Controversies in Interventional Cardiology

Mark A Law, M.D., Associate Professor University of Alabama at Birmingham Alabama Congenital Disease Center Disclosures l I do not have any relevant financial relationships with any commercial interests to disclose l Off label use of devices will be discussed Controversies in Interventional Cardiology l Definition l Historical controversies l Current controversies Selected cases/diseases Discussion Recommendations

Current Controversies l Controversy – Definition A prolonged public dispute, debate, or contention concerning a matter of opinion Controversy

Historical Controversy Historical Controversy Werner Forssman l Hypothesis Catheter inserted into heart from vein w Deliver drugs, dyes or measure pressure l Risk/Concern w Passing catheter into heart would be fatal Historical Controversy Werner Forssman Historical Controversy Werner Forssman l Opposed department chief l Opposed operating room nurse Restrained her to OR table l Performed procedure on himself l Placed urinary catheter into vein l Walked to x-ray department to document l Repeated this multiple times Until ran out of veins Historical Controversy Werner Forssman

Historical Controversy Werner Forssman Historical Controversy Werner Forssman l Outcome Dismissed from position (1932) “You certainly can’t begin surgery in that manner.” Ferdinand Sauerbruch Nobel Prize in Physiology or Medicine w 1956 along with Cornand and Richards Historical Controversy l 1950 Blalock Hanlon Surgical resection of atrial septum w Thoracotomy w Closed atrial septectomy Palliative treatment for d-TGA w Allowed survival w Pathway for atrial switch operation Historical Controversy Balloon Septostomy l 1966 – Rashkind balloon Historical Controversy Balloon Septostomy

“...The initial response to this report varied between admiration and horror but, in either case, the procedure stirred the imagination of the “invasive” cardiologists throughout the entire cardiology world and set the stage for all future intracardiac interventional procedures – the true beginning of pediatric and adult interventional cardiology.” (Charles E. Mullins, 1998) Current Controversies

Indications for the Hybrid Norwood Does eliminating ASD shunts improve BPD Is erosion risk too high in ASD closure Transcatheter valve versus surgical implant Should small ASD be closed Pre Glenn/Fontan catheterization Should small stents be implanted in growing children Pulmonary vein stenosis interventions PDA stent versus surgical shunt Endomyocardial for Early postoperative interventions

Current Controversies

Surgical versus catheter ASD closure PDA closure – How small is too small (patient)? Balloon versus surgical valvotomy Indications for Fontan fenestration closure Indications for Fontan fenestration creation Best coarctation intervention stent versus surgical patch Transcatheter VSD closure (perimembranous, muscular) Endomyocardial biopsy after transplant Occlusion of collaterals (arterial, venous) Bare metal versus covered stents Current Controversies PDA – How small is too small l 1966 Portsmann 17 year old boy 18F arterial sheath l 1976 Rashkind Rashkind PDA occluder 3500 gram infant in 1978 l 1992 Gianturco Coil closure

Current Controversies PDA – How small is too small l 2003 Amplatzer PDA device

w 5-6F sheath delivery system l Past 5 years Amplatzer Duct Occluder II

w 4-5F catheter Nit-Occlude®

w 4-5F catheter

Current Controversies PDA – How small is too small Under what size is the Amplatzer Duct Occluder contraindicated, based on the FDA approved instructions for use (IFU)? a) 8 kg b) 6 kg c) 5 kg d) 4 kg e) No size contraindication Current Controversies PDA – How small is too small Current Controversies PDA – How small is too small Under what age is the Amplatzer Duct Occluder contraindicated, based on the FDA approved instructions for use (IFU)? a) 6 months b) 4 months c) 2 months d) 1 month e) No age contraindication Current Controversies PDA – How small is too small Current Controversies PDA – How small is too small l Contraindicate ADO, ADO 2 Instructions for use (IFU)

w Contraindicated < 6 kg and 6 months Nit-Occlude®

w Contraindicated < 5 kg Current Controversies PDA – How small is too small l What about less than 6 kg Retrospectively reviewed 62 patients Average weight 4.6 kg +/- 0.9 Success 94%

w Coils, ADO I, Grifka device

*Dimas et al. Outcomes of transcatheter Occlusion of Patent Ductus Arteriosus in Infants Weighing <= 6 kg. JACC Card Interventions. 2010. Current Controversies PDA – How small is too small Current Controversies PDA – How small is too small l Controversy – What is risk less than 6 kg C3PO*

w Project on Outcome Registry Adverse events 27% (<6 kg) versus 8% (>6kg) Major adverse event 10% versus 2% Increase in procedural failure

