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
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
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
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
Holzer RJ et al: Pulmonary arteriovenous malformations and risk of stroke. Cardiol Clin. 34(2):241-6, 2016
Greben CR et al: Pulmonary arteriovenous malformation embolization: how we do it. Tech Vasc Interv Radiol. 16(1):39-44, 2013
Shiga Y et al: Pulmonary artery aneurysm detected by 64-MDCT. Intern Med. 50(7):785, 2011
Banovac F et al: Reporting standards for endovascular treatment of pulmonary embolism. J Vasc Interv Radiol. 21(1):44-53, 2010
Hoag JB et al: An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope. 120(4):838-43, 2010
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
Lin PH et al: Endovascular interventions for acute pulmonary embolism. Perspect Vasc Surg Endovasc Ther. 22(3):171-82, 2010
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
Trerotola SO et al: PAVM embolization: an update. AJR Am J Roentgenol. 195(4):837-45, 2010
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
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
Remy-Jardin M et al: Spiral CT angiography of the pulmonary circulation. Radiology. 212(3):615-36, 1999
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
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
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
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
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
Holzer RJ et al: Pulmonary arteriovenous malformations and risk of stroke. Cardiol Clin. 34(2):241-6, 2016
Greben CR et al: Pulmonary arteriovenous malformation embolization: how we do it. Tech Vasc Interv Radiol. 16(1):39-44, 2013
Shiga Y et al: Pulmonary artery aneurysm detected by 64-MDCT. Intern Med. 50(7):785, 2011
Banovac F et al: Reporting standards for endovascular treatment of pulmonary embolism. J Vasc Interv Radiol. 21(1):44-53, 2010
Hoag JB et al: An epistaxis severity score for hereditary hemorrhagic telangiectasia. Laryngoscope. 120(4):838-43, 2010
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
Lin PH et al: Endovascular interventions for acute pulmonary embolism. Perspect Vasc Surg Endovasc Ther. 22(3):171-82, 2010
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
Trerotola SO et al: PAVM embolization: an update. AJR Am J Roentgenol. 195(4):837-45, 2010
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
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
Remy-Jardin M et al: Spiral CT angiography of the pulmonary circulation. Radiology. 212(3):615-36, 1999
Perlmutt LM et al: Pulmonary arteriography in the high-risk patient. Radiology. 162(1 Pt 1):187-9, 1987
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