Multidisciplinary Thoracic Aortic Rounds Foothills Medical Centre 2016 CCS/CSCS/CSVS Joint Position Statement on Open and Endovascular Thoracic Aortic Surgery Jehangir Appoo Multidisciplinary Thoracic Aortic Rounds Foothills Medical Centre January 29th, 2016

Multidisciplinary Thoracic Aortic Rounds History Feedback Content Format

Why 18mins ? long enough to be serious and short enough to hold people’s attention

Why 18mins ? long enough to be serious and short enough to hold people’s attention Speakers have to think about what they want to say. What is the key point they want to communicate? a clarifying effect brings discipline

Why 18mins ? long enough to be serious and short enough to hold people’s attention Speakers have to really think about what they want to say. What is the key point they want to communicate? a clarifying effect brings discipline “Cognitive Backlog” act of listening can be as equally draining as thinking hard about a subject “the more information we are asked to take in, the heavier and heavier it gets. Eventually, we drop it all, failing to remember anything we've been told.”

CCS/CSCS/CSVS Joint Position Statement on Interventions for Thoracic Aortic Disease Presented @ CCC Oct.2015 – Toronto Canadian Journal of Cardiology, In Press

2014 Topics: Size thresholds, Genetics, Medical Therapy, Diagnostic Imaging Surgery and Endovascular Interventions not covered

Process Proposal for Position Statement accepted Nationally Representative Primary Panel Cardiac & Vascular Surgery Focus on novel and emerging technical aspects of thoracic aortic disease interventions Structured and focused literature review Not “expert” consensus opinion Primary literature Existing systematic reviews when present Creation of summary tables

Process GRADE criteria Quality of Evidence: Low, medium, or high Cohort studies, RCTs… Recommendations: graded as strong or weak Quality of evidence Balance btw desired and undesired effects Values and Preferences

Process Voting by Primary Panel Review by International Secondary Panel Review by CCS Guidelines Committee Review by CCS, CSCS, and CSVS Executive *avoided use of “centres of expertise” term in Recommendation

Primary Panel Jehangir Appoo (Co-chair) University of Calgary John Bozinovski University of British Columbia Michael Chu Western University Ismail El-Hamamsy University of Montreal Tom L. Forbes University of Toronto Michael Moon University of Alberta Maral Ouzounian University of Toronto Mark Peterson University of Toronto Jacques Tittley McMaster University Munir Boodhwani (Co-chair) University of Ottawa

Secondary Panel Joseph E. Bavaria University of Pennsylvania Francois Dagenais Laval University Mark Farber University of North Carolina Chad Hughes Duke University Thoralf Sundt Harvard University

Sections Aortic valve preservation and repair Aortic valve replacement in the young Perfusion techniques for aortic arch surgery Total and Hybrid Arch repair Extended repair for type A dissection Total endovascular arch repair Descending thoracic aortic aneurysms Acute type B dissections Chronic type B dissections Document contains total of 20 Recommendations

Highlights Today Aortic valve preservation and repair Aortic valve replacement in the young Perfusion techniques for aortic arch surgery Contemporary total and hybrid arch repair Extended repair for type A dissection Total endovascular arch repair Descending thoracic aortic aneurysms Acute type B dissections Chronic type B dissections 8 recommendations Share some data behind recommendations

Aortic Valve Preservation

Functioning Aortic Valves in Root Aneurysms

17

Free Margin Plication

Reimplanation and BAV repair

Total Follow-up Time: 11,274 pt-years Meta-Analysis Takkenberg Ann Thorac Surg 2015 N = 2,891 Patients Total Follow-up Time: 11,274 pt-years

Early Mortality Pooled Estimate: 1.53% (0.90 – 2.3)

Endocardits Pooled Estimate: 0.23%/pt-yr (0.08 – 0.44)

Thrombo-embolism Pooled Estimate: 0.33%/pt-yr (0.2 – 0.4)

Late AV Reoperation Pooled Estimate: 1.2%/pt-yr (0.6 – 2.0)

PROACT Trial – Mechanical Valve Outcome Low INR Regular INR P-value Neurologic Events 2.07%/pt-yr 1.46 %/pt-yr 0.38 All TE 2.67%/pt-yr 1.59 %/pt-yr 0.16 TE + Thrombosis 2.96%/pt-yr 1.85 %/pt-yr 0.17 Total Mortality 1.48%/pt-yr 1.46%/pt-yr 0.97 Total Bleeding 6.62%/pt-yr <0.001

