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Pulmonary Arteriovenous Malformations
Jennifer R. Buckley, MD, MBA; Christopher Bailey, MD; Clifford R. Weiss, MD, FSIR, FCIRSE
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KEY FACTS

  • Terminology

    • Procedure

      • Post Procedure

        • Outcomes

          TERMINOLOGY

          • Definitions

            • Pulmonary arteriovenous malformation (PAVM): High-flow, low-resistance direct fistulous communication between pulmonary artery and vein
              • Causes right-to-left shunt
                • May be asymptomatic if small
                • May be symptomatic if larger
                  • Pulmonary-related symptoms may include dyspnea, cyanosis, hemoptysis, polycythemia
                  • PAVMs are potential source for paradoxic emboli; can cause stroke, brain abscess
              • PAVM classification
                • Simple PAVM: Single segmental feeding artery
                  • May have multiple subsegmental arterial branches to malformation
                • Complex PAVM: Multiple segmental feeding arteries perfuse malformation
                • Diffuse PAVMs: Hundreds of malformations (or more)
                  • Estimated to occur in 5% of PAVM patients
                  • May require lung transplant for treatment
              • 80-90% occur in patients with hereditary hemorrhagic telangiectasia (HHT)
              • Acquired causes include trauma, malignancy, hepatopulmonary syndrome, and cardiac surgery
            • HHT: Autosomal dominant genetic abnormality causing development of arteriovenous shunts
              • a.k.a. Osler-Weber-Rendu syndrome
              • May affect nasal mucosa, skin, gastrointestinal tract, lung, CNS
                • Lung manifestations: PAVMs
                  • PAVMs occur in 15-25% of patients with HHT
                  • 60% of HHT patients who have PAVMs have multiple lesions
                  • Untreated PAVMs can predispose patients to increased risk for cerebral abscess, transient ischemic attack, stroke
                  • Screen for PAVMs using transthoracic agitated saline echocardiography bubble study
                  • Can also screen using CTA (include liver to assess for liver AVM)
              • 4 major diagnostic criteria for HHT
                • Epistaxis (main presenting symptom in HHT)
                  • Measured by epistaxis severity score
                • Telangiectasias/arteriovenous shunts
                • Visceral lesions
                • Family history of 1st-degree relative with HHT
              • HHT likelihood by number of criteria present
                • 3 criteria present: Definite
                • 2 criteria present: Possible
                • 1 criterion present: Unlikely

          PREPROCEDURE

          • Indications

            • Contraindications

              • Preprocedure Imaging

                • Getting Started

                  PROCEDURE

                  • Patient Position/Location

                    • Procedure Steps

                      • Alternative Procedures/Therapies

                        POST PROCEDURE

                        • Postprocedure Imaging

                          OUTCOMES

                          • Complications

                            • Expected Outcomes

                              Selected References

                              1. Hong J et al: Iatrogenic pulmonary artery perforation associated with 5-Fr catheter manipulation during pulmonary arteriovenous malformation embolization with a vascular plug. Radiol Case Rep. 17(3):970-3, 2022
                              2. Nagai K et al: Venous sac and feeding artery embolization versus feeding artery embolization alone for treating pulmonary arteriovenous malformations: draining vein size outcomes. J Vasc Interv Radiol. 32(7):1002-8, 2021
                              3. Roberts DG et al: Comparison of feeding-artery-only versus nidus-plus-feeding-artery embolization of pulmonary arteriovenous malformations. J Vasc Interv Radiol. 32(7):993-1001, 2021
                              4. Holzer RJ et al: Pulmonary arteriovenous malformations and risk of stroke. Cardiol Clin. 34(2):241-6, 2016
                              5. Greben CR et al: Pulmonary arteriovenous malformation embolization: how we do it. Tech Vasc Interv Radiol. 16(1):39-44, 2013
                              6. Shiga Y et al: Pulmonary artery aneurysm detected by 64-MDCT. Intern Med. 50(7):785, 2011
                              7. Banovac F et al: Reporting standards for endovascular treatment of pulmonary embolism. J Vasc Interv Radiol. 21(1):44-53, 2010
                              8. Hoag JB et al: An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope. 120(4):838-43, 2010
                              9. Letourneau-Guillon L et al: Embolization of pulmonary arteriovenous malformations with amplatzer vascular plugs: safety and midterm effectiveness. J Vasc Interv Radiol. 21(5):649-56, 2010
                              10. Lin PH et al: Endovascular interventions for acute pulmonary embolism. Perspect Vasc Surg Endovasc Ther. 22(3):171-82, 2010
                              11. Trerotola SO et al: Does use of coils in addition to amplatzer vascular plugs prevent recanalization? AJR Am J Roentgenol. 195(3):766-71, 2010
                              12. Trerotola SO et al: PAVM embolization: an update. AJR Am J Roentgenol. 195(4):837-45, 2010
                              13. Kuo WT et al: Catheter-directed therapy for the treatment of massive pulmonary embolism: systematic review and meta-analysis of modern techniques. J Vasc Interv Radiol. 20(11):1431-40, 2009
                              14. Nguyen ET et al: Pulmonary artery aneurysms and pseudoaneurysms in adults: findings at CT and radiography. AJR Am J Roentgenol. 188(2):W126-34, 2007
                              15. Remy-Jardin M et al: Spiral CT angiography of the pulmonary circulation. Radiology. 212(3):615-36, 1999
                              16. Perlmutt LM et al: Pulmonary arteriography in the high-risk patient. Radiology. 162(1 Pt 1):187-9, 1987
                              Related Anatomy
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                              Related Differential Diagnoses
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                              References
                              Tables

