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Arteriovenous
Malformations in Osler Weber‐Rendu:
A Patient StudyKush M Parmar, Harvard Medical School Year III
Gillian Lieberman, MD
Kush M Parmar, HMS IIIGillian Lieberman, MD
September 2008
Objectives
Kush M Parmar, HMS IIIGillian Lieberman, MD
• Learn what an arteriovenous
malformation (AVM) is.
• Learn imaging modalities that can be used to image AVM.
• Learn the typical appearance of AVM.
• Appreciate the clinical manifestations of AVM in Osler‐Weber‐Rendu/HHT.
2
Mrs. R: History
Kush M Parmar, HMS IIIGillian Lieberman, MD
3
• 37 y/o
woman w/ R. MCA ischemic stroke
• Coagulation studies were negative/normal
• No ASD/PFO (potential cause of paradoxical embolism)
• “Cryptogenic stroke”
presentation (no known cause)
Mrs. R: AP CXR
Kush M Parmar, HMS IIIGillian Lieberman, MD
Rounded opacity at R. cardiophrenic angle
Linear opacity extending between rounded opacity and hilum
PACS/BIDMC
FINDINGS
4
Findings suggestive of PAVM
Kush M Parmar, HMS IIIGillian Lieberman, MD
General Architecture of Arteriovenous
Malformations (AVM)
‐ Dilated, tortuous feeding and draining vessels ‐ A “nidus”
or “serpiginous”
mass of malformed vessels‐ Diminished oxygen diffusion (A‐V shunt); abnormal/absent capillary bed‐ Rapid filling and emptying
http://neuro.wehealny.org
5
Imaging Pulmonary AVM: Menu of Tests
Kush M Parmar, HMS IIIGillian Lieberman, MD
•
Pulmonary angiography•
Used mainly for therapeutic
interventions due to invasiveness
•
MR angiography
•
Chest CT (+/‐
C)
•
Gold standard
•
Chest X‐ray•
Only useful for large AVM
•
Transthoracic
Contrast Echo
•
Shunt quantification
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Features of Pulmonary AVM on Chest X‐Ray
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Kush M Parmar, HMS IIIGillian Lieberman, MD
• Round or oval opacity• Uniform density, noncalcified• Often lobulated
but sharply
defined• Location: frequently in lower lobes• Connection: may
show feeding
artery radiating from hilus
PACS/BIDMC
Features of Pulmonary AVM on CT
• Homogeneous, non‐calcified, circumscribed
nodule or serpiginous
mass
• MUST
have a direct feeding artery
– Dilated efferent draining vein• With contrast:
– Sequential enhancement of feeding artery and
aneurysmal
part– Early filling of draining vein (to differentiate from
highly vascular soft tissue mass)
8
Kush M Parmar, HMS IIIGillian Lieberman, MD
Mrs. R: Chest CT
9
Kush M Parmar, HMS IIIGillian Lieberman, MD
feeding branch of pulmonary artery
nidus
of AVM
R. hemidiaphragm
9
C+CT
Mrs. R: Decision to Embolize
With the clear determination of pulmonary AVM on CT, it was decided to proceed with therapeutic embolization.
Kush M Parmar, HMS IIIGillian Lieberman, MD
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11
Kush M Parmar, HMS IIIGillian Lieberman, MD
L. pulmonary artery
catheter in IVC
PACS/BIDMC
Mrs. R: Digital subtraction pulmonary arteriogram
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(L. Lung, normal)
Mrs. R: Digital subtraction pulmonary arteriogram
12
Kush M Parmar, HMS IIIGillian Lieberman, MD
(R. Lung, before therapeutic embolism)
feeding artery
drainingvein
PACS/BIDMC
AVM
R. pulmonary artery
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Therapeutic Embolism of PAVM
13
Kush M Parmar, HMS IIIGillian Lieberman, MD
www.amplatzer.com
14
Mrs. R: Post‐embolizationKush M Parmar, HMS IIIGillian Lieberman, MD
Digital subtraction pulmonary arteriogram(R. Lung, post‐therapeutic embolism)
R. pulmonary artery
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Mrs. R: Post‐embolizationKush M Parmar, HMS IIIGillian Lieberman, MD
Digital subtraction pulmonary arteriogram(R. Lung, post‐therapeutic embolism)
R. pulmonary artery
pre-embolism
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AVM nidus
No filling in embolized
AVM
Differential Diagnosis of Pulmonary AVM
Congenital (~90%)
• Hereditary Hemorrhagic Telangectasia(Osler‐Weber‐Rendu)
– Type I –
EDG1 mutations– Type II –
ALK1 mutations– Other
–
unmapped loci
Acquired
• Highly vascular parenchymal
lesion (compare kinetics
with other vascular structure)16
Kush M Parmar, HMS IIIGillian Lieberman, MD
Clinical Manifestations of Pulmonary AVM
• Dyspnea
on exertion• Hypoxemia
• Stroke• Transient ischemic attack • Cerebral abscess
17
Kush M Parmar, HMS IIIGillian Lieberman, MD
Primary mechanism
right-left shunt
paradoxical embolism( our patient)
Most PAVM are asymptomatic for years, so many are first encountered incidentally on imaging
Screening for Pulmonary AVM in HHT Patients
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Chest Radiographs
Contrast echocardiography
-
-
+
+
Chest CT
Absence of PAVM
Prophylactic antibioticsto prevent brain abscess from paradoxical emboli
Absence of PAVM Positive PAVM
Kush M Parmar, HMS IIIGillian Lieberman, MD
+-
Hepatic AVM: Menu of Tests
19
Kush M Parmar, HMS IIIGillian Lieberman, MD
•
Doppler Color Ultrasound (Echo Color Doppler)•
Low cost
•
No radiation
•
Highly sensitive
•
CT•
Specific, gold standard
Hepatic AVM on CTKush M Parmar, HMS IIIGillian Lieberman, MD
Early Arterial Phase Late Arterial Phase Portal Venous Phase
hepatic veins
AVM• AVM is most apparent in early arterial phase, no longer visible in portal venous phase
• Hepatic veins fill early
Buonamico et al. J Hepatol. 2008 ;48(5):811-2020
C+CT
21
•
Heterogenous
opacification
of liver
‐ Due to many small hypervascular
spots (in HHT patients)
• Early arterial phase contrast filling of AVM
• Hepatic vein enhancement in early arterial
phase‐ Caused by AVM shunt
Hepatic AVM Features on CT
Kush M Parmar, HMS IIIGillian Lieberman, MD
21
•
Hypervascularization: tortuous hepatic arteries‐Highly visible main intrahepatic
branches of the hepatic artery in the
portal spaces.‐Shunt determines tortuosity
and enlargement of these vessels.
