ everyday lives, as attacks are unpredictable in fre-quency, 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.

Original languageEnglish
Article number2900
Pages (from-to)1-10
Number of pages10
JournalInternational Journal of Environmental Research and Public Health
Issue number6
Publication statusPublished - Mar 2 2021


  • Auto-nomic nervous system
  • Burden of disease
  • C1-inhibitor hereditary angioedema
  • Cardiac neural control
  • Heart rate variability
  • Multiday ECG monitoring
  • Quality of life
  • Spectral analysis

ASJC Scopus subject areas

  • Pollution
  • Public Health, Environmental and Occupational Health
  • Health, Toxicology and Mutagenesis


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