Disclosures Consulting/speakers bureau for: St. Jude Medical - - PowerPoint PPT Presentation

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Treatment of Pulmonary Vein Stenosis: Interventional Approach

Henri Justino MD, CM, FRCPC, FSCAI, FACC, FAAP Director, CE Mullins Cardiac Catheterization Laboratories, Texas Children’s Hospital Associate Professor of Pediatrics, Baylor College of Medicine

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Disclosures

• Consulting/speaker’s bureau for:

‐St. Jude Medical ‐B-Braun Interventional Systems ‐Medtronic

• Scientific advisory board:

‐Janssen Pharmaceutical ‐Pediastent

• Co-founder:

‐PolyVascular

• I will discuss off-label and investigational uses of

medical devices

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• Strategy for transcatheter treatment of pulmonary

vein stenosis (PVS)

‐Philosophical approach ‐Specific techniques:

• Drug-eluting stents
• Recanalization of atretic veins

Outline

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Case 1: One of the Most Challenging Cases PVS I’ve encountered…

• Term baby girl
• Family history of brother who passed away with

PVS at 11 months of age

• Echo at 8 days showed PVS in all veins
• Transferred to our ICU at 3 weeks (3 kg) with

tachypnea and right pleural effusion

• CT showed progression to atresia in 3 of 5 veins
• We began process of listing for lung transplant

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CT Angiogram at Presentation

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• PVS is a rapidly progressive disease that can be

fatal (especially if bilateral)

• Delay in care leads to

‐Worsening disease at the venous ostia ‐Worsening distal hypoplasia ‐Worsening pulmonary hypertension

• i.e. PVS medical emergency!
• Referrals are processed rapidly and a cath date is

provided within a few weeks at most

Take Home Point #1

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• In cases of severe instability… do FIRST what is

likely to help MOST

Take Home Point #2

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• At cath: unstable on induction, started on epi and

vasopressin with BP 40/20

• BP improved to 69/28 after septostomy

Emergent Septostomy

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• Presence of an atrial septal defect (ASD) is very

helpful…

‐In unstable patients: ASD enlargement improves cardiac

• utput (at the expense of desaturation)

‐In all patients: avoids repeated transseptal punctures at future caths to reach the left atrium

• We create or enlarge an ASD in all patients with

PVS

Take Home Point #3

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Immediate Stenting of the Vein with Best Distal Vasculature

4mm x 8mm Promus Premier stent (Everolimus eluting)

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Hemodynamics: RVp ~150% Systemic RML PV Atresia RUL PV Atresia

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Severe RLL PV Stenosis Severe RLL PV Stenosis LLL PV Atresia LLL PV Atresia

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• Every case receives complete evaluation of every

LOBE using wedge pressures and wedge angios

• In some cases, additional pressures and angios

are obtained in multiple SEGMENTS of each lobe

Take Home Point #4

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Wire Recanalization of Atretic RMPV Wire Recanalization of Atretic RMPV RMPV Angioplasty with 2mm Balloon RMPV Angioplasty with 2mm Balloon

Note resistant “waist”

• n the balloon,

indicating a non- compliant lesion

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RLPV Post Angioplasty RLPV Post Angioplasty 2mm Cutting Balloon 2mm Cutting Balloon

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The Role for Cutting Balloons

• 3 or 4 microsurgical blades mounted

longitudinally on outer surface

• Approved to treat lesions resistant to

conventional balloon angioplasty in

‐Coronary arteries (2-4 mm balloons) ‐Peripheral arteries (5-8 mm balloons)

• Used off-label to treat resistant

lesions in a variety of conditions… including PVS

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RML Angioplasty 3mm Balloon RML Angioplasty 3mm Balloon RML Promus 4mm x 8mm DES RML Promus 4mm x 8mm DES

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Wire Recanalization of Atretic RUPV

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Balloon Angioplasty of RUPV

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4mm x 8mm Promus DES in RULPV

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RLPV After Standard & Cutting Balloon Angioplasty RLPV After Standard & Cutting Balloon Angioplasty

RLL Promus drug eluting stent RLL Promus drug eluting stent

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Wire Recanalization of Atretic LLPV

