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Surgical Management of Complex Aortic Valve Disease in Young Adults: Repair, Replacement, and Future Alternatives

      The ideal aortic valve substitute in young adults remains unknown. Prosthetic valves are associated with a suboptimal survival and carry a significant risk of valve-related complications in young patients, mainly reinterventions with tissue valves and, thromboembolic events and major bleeding with mechanical prostheses. The Ross procedure is the only substitute that restores a survival curve similar to that of a matched general population, and permits a normal life without functional limitations. Though the risk of reintervention is the Achilles’ heel of this procedure, it is very low in patients with aortic stenosis and can be mitigated in patients with aortic regurgitation by tailored surgical techniques. Finally, the Ozaki procedure and the transcatheter aortic valve implantation are seen by many as future alternatives but lack evidence and long-term follow-up in this specific patient population.

      Keywords

      Abbreviations:

      AV (aortic valve), AVNeo (aortic valve neocuspidization), AVR (aortic valve replacement), AR (aortic regurgitation), PPM (patient-prosthesis mismatch), EOA (effective orifice area), RV-PA (right ventricle to pulmonary artery), TAVR (transcatheter aortic valve replacement)
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      References

        • Ruel M
        • et al.
        Long-term outcomes of valve replacement with modern prostheses in young adults.
        Eur J Cardiothorac Surg. 2005; 27 (discussion 433): 425-433
        • Kvidal P
        • et al.
        Long-term follow-up of morbidity and mortality after aortic valve replacement with a mechanical valve prosthesis.
        Eur Heart J. 2000; 21: 1099-1111
        • Bouhout I
        • et al.
        Long-term outcomes after elective isolated mechanical aortic valve replacement in young adults.
        J Thorac Cardiovasc Surg. 2014; 148 (e1): 1341-1346
        • de Meester C
        • et al.
        Valve repair improves the outcome of surgery for chronic severe aortic regurgitation: a propensity score analysis.
        J Thorac Cardiovasc Surg. 2014; 148: 1913-1920
        • Aicher D
        • et al.
        Aortic valve repair leads to a low incidence of valve-related complications.
        Eur J Cardiothorac Surg. 2010; 37: 127-132
        • Bouhout I
        • et al.
        Aortic valve interventions in pediatric patients.
        Semin Thorac Cardiovasc Surg. 2019; 31: 277-287
        • Yanagawa B
        • Mazine A
        • El-Hamamsy I
        Predictors of aortic valve repair failure.
        Innovations (Phila). 2019; 14: 199-1208
        • Pibarot P
        • Dumesnil JG
        Prosthetic heart valves: selection of the optimal prosthesis and long-term management.
        Circulation. 2009; 119: 1034-1048
        • Bakhtiary F.
        • et al.
        Impact of patient-prosthesis mismatch and aortic valve design on coronary flow reserve after aortic valve replacement.
        J Am Coll Cardiol. 2007; 49: 790-796
        • Stassano P
        • et al.
        Aortic valve replacement: a prospective randomized evaluation of mechanical versus biological valves in patients ages 55 to 70 years.
        J Am Coll Cardiol. 2009; 54: 1862-1868
        • Goldstone AB
        • et al.
        Mechanical or biologic prostheses for aortic-valve and mitral-valve replacement.
        N Engl J Med. 2017; 377: 1847-1857
        • Puskas J
        • et al.
        Reduced anticoagulation after mechanical aortic valve replacement: interim results from the prospective randomized on-X valve anticoagulation clinical trial randomized Food and Drug Administration investigational device exemption trial.
        J Thorac Cardiovasc Surg. 2014; 147 (discussion 1210-1): 1202-1210
        • Aicher D
        • et al.
        Quality of life after aortic valve surgery: replacement versus reconstruction.
        J Thorac Cardiovasc Surg. 2011; 142: e19-e24
        • Bouhout I
        • et al.
        Cardiac, obstetric, and fetal outcomes during pregnancy after biological or mechanical aortic valve replacement.
        Can J Cardiol. 2014; 30: 801-807
        • Alsoufi B
        • et al.
        Mechanical valves versus the Ross procedure for aortic valve replacement in children: propensity-adjusted comparison of long-term outcomes.
        J Thorac Cardiovasc Surg. 2009; 137 (e9): 362-370
        • McClure RS
        • et al.
        Late outcomes for aortic valve replacement with the Carpentier-Edwards pericardial bioprosthesis: up to 17-year follow-up in 1,000 patients.
