Long Term Outcomes of Aortic Root Operations for Marfan Syndrome: A Comparison of Bentall versus Aortic Valve-Sparing Procedures

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1 Long Term Outcomes of Aortic Root Operations for Marfan Syndrome: A Comparison of Bentall versus Aortic Valve-Sparing Procedures Joel Price, MD, J. Trent Magruder, MD, Allen Young, MPH, Joshua C. Grimm, MD, Nishant D. Patel, MD, Diane Alejo, Luca A. Vricella, MD, and Duke E. Cameron, MD From the Division of Cardiac Surgery, Department of Surgery, Johns Hopkins University, Baltimore, Maryland 2015 AATS Annual meeting

2 No disclosures

3 Background Marfan Syndrome: autosomal dominant condition FBN1 gene, incidence of 1 in Aortic root dilation most common feature, part of diagnostic criteria Dismal natural history, aortic dissection and death Prophylactic aortic root replacement in Marfan syndrome improves survival

4 Background Mechanical Composite Valve-Graft Aortic Valve Sparring Root Replacement

5 Background Optimal procedure unknown Bentall versus valve-sparing root replacement (VSRR) Little published data on long-term comparative outcomes

6 Johns Hopkins experience

7 Objectives To compare long-term clinical and valve-related outcomes of Marfan patients undergoing aortic root replacement with mechanical valve-graft or VSRR

8 Methods Adult MFS patients undergoing mechanical composite valve-graft replacement or VSRR procedures Comprehensive follow-up from records and telephone interview Outcomes: Survival, thromboembolism/ hemorrhage, AV endocarditis, aortic valve/root reoperation Statistics: T-tests/chi2/rank-sum tests, Time-to-event analysis, Cox proportional hazards modeling with propensity score adjustment

9 Results

10 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

11 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

12 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

13 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

14 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

15 Baseline characteristics Bentall VSRR p N = Median age (IQR) 37 (31-50) 36 (29-41) 0.03 Male sex (%) 40 (61.5) 66 (72.5) 0.17 Median sinus diameter (cm) (IQR) 5.5 ( ) 5.0 ( ) Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) < /4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

16 Operative data Bentall VSRR p Reimplantation - 69 (70.4) - Remodeling - 29 (29.6) - CPB time Cross-clamp time Concomitant procedures PFO/ASD closure 18 (26.9) 43 (44.3) 0.03 MV repair 2 (3.0) 7 (7.2) 0.31 MV replacement 4 (6.0) CABG 6 (9.0) 2 (2.1) 0.06

17 Operative data Bentall VSRR p Reimplantation - 69 (70.4) - Remodeling - 29 (29.6) - CPB time Cross-clamp time Concomitant procedures PFO/ASD closure 18 (26.9) 43 (44.3) 0.03 MV repair 2 (3.0) 7 (7.2) 0.31 MV replacement 4 (6.0) CABG 6 (9.0) 2 (2.1) 0.06

18 Operative data Bentall VSRR p Reimplantation - 69 (70.4) - Remodeling - 29 (29.6) - CPB time Cross-clamp time Concomitant procedures PFO/ASD closure 18 (26.9) 43 (44.3) 0.03 MV repair 2 (3.0) 7 (7.2) 0.31 MV replacement 4 (6.0) CABG 6 (9.0) 2 (2.1) 0.06

19 Short-term outcomes Bentall VSRR p In-hospital death Pneumothorax 6 (9.0) 7 (7.1) 0.77 Hemothorax 2 (3.0) 1 (1.0) 0.57 Reoperation for bleeding 1 (1.5) 2 (2.0) 1.00 Atrial fibrillation 14 (20.9) 19 (19.4) 0.85

20 Long-term outcomes

21 Survival by procedure type

22 Freedom from thromboembolic events

23 Freedom from hemorrhagic events

24 Freedom from composite outcome

25 Freedom from aortic valve endocarditis

26 Freedom from aortic valve/root reoperation

27 Sub-group analysis Reimplantation vs Remodelling

28 Freedom from aortic valve/root reoperation 100 Freedom from aortic valve/aortic root reop Number at risk Remodeling Reimplantation Log rank p = Years

29 Freedom from moderate/severe AI 100 Freedom from moderate/severe aortic insufficiency Number at risk Remodeling Reimplantation Log rank p = Years

30 Limitations Retrospective study; method of repair chosen at time of operation Relatively small sample size

31 Conclusions VSRR has a significantly decreased risk of composite thromboembolic/hemorrhagic events as compared to Bentall procedures Survival and freedom from endocarditis were similar in both procedure types VSRR has similar durability compared to Bentall procedures Reimplantation VSRR should continue to play an important role in the treatment of well-selected patients with MFS

32 Thank you 2015 AATS Annual meeting

33 Results late events Bentall (n=67) VSRR (n=98) Log rank p Ten-year survival 90.5% 96.3% 0.10 Deaths 6 3 Linearized event rate (%/yr) 1.23% 0.41% Ten-year freedom from thromboembolism/hemorhage 77.6% 96.7% < Thromboembolic/hemorrhagic events 15 4 Linearized event rate (%/yr) 3.50% 0.55% Ten-year freedom from AV/root reoperation 92.0% 92.9% 0.52 AV/root reoperations 4 4 Linearized event rate (%/yr) 0.86% 0.56% Ten-year freedom from endocarditis 91.7% 98.9% 0.14 Endocarditis events 3 1 Linearized event rate (%/yr) 0.17% 0.04%

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