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

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

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Background Marfan Syndrome: autosomal dominant condition FBN1 gene, incidence of 1 in 3000-5000 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

Background Mechanical Composite Valve-Graft Aortic Valve Sparring Root Replacement

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

Johns Hopkins experience

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

Methods Adult MFS patients undergoing mechanical composite valve-graft replacement or VSRR procedures 1997 2014 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

Results

Baseline characteristics Bentall VSRR p N = 165 67 98 - 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 (5.0-6.0) 5.0 (4.9-5.3) 0.003 Bicuspid valve (%) 5 (7.4) 2 (2.1) 0.20 Aortic dissection (%) 17 (25.4) 4 (4.1) <0.001 3+/4+ AI (%) 33 (49.3) 14 (14.4) <0.001 Urgent/emergent operation (%) 16 (24.6) 3 (3.3) <0.001

Operative data Bentall VSRR p Reimplantation - 69 (70.4) - Remodeling - 29 (29.6) - CPB time 158.8 143.6 0.08 Cross-clamp time 111.6 104.2 0.25 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) 0 0.03 CABG 6 (9.0) 2 (2.1) 0.06

Short-term outcomes Bentall VSRR p In-hospital death 0 0 1.00 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

Long-term outcomes

Survival by procedure type

Freedom from thromboembolic events

Freedom from hemorrhagic events

Freedom from composite outcome

Freedom from aortic valve endocarditis

Freedom from aortic valve/root reoperation

Sub-group analysis Reimplantation vs Remodelling

Freedom from aortic valve/root reoperation 100 Freedom from aortic valve/aortic root reop 0 20 40 60 80 Number at risk Remodeling Reimplantation Log rank p = 0.11 0 5 10 15 Years 29 22 16 6 69 42 17 0

Freedom from moderate/severe AI 100 Freedom from moderate/severe aortic insufficiency 0 20 40 60 80 Number at risk Remodeling Reimplantation Log rank p = 0.32 0 5 10 15 Years 17 9 5 1 61 19 5 0

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

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

Thank you 2015 AATS Annual meeting

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% < 0.001 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%