        Ann Thorac Surg. 2010; 89: 1410-1416
        • Webb JG
        • et al.
        3-year outcomes after valve-in-valve transcatheter aortic valve replacement for degenerated bioprostheses: the partner 2 registry.
        J Am Coll Cardiol. 2019; 73: 2647-2655
        • El-Hamamsy I
        • et al.
        Long-term outcomes after autograft versus homograft aortic root replacement in adults with aortic valve disease: a randomised controlled trial.
        Lancet. 2010; 376: 524-531
        • Sharabiani MT
        • et al.
        Aortic valve replacement and the Ross operation in children and young adults.
        J Am Coll Cardiol. 2016; 67: 2858-2870
        • Mazine A
        • et al.
        Ross procedure vs. mechanical aortic valve replacement in adults: a systematic review and meta-analysis.
        JAMA Cardiol. 2018; 3: 978-987
        • David TE
        • et al.
        When is the Ross operation a good option to treat aortic valve disease?.
        J Thorac Cardiovasc Surg. 2010; 139 (discussion 73-5): 68-73
        • Skillington PD
        • et al.
        The Ross procedure using autologous support of the pulmonary autograft: techniques and late results.
        J Thorac Cardiovasc Surg. 2015; 149 (suppl): S46-S52
        • Doss M
        • et al.
        Comparative evaluation of left ventricular mass regression after aortic valve replacement: a prospective randomized analysis.
        J Cardiothorac Surg. 2011; 6: 136
        • Baird CW
        • et al.
        Congenital aortic and truncal valve reconstruction using the Ozaki technique: short-term clinical results.
        J Thorac Cardiovasc Surg. 2021; 161: 1567-1577
        • Bertrand PB
        • et al.
        Effective orifice area during exercise in bileaflet mechanical valve prostheses.
        J Am Soc Echocardiogr. 2017; 30: 404-413

      Supplemental references

        • Forcillo J
        • et al.
        The perimount valve in the aortic position: twenty-year experience with patients under 60 years old.
        Ann Thorac Surg. 2014; 97: 1526-1532
        • Collins J.D.
        • et al.
        Comparison of hemodynamics after aortic root replacement using valve-sparing or bioprosthetic valved conduit.
        Ann Thorac Surg. 2015; 100: 1556-1562
        • Ashikhmina E
        • et al.
        Repair of the bicuspid aortic valve: a viable alternative to replacement with a bioprosthesis.
        J Thorac Cardiovasc Surg. 2010; 139: 1395-1401
        • Price J
        • et al.
        Risk of valve-related events after aortic valve repair.
        Ann Thorac Surg. 2013; 95 (discussion 613): 606-612
        • Arabkhani B
        • et al.
        Reported outcome after valve-sparing aortic root replacement for aortic root aneurysm: a systematic review and meta-analysis.
        Ann Thorac Surg. 2015; 100: 1126-1131
        • Myers PO
        • et al.
        Aortic valve repair by cusp extension for rheumatic aortic insufficiency in children: long-term results and impact of extension material.
        J Thorac Cardiovasc Surg. 2010; 140: 836-844
        • Hawkins JA
        • et al.
        Intermediate-term results of repair for aortic, neoaortic, and truncal valve insufficiency in children.
        J Thorac Cardiovasc Surg. 2007; 133: 1311-1317
        • Kalangos A
        • Myers PO
        Aortic cusp extension for surgical correction of rheumatic aortic valve insufficiency in children.
        World J Pediatr Congenit Heart Surg. 2013; 4: 385-391
        • d'Udekem Y
        • et al.
        Long-term results of a strategy of aortic valve repair in the pediatric population.
        J Thorac Cardiovasc Surg. 2013; 145 (discussion 467-469): 461-467
        • Noly PE
        • et al.
        New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves.
        Can J Cardiol. 2016; 32: 110-116
        • Aicher D
        • et al.
        Aortic valve function after bicuspidization of the unicuspid aortic valve.
        Ann Thorac Surg. 2013; 95: 1545-1550
        • Schafers HJ
        • et al.
        Bicuspidization of the unicuspid aortic valve: a new reconstructive approach.
        Ann Thorac Surg. 2008; 85: 2012-2018
        • Polimenakos AC
        • et al.
        Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: long-term results and freedom from aortic valve replacement.
        J Thorac Cardiovasc Surg. 2010; 139 (discussion 941): 933-941
        • Bacha EA
        • et al.