Hepatic AVM Features on US
Kush M Parmar, HMS IIIGillian Lieberman, MD
EPA=hepatic arteryPV=portal vein
Buonamico et al. J Hepatol. 2008 ;48(5):811-2022
22
Hepatic AVM Features on US
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• Intrahepatic
“color spots”‐The presence of subcapsular
vascular spots near the hepatic
capsule consisting mainly of arterial blood flow.
Buonamico et al. J Hepatol. 2008 ;48(5):811-20
Kush M Parmar, HMS IIIGillian Lieberman, MD
23
Osler‐Weber‐Rendu/ Hereditary Hemorrhagic
Telangiectasia
(HHT)• One of the most common monogenic diseases (~1 in 5000‐
10000)
• Progressive clinical manifestations over decades
• Epistaxis– Usually 1st
manifestation, ~10 yr of age
• Telangiectasia– Onset at ~3rd
decade of life– Lips, tongue, palate, fingers, and face
24
Kush M Parmar, HMS IIIGillian Lieberman, MD
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Diagnosis of HHT
• Curacao criteria• Definite diagnosis with 3 out of 4 criteria
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Kush M Parmar, HMS IIIGillian Lieberman, MD
Criterium Description
Epistaxis Spontaneous, recurrent nose bleeds; often first
manifestation
Telangiectasias Multiple, at characteristic sites: lips, oral cavity,
fingers, nose
Visceral lesions Gastrointestinal telangiectasias
(+/‐
bleeding)Pulmonary AVMHepatic AVMCerebral AVMSpinal AVM
Family history A first‐degree relative with HHT according to these
criteria
25
Gossage
et al. Am J Respir
Crit
Care Med 1998; 158: 643‐661
Likely locations of AVM
26
Kush M Parmar, HMS IIIGillian Lieberman, MD
‐
lung‐ liver‐ brain‐ spinal cord‐ spleen‐
kidney
‐
spermatic cord
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Osler‐Weber‐
Rendu/HHT
Idiopathic/Other
Imaging AVM: Summary
27
1. Menu of Tests to Image AVM is Organ‐specific• Pulmonary: CT, Angiography (for interventions)• Hepatic: CT, US
2. Typical architecture of AVM:• Entirely vascular structure that ‘shortcuts’
capillaries
• Dilated, tortuous feeding and draining vessels• Rapid filling and draining of contrast
Kush M Parmar, HMS IIIGillian Lieberman, MD
3. Detecting an AVM may provide an opportunity to
pre‐emptively
address it and offer genetic counseling
for Osler‐Weber‐Rendu
patients and their family
27
28
References
•
Cottin
V, Dupuis‐Girod
S, Gaetan
L, Cordier
J‐F. Pulmonary Vascular Manifestations of Hereditary
Hemorrhagic Telangiectasia
(Rendu‐Osler Disease). Respiration
2007; 74:361‐378.
•
Gossage
JR, Kanj
G. Pulmonary Arteriovenous
Malformations: A State of the Art Review. Am J
Respir
Crit
Care Med 1998; 158: 643‐661
•
Remy J, Remy‐Jardin
M, Wattine
L, Deffontaines
C. Pulmonary Arteriovenous
Malformations:
Evaluation with CT of the Chest before and after Treatment. Radiology 1992; 182: 809‐816.
•
Buonamico
P, Suppressa
P, Lenato
GM, Pasculli
G, D'Ovidio
F, Memeo
M, Scardapane
A, Sabbà
C.
Liver involvement in a large cohort of patients with hereditary hemorrhagic telangiectasia: echo‐
color‐Doppler vs multislice
computed tomography study. J Hepatol. 2008 ;48(5):811‐20
Kush M Parmar, HMS IIIGillian Lieberman, MD
28
Acknowledgements
• Salomao
Paintuch, MD
• Dmitri J Rabkin, MD
• Gillian Lieberman, MD
• Larry Barbaras (webmaster)
• Maria Levantakis
29
Kush M Parmar, HMS IIIGillian Lieberman, MD
29