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Radiologic Imaging and Treatment of Pulmonary AVMs
Deborah VintonGillian Lieberman M.D.
March 2008
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Clinical Presentation of Pt A
• 34 yo female with acute onset of R sided hemiparesis and aphasia
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Pt A: Head CT on Presentation
PACS, BIDMC
Head CT without contrast showed a hyperdense L MCA sign
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Pt A: Occlusion of L MCA and ICA
Completed occluded L ICA and L MCA
PACS, BIDMC
Decreased blood flow in are of L MCA
PACS, BIDMC
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Pt A Percutaneous Clot Retrieval
IA tPA administeredMERCI clot retriever deployed
http://www.med.cornell.edu/science/2006/03_ 06/03_06-2.shtml
Good flow achieved through L ICA and MCA
PACS, BIDMC
Angio revealed clot extending from L ICA into L MCA
PACS, BIDMC
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Etiology of Thrombotic Infarct Most Likely < 40yo• Carotid or Vertebral Dissection• Underlying coagulopathy
– Protein S or C Def, Antithrombin III def, Factor V Leiden, Antiphospholipid syndrome
Less Likely •Congenital Heart Defect
– PFO, ASD, PDA• Prolonged immobility• Neoplastic Process• Collagen vascular disorders
– SLE, RA, Sjogrens, FMD, Myositis• Trauma
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Menu of Tests For Pt Work-Up• Most Common
– CTA Neck w & w/out contrast – ECG– ECHO with agitated saline– MR Head w/o Contrast
• Less Common– Chest X-ray– Venous Duplex Lower Extremities
Bilaterally– CT Chest, Abdomen, Pelvis w/ contrast
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Pt A: Opacity in R Lung Base
PACS, BIDMC
• ET tube tip is 3 cm about carina
• Lungs w/ minimal atelectasis at the L base
• Rounded opacity in R cardiophrenic angle– 3 cm in diameter
projecting mostly over R lung base
• Linear opacity proximal to R hilus
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Pt A: AVM on Contrast CT
PACS, BIDMC
• Homogenously enhancing structure in R middle lobe adjacent to pericardial fat
• Large feeding vessel arising from pulmonary artery approaches lesion
• Findings consistent with large pulmonary arteriovenous malformation (AVM)
• AVM enhances homogenously w/out evidence of thrombus
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Pt A: Pulmonary Arteriovenous Malformation (PAVM)
• Aberrant connection between pulmonary artery and venous circulation that bypasses capillary system
• High flow low resistance
• 50-70% of PAVMs are located in the lower lobes
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Normal Anatomy vs PAVM
http://galileo.phys.virginia.edu/classes/304/heart3.gif
http://www.med.yale.edu/intmed/cardio/imaging/findings/pulmonary_angio_a/ graphics/rad1.gif
• Pulmonary angiogram demonstrated substantial aneurysmal dilation of interlobar branch of with finding consistent with a large AVM
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Complications from PAVM
• Hemorrhage: Hemoptysis or Hemothorax
• Massive R to L shunting: Hypoxemia, Dyspnea, Clubbing, Cyanosis, Polycythemia
• Paradoxical Emboli: cerebral abscess, embolic stroke, TIA’s– Serious neurological complications occur in up to 35%
of pt w/ PAVM*
*Cottin V et al. Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore) 2007 Jan;86(1):1-17
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Treatment• AVM > 3 mm require tx• Transcatheter Embolotherapy
(TCE) is treatment of choice– avoids major surgery and
general anesthesia – loss of lung parenchyma
• TCE Procedure: Coils are threaded through a catheter into the pulmonary artery and placed both distally in the draining vein and proximally in the feeding artery of the AVM
http://www.ispub.com/xml/journals/ijpm/vol5n2/pavm-fig4.jpg
Companion Patient #1
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Treatment Complications• Most Common:
– Pleuritic chest pain – Paradoxic embolization– Air emboli– Recanalization
• Less Common– Infection– Pulmonary infarction– Pulmonary Hypertension– Deep venous thrombosis– Dislocation of coils– Bleeding http://www.ispub.com/xml/journals/ijpm/vol5n2/pavm-fig4.jpg
Companion Patient #1
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Pt A: Treatment
• Substantial dilation of interlobar branch appreciated on angiogram
• Feeding artery unsuitable for secure placement of balloons or coils due to size and high flow
• 10mm Amplatz occluder device deployed • No filling or arterial phase opacification of the AV on
follow-up angiogram
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PACS, BIDMC
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DDx: Pulmonary AVM• Most Common:
– Hereditary Hemorragic Telangiectasia (HHT) (80%)• Less Common:
– Chronic Inflammatory Conditions– Hepatic Cirrhosis– Mitral Stenosis– Schistosomiasis– Actinomyces– Trauma– Fanconi’s Syndrome– Metastic Thyroid Carcinoma
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Hereditary Hemorrhagic Telangiectasia• Autosomal Dominant:
– Incidence 1/10,000 – Varying penetrance– Mutations in genes encoding Endoglin or
ALK-1, both membrane receptors of TGF-ß
• Diagnostic Criteria:1. Spontaneous, recurrent epistaxis2. Multiple mucocutaneous telangiectasias3. Visceral involvement4. 1° relative with HHT
Amin H. Pulmonary Arteriovenous Malformations And Osler Weber Rendu Syndrome: An Unusual Cause Of Dyspnea. The Internet Journal of Pulmonary Medicine. Dec. 2007 ISSN: 1531-2984.