        Surgical aortic valvuloplasty in children and adolescents with aortic regurgitation: acute and intermediate effects on aortic valve function and left ventricular dimensions.
        J Thorac Cardiovasc Surg. 2008; 135 (559 e1-3): 552-559
        • Wilder TJ
        • et al.
        Aortic valve repair for insufficiency in older children offers unpredictable durability that may not be advantageous over a primary Ross operationdagger.
        Eur J Cardiothorac Surg. 2016; 49: 883-892
        • Poncelet AJ
        • et al.
        Aortic valve repair in the paediatric population: insights from a 38-year single-centre experience.
        Eur J Cardiothorac Surg. 2017; 51: 43-49
        • Khan SS
        • et al.
        Twenty-year comparison of tissue and mechanical valve replacement.
        J Thorac Cardiovasc Surg. 2001; 122: 257-269
        • Mihaljevic T
        • et al.
        Survival after valve replacement for aortic stenosis: implications for decision making.
        J Thorac Cardiovasc Surg. 2008; 135 (discussion 1278-1279): 1270-1278
        • Chambers JB
        • et al.
        Clinical event rates with the on-X bileaflet mechanical heart valve: a multicenter experience with follow-up to 12 years.
        J Thorac Cardiovasc Surg. 2013; 145: 420-424
        • Pibarot P
        • et al.
        Impact of prosthesis-patient mismatch on hemodynamic and symptomatic status, morbidity and mortality after aortic valve replacement with a bioprosthetic heart valve.
        J Heart Valve Dis. 1998; 7: 211-218
        • Johnston DR
        • et al.
        Long-term durability of bioprosthetic aortic valves: implications from 12,569 implants.
        Ann Thorac Surg. 2015; 99: 1239-1247
        • Krayenbuehl HP
        • et al.
        Left-ventricular myocardial structure in aortic-valve disease before, intermediate, and late after aortic-valve replacement.
        Circulation. 1989; 79: 744-755
        • Weber A
        • et al.
        Ten-year comparison of pericardial tissue valves versus mechanical prostheses for aortic valve replacement in patients younger than 60 years of age.
        J Thorac Cardiovasc Surg. 2012; 144: 1075-1083
        • Dunning J
        • et al.
        Aortic valve surgery: marked increases in volume and significant decreases in mechanical valve use—an analysis of 41,227 patients over 5 years from the Society for Cardiothoracic Surgery in Great Britain and Ireland National database.
        J Thorac Cardiovasc Surg. 2011; 142 (e3): 776-782
        • Kulik A
        • et al.
        Mechanical versus bioprosthetic valve replacement in middle-aged patients.
        Eur J Cardiothorac Surg. 2006; 30: 485-491
        • Ikonomidis JS
        • et al.
        Twenty-year experience with the St Jude Medical mechanical valve prosthesis.
        J Thorac Cardiovasc Surg. 2003; 126: 2022-2031
        • Otto CM
        • et al.
        2020 ACC/AHA guideline for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Joint Committee on clinical practice guidelines.
        Circulation. 2021; 143: e72-e227
        • Lancaster TR
        • et al.
        The impact of long-term warfarin therapy on quality of life. Evidence from a randomized trial. Boston Area Anticoagulation Trial for atrial fibrillation investigators.
        Arch Intern Med. 1991; 151: 1944-1949
        • Abildgaard U
        • et al.
        Management of pregnant women with mechanical heart valve prosthesis: thromboprophylaxis with low molecular weight heparin.
        Thromb Res. 2009; 124: 262-267
        • Hung L
        • Rahimtoola SH
        Prosthetic heart valves and pregnancy.
        Circulation. 2003; 107: 1240-1246
        • Yinon Y
        • et al.
        Use of low molecular weight heparin in pregnant women with mechanical heart valves.
        Am J Cardiol. 2009; 104: 1259-1263
        • James AH
        • et al.
        Low-molecular-weight heparin for thromboprophylaxis in pregnant women with mechanical heart valves.
        J Matern Fetal Neonatal Med. 2006; 19: 543-549
        • McLintock C
        • McCowan LM
        • North RA
        Maternal complications and pregnancy outcome in women with mechanical prosthetic heart valves treated with enoxaparin.
        BJOG. 2009; 116: 1585-1592
        • Coskun KO
        • et al.
        Aortic valve surgery in congenital heart disease: a single-center experience.
        Artif Organs. 2010; 34: E85-E90
        • Khan MS
        • et al.