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LLPV Balloon Angioplasty

• Note resistant “waist”
• n the balloon,

indicating a non- compliant lesion

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LLPV Standard Balloon Angioplasty LLPV Standard Balloon Angioplasty Cutting Balloon Rupture with Contrast Extravasation Cutting Balloon Rupture with Contrast Extravasation

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• Atretic pulmonary veins can often be recanalized
• We use CUTTING BALLOONS to overcome

resistant lesions (lesions that cannot be dilated despite high pressures of ~20 ATM)

• Lesion preparation prior to stent placement is

paramount…

‐Once stented, resistant lesions can no longer be treated with cutting balloons

Take Home Point #5

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LLPV Promus DES LLPV Promus DES Final, After 5 Drug-Eluting Stents Final, After 5 Drug-Eluting Stents

RV: 63/0/11 Fem art: 75/37/52 RV: 63/0/11 Fem art: 75/37/52

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• We aim to open EVERY LOBAR PULMONARY

VEIN at the initial catheterization

• When necessary, we treat first or second order

divisions deep into the lung (segmental or sub- segmental veins)

• These are LONG CASES (6-8 hours)

Take Home Point #6

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• We use drug-eluting stents (DES) in infants to

reduce intimal proliferation within the stents

Take Home Point #7

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• 10 year follow-up: no hemoptysis after DES, LLPV is

widely patent at stents and has grown distally…

• No recurrence of hemoptysis, but needed multiple

interventions

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• We repeat cath 3-4 mos after the initial intervention
• We DO NOT WAIT for

‐Evidence of worsening PVS on echo (unreliable for ostial disease, useless for distal disease) ‐Evidence of worsening PH ‐Symptoms to develop

• We don’t generally use CT angio to monitor

‐Radiation + contrast, without opportunity to intervene

• We don’t generally use MRI to monitor

‐General anesthesia, stent artifacts, without opportunity to intervene

Take Home Point #7

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• Because of severity of disease and rapidity of

progression, I chose to repeat cath at 4 weeks…

• All stents widely patent, BUT…
• New severe stenosis just beyond each stent

Cath #2 - 1 Month Later…

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RUL PV

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RML PV

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RLL PV

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LLL PV

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LUL PV

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• DES are very thin, delicate, and hard to see!
• Each stent must be very carefully re-entered at

subsequent caths for re-dilation

‐Crossing a side cell of the stent with a wire is easy to do, and must be detected and corrected immediately before stent is deformed

• We use 2 or more coaxial catheters to allow us to

point in a variety of angles and directions

Take Home Point #8

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All veins except LUPV were re- stented distally

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• All stents widely patent, BUT…
• New severe stenosis just beyond all 5 stents

Cath #3: 1 Month Later…

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Cath 3: 1 Month Later

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• No additional stents were added
• She was started on systemic sirolimus (our first

patient to be treated this way)

Change of Plans…

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Cath 4: 1 Months Later

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• She underwent repeat caths every 3-6 months to

redilate stents

• What happens when we reach the maximal

possible diameter of the “coronary”-type drug- eluting stents?

Over the Next 2 Years…

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How Can we Overcome the Limitation of Re- Expansion of Small Stents in Growing Children?

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At 3 Years Old and 14 Caths Later…

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• PA = 25/10/16 (pre LLL intervention)
• Femoral Artery = 76/41/53
• Asymptomatic
• Normal growth and development
• On aspirin and sirolimus

Best of All…

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PVS After TAPVR Repair

• 1. Obstructed infradiaphragmatic total anomalous

pulmonary venous return, s/p repair (day 1)

• 2. LUL & LLL splayed open & anastomosed to left atrium,

RUL PV atresia, s/p repair and sutureless repair of right common PV (RUL + RLL PV) (2 months)

• 3. Cardiac arrest & CPR
• 4. Balloon angioplasty of LLL PV and confluence (6 months)
• 5. PV confluence balloon angioplasty (8 months)
• 6. LUL PV anastomosis to left atrial appendage and RUL &

RLL PV anastomosis to back of left atrium, fenestrated ASD closure (10 months)

• 7. 6 day ECMO course
• 8. Right diaphragm paralysis required plication (11 months)

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PVS After TAPVR Repair

• When transferred to us, CT showed RL & LLPV

stenosis and RU & LUPV atresia

• At cath: PA= 65/23/40, femoral art= 66/38/49

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RU, RM & most of RLL PV are Atretic

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After Stenting of RL & LLL PV

