Commentary: Standardized aortic valve repair in pediatric patients

Abstract

Central MessageA standardized bicuspidizing repair is an effective and reproducible approach for unicuspid aortic valves in pediatric patients. Functional results are superior to individualized repair procedures.See Article page XXX.Unicuspid aortic valve (UAV) morphology is one of the congenital variants, presenting as stenosis, mixed dysfunction, or pure regurgitation,1Noly P.E. Basmadjian L. Bouhout I. Viet Le V.H. Poirier N. El-Hamamsy I. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves.Can J Cardiol. 2016; 32: 110-116Google Scholar and it is frequently mistaken for bicuspid aortic valve morphology.1Noly P.E. Basmadjian L. Bouhout I. Viet Le V.H. Poirier N. El-Hamamsy I. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves.Can J Cardiol. 2016; 32: 110-116Google Scholar,2Roberts W.C. Ko J.M. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.Circulation. 2005; 111: 920-925Google Scholar Importantly, patients with UAV require treatment at a young age,2Roberts W.C. Ko J.M. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.Circulation. 2005; 111: 920-925Google Scholar at times even as neonates or infants.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar UAV morphology has been consistently found in a series of patients with congenital aortic stenosis.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar Congenital stenosis and the sequelae of its treatment—either by commissurotomy or by balloon valvuloplasty—are thus important substrates for cardiac surgery in the pediatric age group.Surgical management of aortic valve dysfunction in pediatric patients must accommodate their specific characteristics. Replacement with a mechanical or biological prosthesis has its inherent difficulties, including excess mortality.4Sharabiani M.T. Dorobantu D.M. Mahani A.S. Turner M. Peter Tometzki A.J. Angelini G.D. et al.Aortic valve replacement and the Ross operation in children and young adults.J Am Coll Cardiol. 2016; 67: 2858-2870Google Scholar Repair has been performed using different techniques by different groups but not achieved predictable results. The pulmonary autograft is an invasive procedure and not without the need for reoperations.5Danial P. Neily A. Pontailler M. Gaudin R. Khraiche D. Osborne-Pellegrin M. et al.Ross procedure or complex aortic valve repair using pericardium in children: a real dilemma.J Thorac Cardiovasc Surg. 2022; 163: 1180-1191.e6Google Scholar More recently, replacement with autologous pericardium6Mylonas K.S. Tasoudis P.T. Pavlopoulos D. Kanakis M. Stavridis G.T. Avgerinos D.V. Aortic valve neocuspidization using the Ozaki technique: a meta-analysis of reconstructed patient-level data.Am Heart J. 2022; 255: 1-11Google Scholar has become popular. With this procedure, however, aortic valve function depends completely on the pericardium, which will degenerate.7Karliova I. Schneider U. Ehrlich T. Schäfers H.J. Results of pericardial patches in tricuspid and bicuspid aortic cusp repair.Ann Thorac Surg. 2020; 109: 728-735https://doi.org/10.1016/j.athoracsur.2019.07.025Google ScholarWe have been stimulated by the characteristics of UAV anatomy to develop a standardized repair concept. The unicommissural UAV is characterized by hypoplasia of 2 commissures with variable degrees of cusp fusion. There is one normally developed commissure without cusp fusion in its vicinity.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar Thus, the respective part of the valve has normal configuration and tissue quality, and the pathology is commonly defined to the anterior part with abnormal commissures. This key finding encouraged us to repair the valve by creating a symmetric bicuspid valve with a second functional commissure, adding tissue as needed.8Schäfers H.J. Aicher D. Riodionycheva S. Lindinger A. Rädle-Hurst T. Langer F. et al.Bicuspidization of the unicuspid aortic valve: a new reconstructive approach.Ann Thorac Surg. 2008; 85: 2012-2018Google Scholar The amount of tissue added, eg, pericardium, is thus limited to generally less than 30% of cusp size in children.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar Over time, the patch will degenerate9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar; degeneration commonly spares the native cusp tissue for some time, extending the durability of the valve beyond that of the patch tissue.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar Good hemodynamics have been achieved with this approach.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google ScholarAny surgical approach must be both effective and reproducible in order to be of value for a larger patient population. The current report10Chiu P. Chávez M. Zubair M.M. Friedman K. Marx G.R. del Nido P.J. et al.Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.J Thorac Cardiovasc Surg. October 14, 2022; ([Epub ahead of print])Google Scholar is important in 2 respects. First, it confirms the effectiveness of the procedure over more individualized repair operations. Second, it demonstrates the reproducibility of the concept, consisting of the creation of a second commissure, adding limited tissue, and judging cusp configuration by measurement of effective height. The authors have carried the concept even further by using the native right cusp tissue and attaching it to the aortic wall to achieve the intended configuration, thus minimizing the need for patch material. This experience may further support the concept of choosing aortic valve repair as the first surgical step in treating congenital aortic valve disease, postponing replacement to a later age.5Danial P. Neily A. Pontailler M. Gaudin R. Khraiche D. Osborne-Pellegrin M. et al.Ross procedure or complex aortic valve repair using pericardium in children: a real dilemma.J Thorac Cardiovasc Surg. 2022; 163: 1180-1191.e6Google Scholar A standardized bicuspidizing repair is an effective and reproducible approach for unicuspid aortic valves in pediatric patients. Functional results are superior to individualized repair procedures. A standardized bicuspidizing repair is an effective and reproducible approach for unicuspid aortic valves in pediatric patients. Functional results are superior to individualized repair procedures. See Article page XXX. See Article page XXX. Unicuspid aortic valve (UAV) morphology is one of the congenital variants, presenting as stenosis, mixed dysfunction, or pure regurgitation,1Noly P.E. Basmadjian L. Bouhout I. Viet Le V.H. Poirier N. El-Hamamsy I. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves.Can J Cardiol. 2016; 32: 110-116Google Scholar and it is frequently mistaken for bicuspid aortic valve morphology.1Noly P.E. Basmadjian L. Bouhout I. Viet Le V.H. Poirier N. El-Hamamsy I. New insights into unicuspid aortic valve disease in adults: not just a subtype of bicuspid aortic valves.Can J Cardiol. 2016; 32: 110-116Google Scholar,2Roberts W.C. Ko J.M. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.Circulation. 2005; 111: 920-925Google Scholar Importantly, patients with UAV require treatment at a young age,2Roberts W.C. Ko J.M. Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.Circulation. 2005; 111: 920-925Google Scholar at times even as neonates or infants.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar UAV morphology has been consistently found in a series of patients with congenital aortic stenosis.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar Congenital stenosis and the sequelae of its treatment—either by commissurotomy or by balloon valvuloplasty—are thus important substrates for cardiac surgery in the pediatric age group. Surgical management of aortic valve dysfunction in pediatric patients must accommodate their specific characteristics. Replacement with a mechanical or biological prosthesis has its inherent difficulties, including excess mortality.4Sharabiani M.T. Dorobantu D.M. Mahani A.S. Turner M. Peter Tometzki A.J. Angelini G.D. et al.Aortic valve replacement and the Ross operation in children and young adults.J Am Coll Cardiol. 2016; 67: 2858-2870Google Scholar Repair has been performed using different techniques by different groups but not achieved predictable results. The pulmonary autograft is an invasive procedure and not without the need for reoperations.5Danial P. Neily A. Pontailler M. Gaudin R. Khraiche D. Osborne-Pellegrin M. et al.Ross procedure or complex aortic valve repair using pericardium in children: a real dilemma.J Thorac Cardiovasc Surg. 2022; 163: 1180-1191.e6Google Scholar More recently, replacement with autologous pericardium6Mylonas K.S. Tasoudis P.T. Pavlopoulos D. Kanakis M. Stavridis G.T. Avgerinos D.V. Aortic valve neocuspidization using the Ozaki technique: a meta-analysis of reconstructed patient-level data.Am Heart J. 2022; 255: 1-11Google Scholar has become popular. With this procedure, however, aortic valve function depends completely on the pericardium, which will degenerate.7Karliova I. Schneider U. Ehrlich T. Schäfers H.J. Results of pericardial patches in tricuspid and bicuspid aortic cusp repair.Ann Thorac Surg. 2020; 109: 728-735https://doi.org/10.1016/j.athoracsur.2019.07.025Google Scholar We have been stimulated by the characteristics of UAV anatomy to develop a standardized repair concept. The unicommissural UAV is characterized by hypoplasia of 2 commissures with variable degrees of cusp fusion. There is one normally developed commissure without cusp fusion in its vicinity.3McKay R. Smith A. Leung M.P. Arnold R. Anderson R.H. Morphology of the ventriculoaortic junction in critical aortic stenosis. Implications for hemodynamic function and clinical management.J Thorac Cardiovasc Surg. 1992; 104: 434-442Google Scholar Thus, the respective part of the valve has normal configuration and tissue quality, and the pathology is commonly defined to the anterior part with abnormal commissures. This key finding encouraged us to repair the valve by creating a symmetric bicuspid valve with a second functional commissure, adding tissue as needed.8Schäfers H.J. Aicher D. Riodionycheva S. Lindinger A. Rädle-Hurst T. Langer F. et al.Bicuspidization of the unicuspid aortic valve: a new reconstructive approach.Ann Thorac Surg. 2008; 85: 2012-2018Google Scholar The amount of tissue added, eg, pericardium, is thus limited to generally less than 30% of cusp size in children.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar Over time, the patch will degenerate9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar; degeneration commonly spares the native cusp tissue for some time, extending the durability of the valve beyond that of the patch tissue.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar Good hemodynamics have been achieved with this approach.9Igarashi T. Matsushima S. Shimizu A. Ehrlich T. Karliova I. Schäfers H.J. Bicuspidization and annuloplasty provide a functioning configuration to the unicuspid aortic valve.Ann Thorac Surg. 2020; 110: 111-119Google Scholar Any surgical approach must be both effective and reproducible in order to be of value for a larger patient population. The current report10Chiu P. Chávez M. Zubair M.M. Friedman K. Marx G.R. del Nido P.J. et al.Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve disease.J Thorac Cardiovasc Surg. October 14, 2022; ([Epub ahead of print])Google Scholar is important in 2 respects. First, it confirms the effectiveness of the procedure over more individualized repair operations. Second, it demonstrates the reproducibility of the concept, consisting of the creation of a second commissure, adding limited tissue, and judging cusp configuration by measurement of effective height. The authors have carried the concept even further by using the native right cusp tissue and attaching it to the aortic wall to achieve the intended configuration, thus minimizing the need for patch material. This experience may further support the concept of choosing aortic valve repair as the first surgical step in treating congenital aortic valve disease, postponing replacement to a later age.5Danial P. Neily A. Pontailler M. Gaudin R. Khraiche D. Osborne-Pellegrin M. et al.Ross procedure or complex aortic valve repair using pericardium in children: a real dilemma.J Thorac Cardiovasc Surg. 2022; 163: 1180-1191.e6Google Scholar Symmetric bicuspidizing repair for patients with congenital aortic or truncal valve diseaseThe Journal of Thoracic and Cardiovascular SurgeryPreviewSymmetric bicuspidizing repair has been shown to be safe and effective in the short term in adults and children with unicuspid aortic valve. Outcomes of extending this technique to patients with other forms of aortic and truncal valve disease have not been reported. Full-Text PDF