A Word of Caution Prospective, multi-center, international registry –ao root replacement in Marfans Ao Valve Sparing vs. Replacement 316 pts – 76% AVS Early Mortality 0.6% Early (1-year) AI recurrence 7% JTCVS 2014

#1 We recommend aortic root and ascending aortic aneurysms in patients with normally functioning or mildly regurgitant trileaflet aortic valves be treated with valve sparing operations whenever feasible Strong recommendation Medium quality evidence Values and Preferences: A composite valve and root replacement may be preferred in emergency settings, in elderly patients, those with multiple co-morbidities, poor left ventricular function, or with poor quality cusp tissue. A reimplantation approach to valve sparing root replacement may be preferred in those with connective tissue diseases and bicuspid aortic valves.

Recommendation #2 We suggest aortic root and ascending aortic aneurysms in patients with moderate or greater insufficiency with or without bicuspid aortic valves be considered for valve sparing root replacement with or without cusp repair. Weak recommendation Medium quality evidence Values and Preferences: A number of important considerations should guide this decision including surgeon experience, patient age and preference, quality of cusp tissue, and the ability to perform these procedures with similar mortality and morbidity as composite valve and root replacement procedures.

Considerations for Aortic Valve Replacement inYoung Patients with Aortic Dilatation

Considerations for Aortic Valve Replacement in Young Patients with Aortic Dilatation Aortic valve replacement is required if a successful and durable valve-sparing/repair operation can not be performed The ideal valve substitute remains elusive There is a paucity of data especially in patients with associated aortic dilatation

Considerations for Aortic Valve Replacement in Young Patients with Aortic Dilatation Growing body of literature focusing on this specific patient subset in the last 5-10 years Options Mechanical Tissue Ross Homograft

Operative mortality (%) 5-Year Survival (%) 10-Year Survival (%) Study Study Type Study Period N Mean Follow-up (years) Mean Age BAV (%) AS (%) / AI (%) Mixed AS-AI (%) Operative mortality (%) 5-Year Survival (%) 10-Year Survival (%) 15-Year Survival (%) 5-Year Freedom from reoperation (%) 10-Year Freedom from reoperation (%) 15-Year Freedom from reoperation (%) El-Hamamsy et al. (2010)2 RCT 1994-2001 216 pts (108 Ross) 10.2 (2173 pt-yrs) 38 49% 28% 45% 27% 0.9% 97% 95%** 96% 95% 94% Sievers et al. (2015)3 Multicenter Ross Registry (prospective) 1990-2013 1779 8.3 (14,288 pt-yrs) 44.7 64.8% 24% 22% 52% 1.1% NA 90%** 94.9% 91.1% 82.7% David et al. (2014)4 Single center 1990-2004 212 13.8* 34 71.7% 50% 36% 13% 0.4% 98.6% 97.5% 93.6%** AG 98% HG 100% AG 97% HG 98% AG 93% HG 96% Mastrobuoni et al. (2015)5 1991-2014 306 10.6* 42 58.5% 68% 31% 0% 2.3% 88%** 75% (AS 83%) (AI 65%) Skillington et al. (2013)6 1992-2012 310 9.4 39.3 92% 46% 32% 22% 0.3% 98% 97%** 93% Da Costa et al. (2014)7 1995-2013 414 8.2 30.8 50% 29% 39% 31% 2.7% 89.3%** 81% Kalfa et al. (2015)8 221 11.4* 41.5 76.5% 19% 90.5% 88% Andreas et al. (2014)9 1991-2011 246 10* 29 29% 40% 31% 1.6% 91%**

(Strong recommendation, Medium Quality Evidence) Considerations for Aortic Valve Replacement in Young Patients with Aortic Dilatation #3 We recommend that the Ross procedure be considered as an alternative for prosthetic valve replacement in young adults with bicuspid or tricuspid aortic valve stenosis and aortic dilatation. (Strong recommendation, Medium Quality Evidence) Values and Preferences: The Ross procedure is most appropriate in patients with high levels of physical activity, those contemplating pregnancy and patients with small aortic annuli at risk of patient-prosthesis mismatch. Patients with aortic regurgitation and a dilated annulus may be at higher risk of a late operation. This recommendation elicited varied opinions from the expert panel, but was ultimately approved by the majority of panel members following extensive review of the available literature.

