Pulmonary arteriovenous malformations: what the interventional radiologist needs to know

Radiol Med. 2019 Oct;124(10):973-988. doi: 10.1007/s11547-019-01051-7. Epub 2019 Jun 17.

Abstract

Pulmonary arteriovenous malformations (PAVMs) or fistulas are rare direct pathological connections between pulmonary arterial and venous circulation. Most of PAVMs are congenital and closely associated with hereditary hemorrhagic telangiectasia, but acquired PAVMs have also been described in the literature. Diagnosis of PAVMs is a priority for clinicians, in order to prevent potentially fatal events such as cerebrovascular stroke, systemic septic embolization, hemoptysis and hemothorax. In this scenario, the radiologist plays a key role in both diagnostic and therapeutic workups of PAVMs: Chest X-ray, computed tomography and magnetic resonance are effective tools for PAVMs identification and confirmation of the suspected diagnosis. Furthermore, imaging modalities provide most of the elements for PAVMs classification according to their angioarchitecture (simple and complex) and help the clinicians in establishing which lesion requires prompt treatment and which one will benefit of imaging follow-up alone. Endovascular management of PAVMs has grown up as the first-line treatment in respect of surgery during last decades, showing lower risk of intra- and post-procedural complications and offering a wide number of treatment options and materials, ensuring effective management in virtually any clinical situation; interventional treatment aims to exclude PAVMs from pulmonary circulation, and specific technique and embolic agents should be selected according to pre-treatment imaging, in order to obtain the best procedural outcome. This paper proposes a review of the clinical and radiological features that a radiologist needs to know for PAVMs diagnosis and proper management, also showing an overview of the most common endovascular treatment strategies and embolization materials.

Keywords: CT imaging; Endovascular treatment; Hereditary hemorrhagic telangiectasia; Osler–Weber–Rendu disease; Pulmonary arteriovenous fistula; Pulmonary arteriovenous malformation.

Publication types

  • Review

MeSH terms

  • Arteriovenous Fistula / diagnostic imaging*
  • Arteriovenous Fistula / therapy*
  • Arteriovenous Malformations / diagnostic imaging*
  • Arteriovenous Malformations / therapy*
  • Diagnosis, Differential
  • Humans
  • Pulmonary Artery / abnormalities*
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Veins / abnormalities*
  • Pulmonary Veins / diagnostic imaging
  • Radiography, Interventional*

Supplementary concepts

  • Pulmonary Arteriovenous Fistulas