Tangle Web of HHT (AVMs) - CureHHTTangled Web of HHT: Lung AVMs David Riggans, MD 2 David H....
Transcript of Tangle Web of HHT (AVMs) - CureHHTTangled Web of HHT: Lung AVMs David Riggans, MD 2 David H....
Tangled Web of HHT: Lung AVMs
David Riggans, MD 1
David H. Riggans, MD
Tangle Web of HHT (AVMs)
Lung and Brain
David H. Riggans, MD David H. Riggans, MD
HHT
• Hereditary Hemorrhagic Telangiectasia
• (Osler-Weber-Rendu Syndrome)– multi-system vascular d/o
– 1:5,000 effected
– telangiectasia and AVMs
– nose, lungs, GI tract, brain, liver
– AD inheritance
– txs available for all effected organ systems
2David H. Riggans, MD
David H. Riggans, MD
HHT
• 90-95% with HHT have nosebleeds
• 90-95% have few telangiectasias on face
• 20-25% develop significant GI bleeding
• 40% have pulm AVMs
• 5-20% have at least 1 brain AVM
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Blood Flow Through Lungs
David H. Riggans, MD
David H. Riggans, MD
Blood Flow Through Lungs
David H. Riggans, MD David H. Riggans, MD
Blood Flow Through Lungs
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Tangled Web of HHT: Lung AVMs
David Riggans, MD 2
David H. Riggans, MD
Blood Flow Through Lungs
David H. Riggans, MD David H. Riggans, MD
Blood Flow Through Lungs
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Blood Flow Through Lungs
• This is a more typical appearance for an AVM but for the purposes of this discussion, lets stick with the other.
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Blood Flow Through Lungs
• Clot breaks loose from the veins in your leg
David H. Riggans, MD
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Blood Flow Through Lungs
• Clot breaks loose from the veins in your leg
• And travels into the normal lung vasculature
David H. Riggans, MD David H. Riggans, MD
Blood Flow Through Lungs
• Clot breaks loose from the veins in your leg
• And travels into the normal lung vasculature
• And gets trapped as the vessel get smaller
• Lungs = filterDavid H. Riggans, MD
Tangled Web of HHT: Lung AVMs
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Blood Flow Through Lungs
• Now, take the same clot
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Blood Flow Through Lungs
• Now, take the same clot
• And instead it travels into the abnormal vasculature where the AVM is located
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Blood Flow Through Lungs
• Now, take the same clot
• And instead it travels into the abnormal vasculature where the AVM is located
• It travels through the AVMDavid H. Riggans, MD David H. Riggans, MD
Blood Flow Through Lungs
• Now, take the same clot
• And instead it travels into the abnormal vasculature where the AVM is located
• It travels through the AVM and out the other sideDavid H. Riggans, MD
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Blood Flow Through Lungs
• Lungs ≠ filter
• Stroke, TIA, brain abscess, MI, seizure
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Blood Flow Through Lungs
• Now forget about the clot and just consider the flow through the AVM rather than the capillary bed
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Tangled Web of HHT: Lung AVMs
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Blood Flow Through Lungs
• Oxygen isn’t getting picked up, CO2 isn’t getting deposited
• Heart is wasting a lot of energy pumping blood through the AVM!
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Blood Flow Through Lungs
• Supplemental oxygen
• Exercise intolerance
• Heart failure
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Blood Flow Through Lungs
• AVMs can expand
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Blood Flow Through Lungs
• AVMs can expand, particularly during pregnancy
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Blood Flow Through Lungs
• AVMs can expand, particularly during pregnancy
• And there is a risk of rupture, particularly in the 3rd trimester
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Clinical Manifestations
– Brain– Stroke/TIA (Transient Ischemic Attack): 46%
– Brain Abscess: 11%
– Seizures: 5-15%
– Migraine headaches: 34%
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Tangled Web of HHT: Lung AVMs
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Clinical Manifestations
– Heart– Heart Failure
– MI
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Clinical Manifestations
– Lungs
– Dyspnea (shortness of breath)–Exercise Intolerance (60%)
–Supplemental Oxygen
–Hemoptysis (2%)
–Sac Rupture (3rd trimester of pregnancy)
–Bleeding into chest/death of mother
–Compromise/death of fetus
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David H. Riggans, MD
History
• Prior to 1997– Thoracic surgeons advocated thoracotomy and
wedge resection of the lung or lobectomy for large PAVMs
• 1997– Successful outcome and long term results
published for percutaneous treatment of PAVMs• Rendered surgical therapy obsolete
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Pre-Embolization Evaluation
Most referrals: routine screening eval
– Bubble echo (grades 1-3)– take still frame image with the most bubbles
– grade 1: <30 bubbles in LV
–low risk (<2%) for treatable PAVM, no CT
– grade 2-3: higher risk, CT obtained
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Pre-Embolization Evaluation
Some referrals: come with known PAVM
– Bubble echo not necessary
– Referral to IR for PAVM embo
Some referrals have suspicious CT