Extended Repair Type A Dissection Goals of Surgery Acute Valvular Insufficiency Ascending aortic rupture Coronary Ischemia But Dissection is a diffuse process involving other organ systems

Case Example: 46y.o male flown in from OSH Hemodynamic shock Abdomen tender Intima intussuscepted through arch

Case Example: 46y.o male flown in from OSH Compromised visceral flow Renal infarct/malperfusion

Case Example: 46y.o male flown in from OSH Both legs: Cold Mottled Pulseless Paralyzed

46y.o male Will visceral, renal, & peripheral malperfusion be resolve?

Extending the Distal Repair THE PROBLEM How much distal aorta should, or must, be repaired in an acute type A aortic dissection Surgical principles Resect dissected aorta Resect primary intimal tear Re-establish flow downstream, preferably in true lumen Obliterate the false lumen Basic techniques Open distal anastomosis Period of circulatory arrest Hypothermia cerebral perfusion during distal aortic repair What we are less certain of is….

Standard hemiarch What most people would consider current standard of care. Extended repair involves moving the distal repair beyond the IA or LCCA in the case of parital arch, or the LSCA in total arch.

Extended Arch

Extending the Distal Repair Is it necessary? Potential risks Longer and more technically challenging operation Potential benefits Seal distal tears Better likelihood of obliterating false lumen Prevention of complications Malperfusion Aortic dilation Re-intervention Death

Extending the Distal Repair What does the literature tell us? Randomized controlled trials? No RCT comparing extent of distal repair in acute type A dissection exist Unlikely for one to be forthcoming To establish whether it is necessary to extend the repair, ideally, we would look at RCTs comparing current standard hemiarch to extended repairs.

The Primary Intimal Tear DOES A STANDARD HEMIARCH ADDRESS ALL PRIMARY INTIMAL TEARS Not all “Only 60 % of patients with acute type A dissections arose from solitary primary intimal tears in the ascending aorta” Lansman et al. Ann Thorac Surg 1999; 67: 1975-1980 Those not in the ascending aorta are not addressed by hemiarch procedures

Extending the Distal Repair RESECTION OF THE PRIMARY INTIMAL TEAR Does it decrease the need for reoperation? Failure to resect the intimal tear was independent determinant for late re-operation in these studies Moon et al. Ann Thorac Surg 2001; 71:1244-1250 In 95 survivors of ATAD repair Kazui et al. Ann Thorac Surg 2002; 74: S1844-1847 In 113 survivors of ATAD repair Zeirer et al. Ann Thorac Surg 2007; 84: 479-487 Odds ratio 4.0 (168 survivors of ATAD repair) So what if you don’t get the primary intimal tear?

Extending the Distal Repair PATENCY OF THE FALSE LUMEN Does it increase risk of death YES Halstead et al. J Thorac Cardiovasc Surg 2007; 133:127-135 179 patients with type A dissections (DeBakey I) Patency of the false lumen was a predictor of death after discharge Sagaguchi et al. ICVTS 2007; 6: 204-208 52 patients So what if you leave he false lumen patent? Is survival any worse? survival months

Extending the Distal Repair PATENCY OF THE FALSE LUMEN Does it increase risk of death NO Kimura et al. J Thorac Cardiovasc Surg 2008; 136:1160-1166 193 patients with type A (DeBakey I) dissections 124 patent false lumen; 69 thrombosed

Extending the Distal Repair PATENCY OF THE FALSE LUMEN Does it increase risk of reoperation YES Sagaguchi et al. ICVTS 2007; 6: 204-208 NO Kimura et al. J Thorac Cardiovasc Surg 2008; 136:1160-1166 Similarly with the risk of reoperation evidence is equivocal

Extending the Distal Repair Malperfusion Patients with malperfusion have an increased mortality Mortality increases depending on number of organ systems involved None 12.6% One 21.3% Two 30.9% Three 43.4% . We know that preoperative malperfusion is a risk for death. GERAADA REGISTRY, JACC 2015; 65(24)

