Disclosures. Treatment of Pulmonary Vein Stenosis: Interventional Approach. Case 1: One of the Most Challenging Cases PVS I ve encountered.

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1 Disclosures 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, Baylor College of Medicine 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 Page 2 Outline Strategy for transcatheter treatment of pulmonary vein stenosis (PVS) Philosophical approach Specific techniques: Drug-eluting stents Recanalization of atretic veins 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 Page 3 Page 4

2 CT Angiogram at Presentation Take Home Point #1 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 Page 5 Page 6 Take Home Point #2 In cases of severe instability do FIRST what is likely to help MOST Emergent Septostomy At cath: unstable on induction, started on epi and vasopressin with BP 40/20 BP improved to 69/28 after septostomy Page 7 Page 8

3 Take Home Point #3 Presence of an atrial septal defect (ASD) is very helpful In unstable patients: ASD enlargement improves cardiac output (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 Immediate Stenting of the Vein with Best Distal Vasculature 4mm x 8mm Promus Premier stent (Everolimus eluting) Page 9 Page 10 Hemodynamics: RVp ~150% Systemic RUL PV Atresia RML PV Atresia Severe RLL PV Stenosis LLL PV Atresia Page 11 Page 12

4 Take Home Point #4 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 Page 13 Page 14 Wire Recanalization of Atretic RMPV RMPV Angioplasty with 2mm Balloon RLPV Post Angioplasty 2mm Cutting Balloon Note resistant waist on the balloon, indicating a noncompliant lesion Page 15 Page 16

5 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 RML Angioplasty 3mm Balloon RML Promus 4mm x 8mm DES Page 17 Page 18 Wire Recanalization of Atretic RUPV Balloon Angioplasty of RUPV Page 19 Page 20

6 4mm x 8mm Promus DES in RULPV RLPV After Standard & Cutting Balloon Angioplasty RLL Promus drug eluting stent Page 21 Page 22 Wire Recanalization of Atretic LLPV LLPV Balloon Angioplasty Note resistant waist on the balloon, indicating a noncompliant lesion Page 23 Page 24

7 LLPV Standard Balloon Angioplasty Cutting Balloon Rupture with Contrast Extravasation Take Home Point #5 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 Page 25 Page 26 LLPV Promus DES Final, After 5 Drug-Eluting Stents RV: 63/0/11 Fem art: 75/37/52 Take Home Point #6 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 subsegmental veins) These are LONG CASES (6-8 hours) Page 27 Page 28

8 Take Home Point #7 We use drug-eluting stents (DES) in infants to reduce intimal proliferation within the stents 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 Page 29 Page 30 Take Home Point #7 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 Cath #2-1 Month Later 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 Page 32 Page 33

9 RUL PV RML PV Page 34 Page 35 RLL PV LLL PV Page 36 Page 37

10 LUL PV Take Home Point #8 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 Page 38 Page 39 All veins except LUPV were restented distally Page 40 Page 42

11 Page 43 Page 44 Cath #3: 1 Month Later Cath 3: 1 Month Later All stents widely patent, BUT New severe stenosis just beyond all 5 stents Page 46 Page 47

12 Page 48 Page 49 Page 50 Page 51

13 Change of Plans Cath 4: 1 Months Later No additional stents were added She was started on systemic sirolimus (our first patient to be treated this way) Page 52 Page 53 Page 54 Page 55

14 Page 56 Page 57 Over the Next 2 Years How Can we Overcome the Limitation of Re- Expansion of Small Stents in Growing Children? She underwent repeat caths every 3-6 months to redilate stents What happens when we reach the maximal possible diameter of the coronary -type drugeluting stents? Page 58 Page 59

15 At 3 Years Old and 14 Caths Later Page 60 Page 61 Page 64 Page 65

16 Page 66 Page 67 Page 68 Page 69

17 Best of All PA = 25/10/16 (pre LLL intervention) Femoral Artery = 76/41/53 Asymptomatic Normal growth and development On aspirin and sirolimus Page 70 Page 71 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) 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 Page 72 Page 73

18 RU, RM & most of RLL PV are Atretic After Stenting of RL & LLL PV Page 74 Page 75 Atretic LUL PV Collateralized to LLL PV Atretic LUPV Page 76 Page 77

19 LLL PV Atresia Could not be Crossed with Wires: Resort to Radiofrequency LLL PV Atresia Could not be Crossed with Wires: Resort to Radiofrequency Catheterization and Cardiovascular Interventions 56:72 82 (2002) Page 78 Page 79 Confirmation of Intraluminal Location 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 After Drugeluting Stent We proposed another attempt to recanalize RUL PV Page 80 Page 81

20 Cath 2: RUL PA Wedge Angio RPA Angio Page 82 Page 83 PA Angio Overlay & Aim for RUL PV PA Wedge Angio Overlay for RF Page 84 Page 85

21 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! Page 86 Page 87 After a Few Tries Where Are We? Page 88 Page 89

22 Conclusion? Conclusion? It s almost time to stop! Page 90 Page 91 After a Few More Failed Attempts Next Steps Parents agreed to a 3 rd try, 1 month later Page 92 Page 93

23 RF Perforation Microcatheter advancement, and Where are we? Page 94 Page 95 After More Trying Eureka! Balloon angioplasty and stenting Page 96 Page 97

24 Final Result LUL PV Reassessment Occluded Page 98 Page 99 Unable to Recanalize with Wires Use RF! After Placement of 3 Stents Page 100 Page 101

25 2 Years Later PA= 42/14/27 Before RUL PV After Femoral artery= 82/46/58 All 4 PV stents were re-dilated Page 102 Page 103 LUL PV After Dilation to 10 mm with Intentional Stent Fracture Page 104 Page 105

26 LLL PV RLL PV Before After Before After Page 106 Page 107 Clinical Course 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 Take Home Points 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 Page 108 Page 109

27 Acknowledgements 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 Page 110 Page 111 How do DES Work? How do DES Work in PVS? 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 of rapamycin) Inhibit conversion to the synthesis cell phase Paclitaxel Inhibit microtubule depolymerization, excessively stable microtubules inhibit spindle formation and mitosis Unknown Inhibit myofibroblast proliferation? Inhibit myofibroblast differentiation into smooth muscle cells? Page 113 Page 114

28 How We Use DES in PVS 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) Page 115