        Contemporary experience with surgical treatment of aortic valve disease in children.
        J Thorac Cardiovasc Surg. 2013; 146 (discussion 520-521): 512-520
        • Xu Z
        • et al.
        Long-term outcomes after mechanical aortic valve replacement with aortic root enlargement in adolescents.
        J Card Surg. 2017; 32: 133-137
        • Arnold R
        • et al.
        Outcome after mechanical aortic valve replacement in children and young adults.
        Ann Thorac Surg. 2008; 85: 604-610
        • Sachweh JS
        • et al.
        Mechanical aortic and mitral valve replacement in infants and children.
        Thorac Cardiovasc Surg. 2007; 55: 156-162
        • Etnel JR
        • et al.
        Outcome after aortic valve replacement in children: a systematic review and meta-analysis.
        J Thorac Cardiovasc Surg. 2016; 151 (e1-3): 143-152
        • Brown JW
        • et al.
        Ross versus non-Ross aortic valve replacement in children: a 22-year single institution comparison of outcomes.
        Ann Thorac Surg. 2016; 101: 1804-1810
        • Masuda M
        • et al.
        Intermediate-term results after the aortic valve replacement using bileaflet mechanical prosthetic valve in children.
        Eur J Cardiothorac Surg. 2008; 34: 42-47
        • Welke KF
        • et al.
        Long-term results after Carpentier-Edwards pericardial aortic valve implantation, with attention to the impact of age.
        Heart Surg Forum. 2011; 14: E160-E165
        • Hammermeister K
        • et al.
        Outcomes 15 years after valve replacement with a mechanical versus a bioprosthetic valve: final report of the Veterans Affairs randomized trial.
        J Am Coll Cardiol. 2000; 36: 1152-1158
        • Nelson JS
        • et al.
        Aortic valve replacement in young and middle-aged adults: current and potential roles of TAVR.
        Ann Thorac Surg. 2021; 112: 132-138
      1. Karamlou, T, et al., Outcomes and associated risk factors for aortic valve replacement in 160 children: a competing-risks analysis. Circulation, 2005. 112: p. 3462-3469.

        • Alsoufi B
        • et al.
        Aortic and mitral valve replacement in children: is there any role for biologic and bioprosthetic substitutes?.
        Eur J Cardiothorac Surg. 2009; 36 (discussion 90): 84-90
        • David TE
        • et al.
        The Ross procedure: outcomes at 20 years.
        J Thorac Cardiovasc Surg. 2014; 147: 85-93
        • Sievers HH
        • et al.
        Fourteen years’ experience with 501 subcoronary Ross procedures: surgical details and results.
        J Thorac Cardiovasc Surg. 2010; 140 (822 e1-5): 816-822
        • Mazine A
        • et al.
        Long-term outcomes of the Ross procedure versus mechanical aortic valve replacement: propensity-matched cohort study.
        Circulation. 2016; 134: 576-585
        • Karaskov A
        • et al.
        Results of the Ross procedure in adults: a single-centre experience of 741 operations.
        Eur J Cardiothorac Surg. 2016; 49: e97-e104
        • David TE
        • et al.
        When is the Ross operation a good option to treat aortic valve disease?.
        J Thorac Cardiovasc Surg. 2010; 139 (discussion 73-75): 68-73
        • Mastrobuoni S
        • et al.
        The Ross procedure in young adults: over 20 years of experience in our institution dagger.
        Eur J Cardiothorac Surg. 2015;
        • Charitos EI
        • et al.
        Long-term results of 203 young and middle-aged patients with more than 10 years of follow-up after the original subcoronary Ross operation.
        Ann Thorac Surg. 2012; 93: 495-502
        • Miskovic A
        • et al.
        A 17-year, single-centre experience with the Ross procedure: fulfilling the promise of a durable option without anticoagulation?.
        Eur J Cardiothorac Surg. 2016; 49 (discussion 519): 514-519
        • Sievers HH
        • et al.
        A multicentre evaluation of the autograft procedure for young patients undergoing aortic valve replacement: update on the German Ross Registrydagger.
        Eur J Cardiothorac Surg. 2016; 49: 212-218
        • Martin E
        • et al.
        Clinical outcomes following the Ross procedure in adults: a 25-year longitudinal study.
        J Am Coll Cardiol. 2017; 70: 1890-1899
        • Buratto E
        • Konstantinov IE
        Commentary: aortic valve surgery in children: repair now, Ross procedure later.