Review of Publications for Extended Distal Repair for Acute Type A Dissection Author (Reference) Year Type of repair N 30-day / in- hospital mortality (%) Permanent Stroke (%) Permane nt SCI (%) Yun et al.10 1991 Total Arch 7 29 N/A Kazui et al.11 2000 70 16 2.9 1.4 Takahara et al.12 2002 37 8.1 Ohtsubo et al.13 24 33.3 12.5 Watanuki et al.14 2007 54 3.7 5.6 Kim et al.15 2011 44 13.4 15.9 2.3 Mizuno et al.16 FET & Total arch 9 11.1 22.2 Tsagakis et al.17 2010 68 13 10 1 Ma et al.18 2013 FET & Total Arch 398 7.8 2.5 Katayama et al.19 2015 120 6 3 2 Roselli et al.20 FET & Hemiarch 17 Pan et al.21 27 Chen et al.22 2014 FET & Hemi Arch 122 4.93 Preventza et al.23 25 12 Vallabhajosyul a et al.24 62 14 8 Diethrich et al.25 2005 Arch Debranching Marullo et al.26 15 4.2 Kent et al.27 2012 Chang et al.28 21 4.8

Summary – Extended Distal Repair Organ malperfusion portends to poorer outcomes The primary intimal tear is not amenable to resection in many acute type A dissections Resection of the primary intimal tear is likely to decrease reoperation rates Obliteration of the false lumen may increase survival & decrease risk of reoperation Extended surgery can be done with similar morbidity and mortality risk

RECOMMENDATIONS #4 We recommend an extended distal arch repair technique be considered for patients who present with acute Type A dissection and one of the following Primary intimal entry tear in the arch or descending aorta Significant aneurysmal disease of the arch (Strong recommendation, Low Quality Evidence)

RECOMMENDATIONS #5 We suggest that it is reasonable to consider an extended distal arch repair technique for patients who present with acute Type A dissection and one of the following: Distal malperfusion Concomitant descending thoracic aortic aneurysm Young patients Patients with connective tissue disorders (Weak recommendation, Low Quality Evidence)

Since 2008, known that in complicated acute Type B Dissection, Endovascular Rx is first line of therapy. Management of Acute Uncomplicated Type B is not as clear

Admission acute Type B 6 months 2 years post Type B “Uncomplicated” Type B - ? misnomer Admission mortality < 10% 5 year mortality substantially higher in some publications 51 y.o male Admission acute Type B 6 months 2 years post Type B

Uncomplicated Type B Medical Management Alone vs. TEVAR & Medical management Instead XL Circ Cardiovasc Int 2013 RCT -140 pts OMT vs. OMT + TEVAR Improved aortic remodelling & aorta specific survival in TEVAR group at 5 years ADSORB European J Vasc Endovasc Surg 2014 RCT 61 pts OMT vs. OMT + TEVAR Improved aortic remodeling at 1 year IRAD Ann Cardiothorac Surg 2014 Retrospective review of registry patients Improved aorta related survival at 5years

Predictors of Growth: Initial aortic diameter > 4cm False Lumen > 22mm Large proximal entry tear >1.0cm

#7 We recommend that patients with uncomplicated acute type B aortic dissections be managed with hypertension and pain control and radiologic surveillance. (Strong Recommendation, Medium quality evidence) Values and Preferences: If patients remains “uncomplicated” early follow up imaging at 48-72 hrs and 1-4 weeks is recommended to detect early signs of aneurysm expansion and radiologic malperfusion.

#8 We suggest that endovascular repair be considered for patients with uncomplicated type B aortic dissections to improve aorta-specific endpoints (Weak recommendation, Low quality evidence) Values and Preferences: The Instead XL trial which randomized patients in the delayed phase (2-52 weeks) showed decreased aorta specific 5-year mortality and improved aortic remodelling. The ADSORB trial which randomized patients in the acute phase (< 2 weeks) showed improvement in aortic remodelling at one year.

Summary: Evolution in open and endovascular aortic surgery Improved patient outcomes Rapid change – thus, little high quality evidence to make strong recommendations New Recommendations: Valve Repair….with caution in regurgitant valves… Extended arch at time of Type A….distal tears, aneurysm – strong recommendation Asymptomatic Type B Dissections….consider early TEVAR – weak recommendation

Highlights Today Aortic valve preservation and repair Aortic valve replacement in the young Perfusion techniques for aortic arch surgery Total and hybrid arch repair Extended repair for type A dissection Total endovascular arch repair Descending thoracic aortic aneurysms Acute type B dissections Chronic type B dissections