*El-Said et al. Safety of Percutaneous Patent Ductus Arteriosus Closure: An Unselected Multicenter Population Experience. J Am Heart Assoc. 2013. Current Controversies PDA – How small is too small l What about smaller infant/neonates < 2.5 kg Device limitations Arterial access and injury Anatomic substrate Environmental conditions Does PDA closure benefit these patients? Current Controversies PDA – How small is too small l 10 patients undergoing PDA closure 7 patients less than < 4 kg l Amplatzer Vascular Plug II Premature ductus arteriosus, sub type Long tubular 4F delivery system, less stiffness

* Donnelly J et al. Closure of Tubular Patent Ductus Arteriosus with the Amplatzer Vascular Plug II. CCI. 81:1188-93: 2013. Current Controversies PDA – How small is too small l Zahn et al* 6 infants, weight 440-2480 grams Amplatzer Vascular Plug II (3-4) Venous sheath with echo guidance

*A Novel Technique for Transcatheter Patent Ductus Arteriosus Closure in Extremely Preterm Infants Using Commercially Available Technology, Cath and Card Intervention (2015). Current Controversies PDA – How small is too small Current Controversies PDA – How small is too small l 6 Abstracts PICS last week PDA closure < 4 kg Many non US used ADO II AS

w Not available in the US

w Easy to deliver/recapture

w Hybrid of AVP II and ADO II Current Controversies PDA – How small is too small l Summary and Recommendations Minimal controversy > 5-6 kg

w Technically easier and lower risk as bigger 2.5-5 kg

w Anatomic delineation beneficial

§ Device appropriateness

w Increased adverse events < 2.5 kg

w Anatomy and device availability is key

w Consider echo guidance

w Creative bedside closure for very small neonates

Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration

Closure Which of the following Fontan fenestration characteristics is ideal in a patient who is otherwise has a “perfect” Fontan (left , no mitral regurgitation, normal PA’s, Fontan pressure of 14 mmHg)? a) Fenestration closed, room air saturation 97% b) Fenestration patent, room air saturation 92% c) Fenestration patent, room air saturation 88% d) Fenestration patent, room air saturation 85% Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration

Closure Which of the following Fontan fenestration characteristics is ideal in a patient who is otherwise has a “imperfect,” but not failing Fontan (AV regurgitation, pulmonary artery hypoplasia, Fontan pressure 18 mmHg)? a) Fenestration closed, room air saturation 97% b) Fenestration patent, room air saturation 92% c) Fenestration patent, room air saturation 88% d) Fenestration patent, room air saturation 85% Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration Closure Does the “perfect” Fontan remain “perfect”?

a) Yes b) No Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration Closure l History of Fontan fenestration l Reported by Bridges in 1990 20 consecutive patient, 19 surviors Goals

w Lower risks of mortality in early post operative period l Intent for transcatheter closure at 3-6 months

* Bridges et al. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for paitents with increased risk. Circ 1990, 82(5):1681-9 Current Controversies Indications for Fenestration Closure l Short term benefits of fenestration Post operative, randomized trial w Decreased duration of pleural effusions (10 v 16 days) w Shortened hospital stay (12 v 23 days)

* Lemier, et al. Fenestration improves clinical outcome of the . A prospective, randomized trial. 2002 Circ 15;105(2):207-12. Current Controversies Indications for Fenestration Closure l Long-term costs of fenestration Fenestrated patients higher incidence of failure Fenestration was used selectively

* Tweddell et al Fontan palliation in the modern era: factors impacting mortality and morbidity. Ann Thorac Surg 2009. 88(4). 1291-9. Current Controversies Indications for Fenestration Closure l Long-term costs of fenestration No deleterious long term effects Fenestration associated with lower saturation w Potentially limiting growth and development Stroke w Multiple studies § Mostly no association

* Atz et al. Late status of Fontan patients with persistent surgical fenestration. JACC. 2011;57:2437-43. Current Controversies Indications for Fenestration Closure l Long term benefits of fenestration closure 10 year post closure Improved oxygen saturation Improved somatic growth Decreased anticongestive medications

* Goff et al. Clinical outcome of fenestrate Fontan patient after closure: the first 10 years. Circ. 2004. 102(17):2094-9. Current Controversies Indications for Fenestration Closure l Closure of venous collaterals Natures fenestration l Mayo Clinic Decreased 5 year survival Following embolization of venous collaterals Cautious closure with Fontan pressure > 18 mmHg