        J Thorac Cardiovasc Surg. 2021;
        • McClure GR
        • et al.
        The Ross procedure versus prosthetic and homograft aortic valve replacement: a systematic review and meta-analysis.
        Eur J Cardiothorac Surg. 2018;
        • Elkins RC
        • et al.
        Ross operation: 16-year experience.
        J Thorac Cardiovasc Surg. 2008; 136 (630 e1-5): 623-630
        • Ryan WH
        • et al.
        The Ross procedure performed for aortic insufficiency is associated with increased autograft reoperation.
        Ann Thorac Surg. 2011; 91 (discussion 69–70): 64-69
        • Juthier F
        • et al.
        Reoperation after the Ross procedure: incidence, management, and survival.
        Ann Thorac Surg. 2012; 93 (discussion 605): 598-604
        • Starnes VA
        • et al.
        Long-term outcomes with the pulmonary autograft inclusion technique in adults with bicuspid aortic valves undergoing the Ross procedure.
        J Thorac Cardiovasc Surg. 2021;
        • Hatoum H
        • Dasi LP
        • Thourani VH
        Commentary: complying with the compliance of Ross procedure reinforcing grafts.
        Semin Thorac Cardiovasc Surg. 2020; 32: 823-824
        • Vanderveken E
        • et al.
        Reinforcing the pulmonary artery autograft in the aortic position with a textile mesh: a histological evaluation.
        Interact Cardiovasc Thorac Surg. 2018; 27: 566-573
        • Puranik R
        • et al.
        Functional outcomes after the Ross (pulmonary autograft) procedure assessed with magnetic resonance imaging and cardiopulmonary exercise testing.
        Heart. 2010; 96: 304-308
        • Notzold A
        • et al.
        Quality of life in aortic valve replacement: pulmonary autografts versus mechanical prostheses.
        J Am Coll Cardiol. 2001; 37: 1963-1966
        • Aicher D
        • et al.
        Quality of life after aortic valve surgery: replacement versus reconstruction.
        J Thorac Cardiovasc Surg. 2011; 142: e19-e24
        • Ozaki S
        • et al.
        Midterm outcomes after aortic valve neocuspidization with glutaraldehyde-treated autologous pericardium.
        J Thorac Cardiovasc Surg. 2018; 155: 2379-2387
        • Wiggins LM
        • et al.
        The utility of aortic valve leaflet reconstruction techniques in children and young adults.
        J Thorac Cardiovasc Surg. 2020; 159: 2369-2378
        • Mack MJ
        • et al.
        Transcatheter aortic-valve replacement with a balloon-expandable valve in low-risk patients.
        N Engl J Med. 2019; 380: 1695-1705
        • Popma JJ
        • et al.
        Transcatheter aortic-valve replacement with a self-expanding valve in low-risk patients.
        N Engl J Med. 2019; 380: 1706-1715
        • Makkar RR
        • et al.
        Association between transcatheter aortic valve replacement for bicuspid vs tricuspid aortic stenosis and mortality or stroke.
        JAMA. 2019; 321: 2193-2202
        • Mangieri A
        • et al.
        Balloon versus self-expandable valve for the treatment of bicuspid aortic valve stenosis: insights from the beat international collaborative registry.
        Circ Cardiovasc Interv. 2020; 13e008714
        • Halim SA
        • et al.
        Outcomes of transcatheter aortic valve replacement in patients with bicuspid aortic valve disease: a report from the Society of Thoracic Surgeons/American College of Cardiology transcatheter valve therapy registry.
        Circulation. 2020; 141: 1071-1079
        • Sondergaard L
        • et al.
        Durability of transcatheter and surgical bioprosthetic aortic valves in patients at lower surgical risk.
        J Am Coll Cardiol. 2019; 73: 546-553
        • Tam D.Y.
        • et al.
        Impact of transcatheter aortic valve durability on life expectancy in low-risk patients with severe aortic stenosis.
        Circulation. 2020; 142: 354-364
        • Makkar RR
        • et al.
        Five-year outcomes of transcatheter or surgical aortic-valve replacement.
        N Engl J Med. 2020; 382: 799-809
        • Zhang S
        • Zhou X
        • Gold MR
        Left bundle branch pacing: JACC review topic of the week.
        J Am Coll Cardiol. 2019; 74: 3039-3049
        • Navarese EP
        • et al.
        Age-related 2-year mortality after transcatheter aortic valve replacement: the young TAVR registry.
        Mayo Clin Proc. 2019; 94: 1457-1466