– May get echo to confirm shunt
– If positive, refer to IR for embo
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Pre-Embolization Evaluation
CT chest PAVMs are present– Size of the AVM
– Diameter of the feeding artery
– Pulmonary arterial anatomy
– Plan my approach
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Pre-Embolization Evaluation
CT chest with large right upper lobe AVMs
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Catheters
Used for selecting the right and left pulmonary arteries
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Evaluate AVMs• Size
• Location
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Pulmonary Arteriogram
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Pulmonary Arteriogram
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Pulmonary Arteriogram
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Tangled Web of HHT: Lung AVMs
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Pulmonary Arteriogram
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Pulmonary Arteriogram
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Pulmonary Arteriogram
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Pulmonary Arteriogram
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Evaluate Feeding Artery
• Catheter tip at the origin of feeding artery
• Additional X-rays from several viewing angles
• Size
• Configuration
• Determine a safe place to position the embolization coil
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Standard Push CoilsCannot reposition once deployed
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Standard Push Coils
• Method of coil deployment
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Catheter
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Standard Push Coils
• Method of coil deployment
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CoilCatheter
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Standard Push Coils
• Method of coil deployment
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Pushing wire CoilCatheter
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Standard Push Coils
• Method of coil deployment
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Pushing wire CoilCatheter
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Standard Push Coils
• Method of coil deployment
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Pushing wire CoilCatheter
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Standard Push Coils
• Method of coil deployment
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Pushing wire CoilCatheter
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Flipper Coils
• Detachable
• Unscrew to release from guidewire
• Can reposition before deploying
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Trigger Release Coils
• Detachable
• Trigger release mechanism
• Can reposition before deploying
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Amplatz Spider
• Used in large feeding arteries
• Forms stable scaffold for subsequent coil placement
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Amplatzer Vascular Plug
• Used in large feeding arteries
• Can reposition before deploying
• Used alone or with coils
• 2 occlusive surfaces
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Amplatzer Vascular Plug
• Introduction of Amplatzer 2 and 4
• Amplatzer 2 has 6 occlusive surfaces (more effective)
• Amplatzer 4 can be introduced through a catheter
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Amplatzer Vascular Plug
• Introduction of Amplatzer 2 and 4
• Amplatzer 2 has 6 occlusive surfaces (more effective)
• Amplatzer 4 can be introduced through a catheter
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Amplatzer Vascular Plug
• Introduction of Amplatzer 2 and 4
• Amplatzer 2 has 6 occlusive surfaces (more effective)
• Amplatzer 4 can be introduced through a catheter
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Amplatzer Vascular Plug
• Introduction of Amplatzer 2 and 4
• Amplatzer 2 has 6 occlusive surfaces (more effective)
• Amplatzer 4 can be introduced through a catheter
• Amplatzer diameter should be 30-50% greater than arterial diameter
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Micro Vascular Plug
• Introduced through a microcatheter
• Wrapped with occlusive PTFE
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Occlusive Micro Balloon Catheter
• Microcatheter can get into tiny branches
• Balloon is used to occlude flow so micro coils can be introduced with less risk
• Microvascular plugs (similar to Amplatzer plug) can be deployed through this catheter
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Going…Going…GONE!
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Going…GONE!
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Searching…
Normal artery this time…no AVM here
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Embolization
• 2 views of same AVM
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• Delayed view draining vein
• After embolization
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• When do we treat?
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Embolization
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• When do we treat?
• Historically– 3mm or larger
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Embolization
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• When do we treat?