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Atretic LUL PV Collateralized to LLL PV

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Atretic LUPV

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LLL PV Atresia Could not be Crossed with Wires: Resort to Radiofrequency

Catheterization and Cardiovascular Interventions 56:72–82 (2002)

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LLL PV Atresia Could not be Crossed with Wires: Resort to Radiofrequency

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After Drug- eluting Stent After Drug- eluting Stent Confirmation of Intraluminal Location Confirmation of Intraluminal Location

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PVS After TAPVR Repair

• At first cath RUL PV could not be recanalized
• 6 months later…
• Patient presented with severe tricuspid

regurgitation, near-systemic RV pressure, and RV dysfunction

• We proposed another attempt to recanalize RUL

PV

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Cath 2: RUL PA Wedge Angio

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RPA Angio

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PA Angio Overlay & Aim for RUL PV

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PA Wedge Angio Overlay for RF

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Next Steps

• 1. Advance microcatheter over RF wire into lung
• 2. Remove RF wire
• 3. Inject contrast into microcatheter to find out if

catheter is intravascular or not

• 4. If staining (i.e. extravascular location), withdraw

microcatheter while injecting contrast to determine if can re-enter vascular space

• 5. If at first you don’t succeed, try and try again!
• 6. But… realize when to stop trying!

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After a Few Tries…

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Where Are We?

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Conclusion?

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Conclusion?

• It’s almost time to stop!

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After a Few More Failed Attempts…

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Next Steps…

• Parents agreed to a 3rd try, 1 month later

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RF Perforation

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Microcatheter advancement, and… Where are we?

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After More Trying… Eureka!

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Balloon angioplasty and stenting…

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Final Result

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LUL PV Reassessment… Occluded

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Unable to Recanalize with Wires… Use RF!

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After Placement of 3 Stents…

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• PA= 42/14/27
• Femoral artery= 82/46/58
• All 4 PV stents were re-dilated

2 Years Later…

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RUL PV After Before

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LUL PV

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After Dilation to 10 mm with Intentional Stent Fracture

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LLL PV After Before

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RLL PV After Before

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• Transferred to ICU in stable condition after cath
• Unplanned self-extubation overnight, required brief

CPR

• Intraperitoneal bleeding from liver puncture site

after CPR (did not require drainage)

• Several more episodes of self-extubations while

weaning sedation, CPR each time

• After a major cardiac arrest, developed severe

ventricular dysfunction, and eventually died

Clinical Course

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• PVS remains a serious condition
• Initial threat is severe PH with RV dysfunction

‐Requires aggressive attempts to restore patency of pulmonary veins

• Other threats exist, such as complex co-morbidities

‐Developmental delay ‐Swallowing dysfunction & aspiration ‐Chronic lung disease ‐Diaphragmatic dysfunction ‐Iatrogenesis

Take Home Points

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• Interdisciplinary collaborators:

‐Interventional cardiology ‐Pulmonary hypertension team ‐Critical care ‐CV surgery

• Generous families who donated funds to start the

PVS registry

• Dr. Ron Day for securing funding, Dr. Rachel

Vanderlaan for spearheading the PVS registry

Acknowledgements

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hjustino@bcm.edu

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How do DES Work?

• 2 categories of drugs (Katz Curr Atheroscler Rep (2015) 17: 11)

‐Rapamycin-like (sirolimus, everolimus, zotarolimus, “-olimus”)

• Bind to cytosolic FK binding protein, inhibit mTOR (target
• f rapamycin)
• Inhibit conversion to the synthesis cell phase

‐Paclitaxel

• Inhibit microtubule depolymerization, excessively stable

microtubules inhibit spindle formation and mitosis

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How do DES Work in PVS?

• Unknown

‐Inhibit myofibroblast proliferation? ‐Inhibit myofibroblast differentiation into smooth muscle cells?

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• DES are currently available only in coronary format

(delivered at 2-5 mm, can reach max diameters of ~8 mm)

‐We use 4 mm almost every time, regardless of vein size

• Stents must span the lesion

‐Ostial lesions are most common, requiring stents to protrude a variable length into the left atrium and into the lung

• We use the shortest stents available (8mm)
• Need to limit proximal left atrial protrusion (hard to

re-enter) and distal protrusion (jailing of healthy branches)

How We Use DES in PVS