                              Tables

                              KEY FACTS

                              • Terminology

                                • Procedure

                                  • Post Procedure

                                    • Outcomes

                                      TERMINOLOGY

                                      • Definitions

                                        • Pulmonary arteriovenous malformation (PAVM): High-flow, low-resistance direct fistulous communication between pulmonary artery and vein
                                          • Causes right-to-left shunt
                                            • May be asymptomatic if small
                                            • May be symptomatic if larger
                                              • Pulmonary-related symptoms may include dyspnea, cyanosis, hemoptysis, polycythemia
                                              • PAVMs are potential source for paradoxic emboli; can cause stroke, brain abscess
                                          • PAVM classification
                                            • Simple PAVM: Single segmental feeding artery
                                              • May have multiple subsegmental arterial branches to malformation
                                            • Complex PAVM: Multiple segmental feeding arteries perfuse malformation
                                            • Diffuse PAVMs: Hundreds of malformations (or more)
                                              • Estimated to occur in 5% of PAVM patients
                                              • May require lung transplant for treatment
                                          • 80-90% occur in patients with hereditary hemorrhagic telangiectasia (HHT)
                                          • Acquired causes include trauma, malignancy, hepatopulmonary syndrome, and cardiac surgery
                                        • HHT: Autosomal dominant genetic abnormality causing development of arteriovenous shunts
                                          • a.k.a. Osler-Weber-Rendu syndrome
                                          • May affect nasal mucosa, skin, gastrointestinal tract, lung, CNS
                                            • Lung manifestations: PAVMs
                                              • PAVMs occur in 15-25% of patients with HHT
                                              • 60% of HHT patients who have PAVMs have multiple lesions
                                              • Untreated PAVMs can predispose patients to increased risk for cerebral abscess, transient ischemic attack, stroke
                                              • Screen for PAVMs using transthoracic agitated saline echocardiography bubble study
                                              • Can also screen using CTA (include liver to assess for liver AVM)
                                          • 4 major diagnostic criteria for HHT
                                            • Epistaxis (main presenting symptom in HHT)
                                              • Measured by epistaxis severity score
                                            • Telangiectasias/arteriovenous shunts
                                            • Visceral lesions
                                            • Family history of 1st-degree relative with HHT
                                          • HHT likelihood by number of criteria present
                                            • 3 criteria present: Definite
                                            • 2 criteria present: Possible
                                            • 1 criterion present: Unlikely

                                      PREPROCEDURE

                                      • Indications

                                        • Contraindications

                                          • Preprocedure Imaging

                                            • Getting Started

                                              PROCEDURE

                                              • Patient Position/Location

                                                • Procedure Steps

                                                  • Alternative Procedures/Therapies

                                                    POST PROCEDURE

                                                    • Postprocedure Imaging

                                                      OUTCOMES

                                                      • Complications

                                                        • Expected Outcomes

                                                          Selected References

                                                          1. Hong J et al: Iatrogenic pulmonary artery perforation associated with 5-Fr catheter manipulation during pulmonary arteriovenous malformation embolization with a vascular plug. Radiol Case Rep. 17(3):970-3, 2022
                                                          2. Nagai K et al: Venous sac and feeding artery embolization versus feeding artery embolization alone for treating pulmonary arteriovenous malformations: draining vein size outcomes. J Vasc Interv Radiol. 32(7):1002-8, 2021
                                                          3. Roberts DG et al: Comparison of feeding-artery-only versus nidus-plus-feeding-artery embolization of pulmonary arteriovenous malformations. J Vasc Interv Radiol. 32(7):993-1001, 2021
                                                          4. Holzer RJ et al: Pulmonary arteriovenous malformations and risk of stroke. Cardiol Clin. 34(2):241-6, 2016
                                                          5. Greben CR et al: Pulmonary arteriovenous malformation embolization: how we do it. Tech Vasc Interv Radiol. 16(1):39-44, 2013
                                                          6. Shiga Y et al: Pulmonary artery aneurysm detected by 64-MDCT. Intern Med. 50(7):785, 2011
                                                          7. Banovac F et al: Reporting standards for endovascular treatment of pulmonary embolism. J Vasc Interv Radiol. 21(1):44-53, 2010
                                                          8. Hoag JB et al: An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope. 120(4):838-43, 2010
                                                          9. Letourneau-Guillon L et al: Embolization of pulmonary arteriovenous malformations with amplatzer vascular plugs: safety and midterm effectiveness. J Vasc Interv Radiol. 21(5):649-56, 2010
                                                          10. Lin PH et al: Endovascular interventions for acute pulmonary embolism. Perspect Vasc Surg Endovasc Ther. 22(3):171-82, 2010
                                                          11. Trerotola SO et al: Does use of coils in addition to amplatzer vascular plugs prevent recanalization? AJR Am J Roentgenol. 195(3):766-71, 2010
                                                          12. Trerotola SO et al: PAVM embolization: an update. AJR Am J Roentgenol. 195(4):837-45, 2010
                                                          13. Kuo WT et al: Catheter-directed therapy for the treatment of massive pulmonary embolism: systematic review and meta-analysis of modern techniques. J Vasc Interv Radiol. 20(11):1431-40, 2009
                                                          14. Nguyen ET et al: Pulmonary artery aneurysms and pseudoaneurysms in adults: findings at CT and radiography. AJR Am J Roentgenol. 188(2):W126-34, 2007
                                                          15. Remy-Jardin M et al: Spiral CT angiography of the pulmonary circulation. Radiology. 212(3):615-36, 1999
                                                          16. Perlmutt LM et al: Pulmonary arteriography in the high-risk patient. Radiology. 162(1 Pt 1):187-9, 1987