• Historically– 3mm or larger
• Currently– 2-3mm or larger
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Embolization
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• 3mm or smaller
• Less blood flow through AVM– less stress on heart
– less risk of rupture
– less risk of dyspnea
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Small AVMs
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• Small AVMs can still cause problems– Stroke
– TIA
– Brain abscess
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Small AVMs
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• Technical goals during the embolization treatment:– get as close to the sac a possible (as far down
the artery as you can go)
– choose the right size embolization devices
– pack the coils as tightly as you can (usually requires multiple coils)
• Indication that the artery is occluded:– no more flow beyond the occlusion devices
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Embolization
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• After successfully occluding the first artery:– make sure there are no additional feeding
arteries to the sac (complex AVM)
– if so, embolize those as well
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Embolization
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• Classification:– Simple (90%) vs Complex (10%)
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• CT image of AVM
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Embolization
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• In all the previous cases, we embolized the artery supplying the AVM or the “feeding artery”
• Other methods:– embolize the sac
– embolize both the sac and the feeding artery
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Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
– draining vein bigger than feeding artery
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
– draining vein bigger than feeding artery
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
– draining vein bigger than feeding artery
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
– draining vein bigger than feeding artery
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Sac Embolization
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• Considered in the following circumstances:– short feeding artery without enough room for
embolization products
– draining vein bigger than feeding artery
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Sac Embolization
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• Sac embolization from article in 2012:
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Sac Embolization
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• Sac embolization from article in 2012:
• If you also embolized the feeding artery, it would be occluded here:
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Embolization
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• Embolization of non-target tissue– same idea as clot in the leg veins passing
through an untreated PAVM causing stroke, TIA, heart attack, blocking of leg arteries
– air: bubbles can get introduced through the catheter and pass through the AVM
– device: if improperly sized, can pass through the AVM
– clot: can form on catheter and pass through the AVM
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Treatment Complications
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• Embolization of non-target tissue– Not really a complication: embolize normal
adjacent pulmonary artery branches
– Happens in every case to some degree
– Usually tiny branches
– Rarely large branches but sometimes inevitable
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Treatment Complications
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• Pleurisy– inflammation related to embolization
– can make breathing painful
– usually lasts ~3-7 days
– treated by anti-inflammatory meds or steroids
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Treatment Complications
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• Recanalization (1-57%)– 10% is about average
– flow re-established through occlusion device(s)
– less common in recent years:• Amplatzer Plugs
• microcatheters, microcoils, microvascular plugs
• Kidney insufficiency or failure– too much contrast during procedure (rare)
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Treatment Complications
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• Bleeding– PAVM sac rupture (rare)
– bleeding from access site in femoral vein (rare)
• Infection– don’t treat patients with active infection until the
infection is successfully treated (rare)
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Treatment Complications
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• CT chest with IV contrast at 3-6 months– Intended findings:
• diminished sac size by >70%
• no contrast in sac or draining vein
• these are critical determinants of successful treatment
• Physiological evaluation
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Follow-Up
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• Diminished sac size:
– Immediately post-embo
– 5mo post-embo
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Follow-Up
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• Original CT chest
• 5 years after treatment with involuted sac
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Follow-Up
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• LEARNING CURVE EFFECT: REPERFUSION
1978 - 88 13.0%
1988 - 95 7.0%
1996 - 00 3.7%
SALUJA ET AL, JVIR 10: 883-89, 1999
Probably a good idea to get AVMs treated at an HHT center where the operators have more experience
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Recannalization
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• Previously treated patient
• AVM recannalized
• Coils present from prior attempt
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Recannalization
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• Additional coils added, more tightly packed and appropriately sized coils
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Recannalization
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• ~4mm feeding artery plus multiple additional smaller feeders
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New Embolization
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• Sagittal view of same AVM with smaller branches
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New Embolization
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• Angiogram showing the same findings
• Amplatzer 4 Plug used to occlude the artery
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New Embolization
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• Multiple feeding arteries
• Multiple sacs of varying sizes
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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• Very complex series of arteries, sacs and draining veins
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Complex AVM
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Complex AVM
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• Essentially pruned that entire section of lung
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Complex AVM
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• Excellent example of embolizing too far away from the sac
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Embolization
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• If you can advance the catheter to here
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Embolization
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• If you can advance the catheter to here, then embolizing this segment would have been enough.
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Embolization
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• These arterial branches would all have been preserved.
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Embolization
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• These arterial branches would all have been preserved.
• Instead, they’re all occluded.121David H. Riggans, MD
Embolization
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• Amplatzer 4 Plug placed followed by coils
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Untreated AVM
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• Amplatzer 4 Plug placed followed by coils
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Untreated AVM
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• Amplatzer 4 Plug placed followed by coils
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Untreated AVM
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Embolization
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Previously Treated AVM
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• Amplatzer 2 Plug with coils placed behind it
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Previously Treated AVM
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• Amplatzer 2 Plug with coils placed behind it
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Previously Treated AVM
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• Amplatzer 2 Plug with coils placed behind it
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Previously Treated AVM
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• Most serious symptoms of PAVM are neurologic–Brain abscess–Stroke/TIA–Migraine
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Neurologic Manifestations HHT Patients with PAVM(s)
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Single (26) Multiple (49)Migraine 50% 63%Abscess 8% 16%Stroke 32% 60%
The more AVMs, the higher the risk!
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Neurologic Manifestations HHT Patients with PAVM(s)