Presence of Non-Cerebral Arteriovenous Malformations Increases the Likelihood of Intracranial AVMs, Regardless of Hereditary Hemorrhagic Telangiectasia Status

Introduction: Hereditary hemorrhagic telangiectasia (HHT) is linked with a predisposition to arteriovenous malformations (AVMs) in various organs, including the brain. While intracranial AVMs (bAVMs) are documented in HHT, the relationship between extracranial AVMs (EC-AVMs) and bAVMs in non-HHT patients remains unclear. This study examines whether EC-AVMs increase the likelihood of bAVMs, irrespective of HHT status.

Methods: A retrospective cohort study was conducted using data from the 2019 National Inpatient Sample, capturing a representative 20% of U.S. hospital discharges. Patients with ICD-10 codes for AVMs, including specific types of EC-AVMs and HHT, were identified. Odds ratios (ORs) and 95% confidence intervals (CIs) for bAVMs associated with EC-AVMs were calculated using multivariate logistic regression, adjusting for HHT, age, and sex.

Results: From 35,419,023 admissions, 12,500 patients had any form of EC-AVMs (35.27 per 100k), with peripheral and digestive system AVMs being most common. HHT patients showed a higher prevalence of EC-AVMs (12.2%) than non-HHT patients (0.034%). Among bAVM patients, congenital pulmonary AVMs (OR = 3.733; 95% CI: 2.747–4.719; p < 0.001), peripheral AVMs (OR = 2.675; 95% CI: 1.958–3.392; p < 0.001), and any EC-AVMs (OR = 3.294; 95% CI: 2.815–3.774; p < 0.001) were associated with increased odds of bAVMs. These associations remained significant after excluding HHT patients and adjusting for HHT, with congenital pulmonary AVMs (OR = 1.806; 95% CI: 0.585–3.026; p = 0.004) and peripheral AVMs (OR = 2.675; 95% CI: 1.958–3.391; p < 0.001) showing persistent associations.

Conclusion : The presence of EC-AVMs significantly elevates the likelihood of bAVMs, independent of HHT status. These findings suggest a systemic predisposition to AVMs across multiple organ systems, supporting the need for vigilant screening of bAVMs in patients presenting with EC-AVMs, regardless of HHT diagnosis.