Tangle Web of HHT (AVMs) - CureHHTTangled Web of HHT: Lung AVMs David Riggans, MD 2 David H....

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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 2 David 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 3 David H. Riggans, MD David H. Riggans, MD 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 David H. Riggans, MD

Transcript of Tangle Web of HHT (AVMs) - CureHHTTangled Web of HHT: Lung AVMs David Riggans, MD 2 David H....

Page 1: Tangle Web of HHT (AVMs) - CureHHTTangled Web of HHT: Lung AVMs David Riggans, MD 2 David H. Riggans, MD Blood Flow Through Lungs David H. Riggans, MD Blood Flow Through Lungs David

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

3David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD

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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.

David H. Riggans, MD David H. Riggans, MD

Blood Flow Through Lungs

• Clot breaks loose from the veins in your leg

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

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

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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

David 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 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

David H. Riggans, MD

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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David H. Riggans, MD

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

David H. Riggans, MD David H. Riggans, MD

Clinical Manifestations

– Brain– Stroke/TIA (Transient Ischemic Attack): 46%

– Brain Abscess: 11%

– Seizures: 5-15%

– Migraine headaches: 34%

David H. Riggans, MD

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Tangled Web of HHT: Lung AVMs

David Riggans, MD 5

David H. Riggans, MD

Clinical Manifestations

– Heart– Heart Failure

– MI

David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD

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

David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD

David H. Riggans, MD

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

David H. Riggans, MD David H. Riggans, MD

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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Tangled Web of HHT: Lung AVMs

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Pre-Embolization Evaluation

CT chest with large right upper lobe AVMs

David H. Riggans, MD David H. Riggans, MD

Catheters

Used for selecting the right and left pulmonary arteries

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Evaluate AVMs• Size

• Location

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Pulmonary Arteriogram

David H. Riggans, MD

David H. Riggans, MD

Pulmonary Arteriogram

David H. Riggans, MD

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Pulmonary Arteriogram

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Tangled Web of HHT: Lung AVMs

David Riggans, MD 7

David H. Riggans, MD

Pulmonary Arteriogram

David H. Riggans, MD David H. Riggans, MD

Pulmonary Arteriogram

David H. Riggans, MD

David H. Riggans, MD

Pulmonary Arteriogram

David H. Riggans, MD David H. Riggans, MD

Pulmonary Arteriogram

David H. Riggans, MD

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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

David H. Riggans, MD David H. Riggans, MD

Standard Push CoilsCannot reposition once deployed

David H. Riggans, MD

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Tangled Web of HHT: Lung AVMs

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Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

Catheter

David H. Riggans, MD

Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

CoilCatheter

David H. Riggans, MD

Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

Pushing wire CoilCatheter

David H. Riggans, MD

Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

Pushing wire CoilCatheter

David H. Riggans, MD

Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

Pushing wire CoilCatheter

David H. Riggans, MD

Standard Push Coils

• Method of coil deployment

David H. Riggans, MD

Pushing wire CoilCatheter

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Tangled Web of HHT: Lung AVMs

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Flipper Coils

• Detachable

• Unscrew to release from guidewire

• Can reposition before deploying

David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD David H. Riggans, MD

Amplatzer Vascular Plug

• Used in large feeding arteries

• Can reposition before deploying

• Used alone or with coils

• 2 occlusive surfaces

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David H. Riggans, MD

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

David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD

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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

David H. Riggans, MD David H. Riggans, MD

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

David H. Riggans, MD

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Micro Vascular Plug

• Introduced through a microcatheter

• Wrapped with occlusive PTFE

David H. Riggans, MD David H. Riggans, MD

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!

David H. Riggans, MD David H. Riggans, MD

Going…GONE!

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Tangled Web of HHT: Lung AVMs

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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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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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Tangled Web of HHT: Lung AVMs

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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

David H. Riggans, MD

• 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

David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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Tangled Web of HHT: Lung AVMs

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• Considered in the following circumstances:– short feeding artery without enough room for

embolization products

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Sac Embolization

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David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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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David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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

David H. Riggans, MD

• 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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Tangled Web of HHT: Lung AVMs

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David H. Riggans, MD115David H. Riggans, MD

Complex AVM

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• Essentially pruned that entire section of lung

116David H. Riggans, MD

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

David H. Riggans, MD

• If you can advance the catheter to here, then embolizing this segment would have been enough.

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Embolization

David H. Riggans, MD

• These arterial branches would all have been preserved.

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Embolization

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Tangled Web of HHT: Lung AVMs

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David H. Riggans, MD

• 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

David H. Riggans, MD

• Amplatzer 4 Plug placed followed by coils

124David H. Riggans, MD

Untreated AVM

David H. Riggans, MD125David H. Riggans, MD

Embolization

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Previously Treated AVM

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• Amplatzer 2 Plug with coils placed behind it

127David H. Riggans, MD

Previously Treated AVM

David H. Riggans, MD

• Amplatzer 2 Plug with coils placed behind it

128David H. Riggans, MD

Previously Treated AVM

David H. Riggans, MD

• 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)

David H. Riggans, MD

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)