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Clinicial Features of HHT• Recurrrent Epistaxis (95%)• Multiple mucocutaneous
telangiectasias (75%) – lips, hands, face
• GI bleeding (20-25%)• AVMs
– Pulmonary (30-50%)– Cerebral (5-20%)– Hepatic (up to 30%)*
• Fe deficiency anemia
Maher et al. Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia. Stroke. 2001;32: 877-882.
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PAVMs: Summary• Morbidity and mortality
– embolic stroke, brain abscess, and intracranial hemorrhage
• HHT is the most common etiology for PAVM– Patients and 1º relatives should be screened
• Embolotherapy or surgery is required for:– Symptomatic patients– PAVM with feeding arteries > 3 mm in
diameter
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References• Plauchu H; de Chadarevian JP; Bideau A; Robert JM . Age-related clinical profile of hereditary hemorrhagic
telangiectasia in an epidemiologically recruited population. Am J Med Genet 1989 Mar;32(3):291-7• Naess, H., Nyland, H. I., Thomassen, L., Aarseth, J. & Myhr, K.-M. Etiology of and risk factors for cerebral
infarction in young adults in western Norway: a population-based case-control study. European Journal of Neurology 11 (1), 25-30.
• Flinte et al. Mechanical Thrombectomy of Intracranial Internal Carotid Occlusion Pooled Results of the MERCI and Multi MERCI Part I Trials. Stoke. 38 (4): 1274. (2007)
• Amin H. Pulmonary Arteriovenous Malformations And Osler Weber Rendu Syndrome: An Unusual Cause Of Dyspnea. The Internet Journal of Pulmonary Medicine. Dec. 2007 ISSN: 1531-2984.
• Cottin V, Chinet T, Lavole A, Corre, Marchand E, Renyaud-Gaubert M, Plauchu H, Cordier JF. Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore) 2007 Jan;86(1):1-17
• Cottin V et al. Pulmonary Vascular Manifestations of Hereditary Hemorrhagic Telangiectasia (Rendu-Osler Disease)Respiration. 2007;74(4):361-78.
• Maher et al. Cerebrovascular Manifestations in 321 Cases of Hereditary Hemorrhagic Telangiectasia. Stroke. 2001;32: 877-882.
• Andersen PE, Kjeldsen A. Clinical and Radiological Long-Term Follow-up After Embolization of Pulmonary Arteriovenous Malformations. Cardiovasc Intervent Radiology. 2006 Jan-Feb;29(1):70-4
• Kjeldsen AD, Oxoj H, Andersen PE, Green A, Vase P. Prevalence of pulmonary arteriovenous malformations (PAVMs) and occurrence of neurological symptoms in patients with hereditary haemorrhagic telangiectasia (HHT). Journal of Internal Medicine [0954-6820] 2000 vol:248 iss:3 pg:255 -6
• Hong Shan Zhong. Ke Xu. Haibo Shao .Transcatheter Closure of Bilateral Multiple Huge Pulmonary Arteriovenous Malformations with Homemade Double-Umbrella Occluders.. Cardiovasc Interventional radiology. 2007 Dec 7
• Gamillscheg, A, Schuchlenz H, Stein JI, Beitzke .Interventional Occlusion of a Large Pulmonary Arteriovenous Malformation with an Amplatzer Septal Occluder . A Journal of Interventional Cardiology Volume 16 Issue 4 Page 335-339, August 2003
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Acknowledgements
• Special thanks to:• Andrew Hines-Peralta, MD• Aarti Sekhar, MD• Prachi Dubey, MD• Gillian Lieberman, MD