4/21/2018 Disclosures •Consulting/speaker’s bureau for: ‐ St. Jude Medical ‐ B-Braun Interventional Systems ‐ Medtronic •Scientific advisory board: Treatment of ‐ Janssen Pharmaceutical ‐ Pediastent Pulmonary Vein Stenosis: •Co-founder: Interventional Approach ‐ PolyVascular •I will discuss off-label and investigational uses of Henri Justino MD, CM, FRCPC, FSCAI, FACC, FAAP Director, CE Mullins Cardiac Catheterization Laboratories, medical devices Texas Children’s Hospital Associate Professor of Pediatrics, Baylor College of Medicine Page 2 Pediatrics Case 1: One of the Most Challenging Outline Cases PVS I’ve encountered… •Strategy for transcatheter treatment of pulmonary •Term baby girl vein stenosis (PVS) •Family history of brother who passed away with ‐ Philosophical approach PVS at 11 months of age ‐ Specific techniques: •Echo at 8 days showed PVS in all veins •Drug-eluting stents •Transferred to our ICU at 3 weeks (3 kg) with •Recanalization of atretic veins 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 Pediatrics Pediatrics 1
4/21/2018 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 Pediatrics Pediatrics Take Home Point #2 Emergent Septostomy •At cath: unstable on induction, started on epi and vasopressin with BP 40/20 •In cases of severe instability… do FIRST what is •BP improved to 69/28 after septostomy likely to help MOST Page 7 Page 8 Pediatrics 2
4/21/2018 Immediate Stenting of the Vein with Best Distal Take Home Point #3 Vasculature 4mm x 8mm Promus Premier stent (Everolimus eluting) •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 Page 9 Page 10 Pediatrics Pediatrics Severe RLL Severe RLL LLL PV LLL PV Hemodynamics: RVp ~150% Systemic PV Stenosis PV Stenosis Atresia Atresia RUL PV Atresia RML PV Atresia Page 11 Page 12 Pediatrics Pediatrics 3
4/21/2018 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 Pediatrics Wire Wire RMPV RMPV RLPV Post RLPV Post 2mm Cutting 2mm Cutting Recanalization of Recanalization of Angioplasty with Angioplasty with Angioplasty Angioplasty Balloon Balloon Atretic RMPV Atretic RMPV 2mm Balloon 2mm Balloon Note resistant “waist” on the balloon, indicating a non- compliant lesion Page 15 Page 16 Pediatrics Pediatrics 4
4/21/2018 RML RML RML Promus RML Promus The Role for Cutting Balloons Angioplasty Angioplasty 4mm x 8mm 4mm x 8mm 3mm Balloon 3mm Balloon DES DES •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 Page 17 Page 18 Pediatrics Pediatrics Balloon Angioplasty of RUPV Wire Recanalization of Atretic RUPV Page 19 Page 20 Pediatrics Pediatrics 5
4/21/2018 RLPV After RLPV After RLL Promus RLL Promus Standard & Standard & 4mm x 8mm Promus DES in RULPV drug eluting drug eluting Cutting Balloon Cutting Balloon stent stent Angioplasty Angioplasty Page 21 Page 22 Pediatrics Pediatrics Wire Recanalization of Atretic LLPV LLPV Balloon Angioplasty •Note resistant “waist” on the balloon, indicating a non- compliant lesion Page 23 Page 24 6
4/21/2018 Cutting Balloon Cutting Balloon LLPV Standard LLPV Standard Take Home Point #5 Rupture with Rupture with Balloon Balloon Contrast Contrast Angioplasty Angioplasty Extravasation Extravasation •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 Pediatrics Final, After 5 Final, After 5 LLPV Promus LLPV Promus Take Home Point #6 RV: 63/0/11 RV: 63/0/11 Drug-Eluting Drug-Eluting DES DES Fem art: 75/37/52 Fem art: 75/37/52 Stents Stents •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) Page 27 Page 28 Pediatrics 7
4/21/2018 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 Pediatrics Pediatrics Take Home Point #7 Cath #2 - 1 Month Later… •We repeat cath 3-4 mos after the initial intervention •We DO NOT WAIT for •Because of severity of disease and rapidity of progression, I chose to repeat cath at 4 weeks… ‐ Evidence of worsening PVS on echo (unreliable for ostial disease, useless for distal disease) ‐ Evidence of worsening PH •All stents widely patent, BUT… ‐ Symptoms to develop •New severe stenosis just beyond each stent •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 Page 32 Page 33 Pediatrics Pediatrics 8
4/21/2018 RUL PV RML PV Page 34 Page 35 Pediatrics Pediatrics RLL PV LLL PV Page 36 Page 37 Pediatrics Pediatrics 9
4/21/2018 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 Pediatrics Pediatrics All veins except LUPV were re- stented distally Page 40 Page 42 Pediatrics Pediatrics 10
4/21/2018 Page 43 Page 44 Pediatrics 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 Pediatrics Pediatrics 11
4/21/2018 Page 48 Page 49 Pediatrics Pediatrics Page 50 Page 51 Pediatrics Pediatrics 12
4/21/2018 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 Pediatrics Pediatrics Page 54 Page 55 Pediatrics Pediatrics 13
4/21/2018 Page 56 Page 57 Pediatrics Pediatrics 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 drug- eluting stents? Page 58 Page 59 Pediatrics Pediatrics 14
4/21/2018 At 3 Years Old and 14 Caths Later… Page 60 Page 61 Pediatrics Pediatrics Page 64 Page 65 Pediatrics Pediatrics 15
4/21/2018 Page 66 Page 67 Pediatrics Pediatrics Page 68 Page 69 Pediatrics Pediatrics 16
4/21/2018 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 Pediatrics Pediatrics PVS After TAPVR Repair PVS After TAPVR Repair 1. Obstructed infradiaphragmatic total anomalous pulmonary venous return, s/p repair (day 1) •When transferred to us, CT showed RL & LLPV 2. LUL & LLL splayed open & anastomosed to left atrium, stenosis and RU & LUPV atresia RUL PV atresia, s/p repair and sutureless repair of right common PV (RUL + RLL PV) (2 months) 3. Cardiac arrest & CPR •At cath: PA= 65/23/40, femoral art= 66/38/49 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) Page 72 Page 73 Pediatrics Pediatrics 17
4/21/2018 After Stenting of RL & LLL PV RU, RM & most of RLL PV are Atretic Page 74 Page 75 Pediatrics Atretic LUL PV Collateralized to LLL PV Atretic LUPV Page 76 Page 77 18
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