Patrick O. Myers, MD, 1,2 Pedro J. del Nido, MD, 1 Sitaram M. Emani, MD, 1 Gerald R. Marx, MD, 3 Christopher W. Baird, MD 1

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Valve-Sparing Aortic Root Replacement and Remodeling with Complex Aortic Valve Reconstruction in Children and Young Adults with Moderate or Severe Aortic Regurgitation Patrick O. Myers, MD, 1,2 Pedro J. del Nido, MD, 1 Sitaram M. Emani, MD, 1 Gerald R. Marx, MD, 3 Christopher W. Baird, MD 1 1. Cardiac Surgery, Boston Children s Hospital & Harvard Medical School, Boston, MA 2. Cardiovascular Surgery, Geneva University Hospitals, Geneva, Switzerland 3. Cardiology, Boston Children s Hospital & Harvard Medical School, Boston, MA

No conflicts of interest to disclose

Valve-sparing aortic root replacement has obvious advantages over Composite graft root replacement Homograft root replacement Aortic root remodeling Excellent long-term results in adults Background David TE et al. JTCVS 2013;145(3 Suppl):S22 5

Background Little data available in children Patel ND et al. Int Cardiovasc Thorac Surg 2011;12(3):415 9 Roubertie F et al. EJCTS 2009;35(1):136 40 Vricella L et al. Ann Thorac Surg 2005;80(5):1622 6 Rakhra SS et al. JTCVS 2012;144(4):980 1 Myers PO et al. JTCVS 2011;141(1):293 4 Patel ND et al. Int Cardiovasc Thorac Surg 2011;12(3):415 9

Background Aortic regurgitation or anomalous leaflet anatomy usually precludes valve-sparing aortic root procedures Leshnower GL et al. JTCVS 2012;143:879-84

Background Avoiding valve replacement in a growing child is desirable Aortic valve repair in children has shown excellent long-term results Bacha EA et al. JTCVS 2008;135(3):552 9 Myers PO et al. JTCVS 2010;140(4):836 44 Baird CW et al. Semin Thorac Cardiovasc Surg 2012;15(1):9 19 d Udekem Y et al. JTCVS 2013;145(2):461 7 Myers PO et al. JTCVS 2010;140(4):836-44

Background Objective Review our initial experience in combined aortic root replacement or remodeling with complex aortic valve reconstruction

Retrospective chart review 2000-2012 IRB approved Inclusion criteria Methods All patients with aortic root procedures and AVP beyond resuspension Root procedures VSRR: valve-sparing aortic root replacement with reimplantation (David V) VSRr: valve-sparing aortic root remodeling (Yacoub or other)

Methods Primary endpoint: structural valve deterioration (SVD) moderate AR AoV reoperation

Results 34 aortic root and valve reconstruction procedures 13 VSRR 21 VSRr 5 Yacoub 11 sinus reduction or resection 5 subannular reduction and STJ stabilization by TG

Baseline characteristics Variable VSRR VSRr P value Patients 13 21 - Age (years) 14.6±8.7 15.9±8.9 0.66 Median AR grade Moderate Moderate 0.48 Connective tissue disorder Marfan syndrome 1 (7.7%) 2 (9.5%) 1.00 Loeys-Dietz syndrome 3 (23.1%) 1 (4.8%) 0.27 Congenital heart disease 6 (46.2%) 15 (71.4%) 0.17 Cono-truncal anomalies 2 (15.4%) 7 (33.3%) 0.43 Truncus arteriosus 0 (0%) 2 (13.3%) 0.44 DORV 0 (0%) 2 (13.3%) 0.44 AoV disease 5 (62.5%) 5 (33.3%) 0.44 D-TGA 1 (12.5%) 1 (6.7%) 0.44 HLHS / SV 0 (0%) 3 (20%) 0.44 cctga 1 (12.5%) 2 (13.3%) 0.44

Baseline characteristics Variable VSRR VSRr P value Patients 13 21 - Aortic valve anatomy 0.47 Unicuspid 1 (7.7%) 0 (0%) Bicuspid 6 (46.2%) 7 (33.3%) Tricuspid 6 (46.2%) 12 (57.1%) Quadricuspid 0 (0%) 2 (9.5%)

Surgical techniques Variable VSRR VSRr P value Leaflet level-avp 12 (92.3%) 13 (61.9%) 0.11 Commissuroplasty 8 (61.5%) 7 (33.3%) 0.16 Commissurotomy 5 (38.5%) 5 (23.8%) 0.45 Leaflet central plication 2 (15.4%) 1 (4.8%) 0.54 Leaflet thinning 4 (30.8%) 6 (28.6%) 1.00 Leaflet patch augmentation 2 (15.4%) 4 (19%) 1.00 Subannular reduction 2 (15.4%) 13 (61.9%) 0.01

Surgical techniques Variable VSRR VSRr P value Aortic root repair Annular stabilization 11 (84.6%) 14 (66.7%) 0.43 STJ stabilization 13 (100%) 13 (61.9%) 0.01 Combined annular and STJ stabilization 11 (84.6%) 9 (42.9%) 0.03 Aortic root replacement 13 (100%) 5 (23.8%) < 0.001 Ascending aortic replacement 13 (100%) 13 (61.9%) 0.01 Graft size 26 [14-30] 27 [22-30] 0.43

Outcomes No early deaths Mean follow-up: 14.4±2.8 months 7 SVD 5 reoperations for AVR for AR 2 patients with moderate AR 4 VSRR (30.8%) 3 VSRr (14.3%, P = 0.39)

Outcomes

Predictors of SVD Graft size relative to native dimensions Graft to aortic root diameter ratio VSRR 0.62±0.07 VSRr 0.72±0.14 (P = 0.03) Graft to annulus and STJ diameter ratios no different between groups (P = 0.64 and P = 0.69, respectively)

Predictors of SVD Risk factor SVD-free SVD P value Aortic regurgitation grade (median) moderate severe 0.001 Graft to aortic annulus ratio 1.1±0.2 0.9±0.04 0.003

Aortic root dimensions P = 0.001 P > 0.05

Aortic root dimensions P = 0.04 P > 0.05

Aortic root dimensions P = 0.009 P > 0.05

Conclusions Combined valve-sparing root and aortic valve reconstruction Low operative risk Valve preservation in majority at mid-term follow-up Prior reports showed better outcomes of VSRR over VSRr in children Caution with AoV reconstruction? Institutional policy Repair the valve at the time of VSRR or VSRr only when minor defects are present

Valve-Sparing Aortic Root Replacement and Remodeling with Complex Aortic Valve Reconstruction in Children and Young Adults with Moderate or Severe Aortic Regurgitation Patrick O. Myers, MD, 1,2 Pedro J. del Nido, MD, 1 Sitaram M. Emani, MD, 1 Gerald R. Marx, MD, 3 Christopher W. Baird, MD 1 1. Cardiac Surgery, Boston Children s Hospital & Harvard Medical School, Boston, MA 2. Cardiovascular Surgery, Geneva University Hospitals, Geneva, Switzerland 3. Cardiology, Boston Children s Hospital & Harvard Medical School, Boston, MA