* Poterucha et al. Embolization of veno-venous collaterals after the Fontan operation is associated with decreased survival. Cong Hrt Dis. 2015. On line. Current Controversies Indications for Fenestration Closure Current Controversies Indications for Fenestration Closure l Summary Fenestration beneficial in short term Mixed data for long term

w Better sats when closed

w Maybe protective when patent

w Theoretical risk of stroke

w Utilized for treatment in Fontan failure

§ Create fenestrations for treatment of PLE, plastic bronchitis Current Controversies Indications for Fenestration Closure l Recommendations Leave fenestration alone > 90% Close < 88-90%

w Symptoms (exercise intolerance)

§ Unrelated to poor hemodynamics

w Desire for pregnancy

w Stroke without other cause

w Primary cardiologist and patient family agree Current Controversies Best Coarctation Intervention Current Controversies Best Coarctation Intervention

Who should undergo transcatheter intervention for disrcete juxtaductal coarctation with a normal transverse aortic arch?

a) Everyone

b) > 6 months

c) > 4-5 years (20 kilograms)

d) > 10 years

e) > Only those fully grown Current Controversies Best Coarctation Intervention Current Controversies Best Coarctation Intervention l Options for coarctation intervention Balloon angioplasty Stent angioplasty

w Bare metal

w Covered stent Surgery Current Controversies Best Coarctation Intervention l Ideal coarctation intervention Lowest complications short term w Mortality Lowest residual gradient Lowest complications long term w Anuerysm w Recoarctation

Current Controversies Best Coarctation Intervention l Surgery Most invasive option Long-term follow up data is present Potentially deal with more complex anatomy Lower likelihood of repeat intervention w Not zero

Current Controversies Best Coarctation Intervention l Interventional Balloon angioplasty w Increased risk of aneurysm formation w Serves role § Small children/growing children § Post operative coarctation w Safe and effective therapy Stent angioplasty w Decreased aneurysm formation w Needs reintervention in growing child Current Controversies Best Coarctation Intervention l Comparison of balloon angioplasty to surgery 36 patients, randomized (3-10) years Balloon – higher rate of restenosis and aneursym w Restenosis 25% cath versus 6% surgery w 20% aneurysm in cath group

Shaddy, et al. Comparision of angioplasty and surgery for unoperated coarctation of the . Circ. 1993; 87:793-9.

Current Controversies Best Coarctation Intervention l Long-term outcomes (10 years) Repeat intervention need in both groups Aneurysm formation 35% in angioplasty group 50% of balloon angioplasty w Aneurysm and or repeat intervention

Cowley, et al. Long-Term, ranodmized comparison of balloon angioplasty and surgery for native coarctation of the aorta in childhood. Circ. 2005;111:3453-6. Current Controversies Best Coarctation Intervention l Definition of aneurysm Bulge Outpouching Enlargement Widening Sac l It is what I say it is

. Current Controversies Best Coarctation Intervention Current Controversies Best Coarctation Intervention l Treatment comparison l Non-randomized – Stent, balloon, surgery l Stent and surgery superior hemodynamics l Balloon highest incidence of wall injury l Stent with lower complications Shorter hospital stay l Stent more likely to require reintervention

Forbes et al. Comparison of sugical, stent and balloon angioplasty treatment of native coarctation of the aorta. JACC;58:2664-74 (2011). Current Controversies Best Coarctation Intervention l Long-term follow up data Surgery

• Complications persist

• Hypertension, CVD, CAD, sudden death, aneurysm and rupture

• Balloon/Stent

• Little data l Long-term comparative data Absent Current Controversies Best Coarctation Intervention l Controversy - Stent, balloon, or surgery Size of patient Reoperation Anatomy of the aortic arch

w Transverse arch hypoplasia

w Discrete isthmus narrowing

w Location of narrowing

§ Mid thoracic Current Controversies Best Coarctation Intervention l Controversy – Covered stent or bare metal Age of patient (>50-60) Biscuspid aortic valve Turner’s syndrome Near interruption Everyone? Bailout for rupture or aneurysm Current Controversies Best Coarctation Intervention Current Controversies Best Coarctation Intervention l Summary All three therapies are affective Balloon angioplasty associated with more aneurysms and restenosis Surgery has most long-term data

w Supports close follow up over long-term Stent angioplasty effective in gradient reduction Aneurysms are problem over the long-term Covered stent availability vital to safe intervention

Current Controversies Best Coarctation Intervention l Recommendations (Primary coarctation) < 6 months

w Surgery unless other compelling reason Adult size

w Stent angioplasty

w Single procedure In between

w Based on anatomy

w Family discussion (surgeon and interventionalist)

w Institutional strategy Summary l Entire field contains controversy l Best strategy Understand anatomy and risks Involve multiple providers in the discussion Involve the patient l Field will continue to evolve Questions