Telangiectatic Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia.

Abstract

Background There are three classic types of pulmonary arteriovenous malformations (PAVMs): simple, complex, and diffuse. Ground-glass opacities (GGOs) with associated feeding arteries or draining veins in patients with hereditary hemorrhagic telangiectasia (HHT) may represent a fourth distinct type of PAVMs-so-called telangiectatic PAVMs (tPAVMs)-which have been variably described but have not been formally studied. Purpose To describe the prevalence, clinical features, and imaging characteristics of tPAVMs in an adult cohort of patients with HHT. Materials and Methods This retrospective observational study was conducted from May 2019 to December 2021 using clinical records and chest CT scans from consecutive adult patients from two HHT centers who had suspected or definite HHT. All nonembolized lesions 3 mm or greater in maximum diameter with an identifiable feeding artery, draining vein, or both were classified into one of three categories: tPAVMs (>70% GGO by volume), conventional PAVMs (>70% vascular density), or mixed lesions (neither GGO nor vascular density >70%). Lesion characteristics at chest CT were compared using the Kruskal-Wallis test. Comparisons of patients according to lesion and tPAVM status were conducted using the Fisher exact test for categorical variables and the Wilcoxon rank sum or Kruskal-Wallis test for continuous variables. Results Of 100 patients with HHT (median age, 56 years [IQR, 37-66 years]; 72 [72%] female patients) who underwent chest CT, 35 had tPAVMs, 40 had PAVMs but not tPAVMs, and 25 had no lesions. There was no evidence of a difference in genotype status across groups (<i>P</i> = .40). There was a difference in sex distribution across groups (no tPAVM, 60% female patients; any tPAVM, 74% female patients; no lesion, 88% female patients; <i>P</i> = .048). Among six patients who had tPAVMs but no conventional PAVMs, mild to moderate pulmonary shunt was observed at transthoracic contrast echocardiography in all six (100%). Conclusion tPAVMs, marked by vascular GGOs, are common in HHT and constitute a fourth type of PAVM. © The Author(s) 2026. Published by the Radiological Society of North America under a CC BY 4.0 license. <i>Supplemental material is available for this article.</i>