Staged Biventricular Repair for Neonates With Left Ventricular Outflow Tract Obstruction, Ventricular Septal Defect, and Aortic Arch Obstruction Mohammad Shihata, MD, Chawki El-Zein, MD, Katie Wittle, APN, Tarek Husayni, MD, and Michel Ilbawi, MD CONGENITAL HEART
Madinah Cardiac Center, Taibah University, Madinah, Saudi Arabia; and Heart Institute for Children, Advocate Children’s Hospital, Oak Lawn, Illinois
Background. The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood operation as a first step of a planned biventricular repair and the impact of associated risk factors. Methods. A retrospective cohort study was performed on all neonates (n [ 44) undergoing the Norwood operation as the first stage of a biventricular (Norwood-Rastelli) repair from January 2000 to December 2012 at a single center. Multivariable analysis was performed to identify predictors of survival. Results. Stage one mortality was 9%. The interstage survival for nonsyndromic and syndromic patients was 100% versus 46%, respectively (p < 0.001). Twenty-four patients (55%) underwent biventricular completion repair with no mortality. Freedom from reintervention after
biventricular completion was 53% at 6 years. The overall survival for nonsyndromic patients versus syndromic patients was 86% versus 43%, respectively (p [ 0.01). Genetic syndromes and prematurity were significant predictors of interstage mortality on multivariable analysis. Conclusions. Staged biventricular repair for patients with complex left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction can be achieved with excellent outcomes for neonates without genetic syndromes. The staged approach is associated with longer time to reintervention after the biventricular completion.
N
the Yasui operation can be performed as a single stage or as a staged repair (Norwood-Rastelli). In the staged approach, a Norwood type repair is performed followed at a later stage by complete septation, channeling the VSD to the native pulmonary valve and establishing right ventricle to pulmonary artery (RV-PA) continuity [5]. Both the anatomic substrate and institutional preference influence the type of surgical repair. The superiority of one approach over the other remains an unanswered question. Complete repair has the advantage of avoiding a shunt type physiology and hypoxemia during early development stages. The staged approach carries less surgical trauma initially and is particularly helpful in borderline left-side heart structures for it preserves the fallback option to a single ventricle repair, should the left ventricle or mitral valve become clearly inadequate on subsequent follow-up. Conversion of biventricular repair to single ventricle repair has been notoriously associated with high mortality [6]. The purpose of this study is to evaluate outcomes of neonates who underwent a Norwood procedure as a first step of a planned biventricular repair and to identify factors that affect these outcomes.
eonates presenting with critical left ventricular outflow tract obstruction (LVOTO), aortic arch obstruction (AAO [interruption or coarctation]), and ventricular septal defect (VSD) can vary in their presentation [1]. These patients are invariably dependent on a patent ductus arteriosus to maintain adequate systemic circulation. Some present with hypoplastic left heart syndrome or one of its variants and are triaged down a single ventricle repair pathway utilizing the Norwood procedure or one of its modifications. Others have a normal left ventricle and mitral valve and are suitable for biventricular repair. Some neonates have borderline left cardiac structures and can present a true challenge in deciding which surgical pathway to follow. If the native left ventricular outflow tract cannot be used as the sole systemic outflow, it needs to be replaced by a Ross or a Ross-Konno operation or augmented with a Damus-Kaye-Stansel procedure, as in the Yasui operation if a VSD is present [2]. The neonatal Ross operation is associated with high mortality, especially when associated with arch repair [3, 4]. Furthermore,
Accepted for publication May 27, 2014. Presented at the Poster Session of the Fiftieth Annual Meeting of The Society of Thoracic Surgeons, Orlando, FL, Jan 25–29, 2014. Address correspondence to Dr Shihata, Madinah Cardiac Center, Taibah University, PO Box 6167, Madinah 41311, Saudi Arabia; e-mail: mshihata@ gmail.com.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2014;98:1394–8) Ó 2014 by The Society of Thoracic Surgeons
Patients and Methods The study was approved by the Institutional Review Board at Advocate Children’s Hospital, Oak Lawn, 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.05.077
Ann Thorac Surg 2014;98:1394–8
SHIHATA ET AL STAGED REPAIR FOR LVOTO, VSD, AND AAO
1395
CONGENITAL HEART
Fig 1. The spectrum of complex left ventricular outflow tract obstruction (LVOTO) and aortic arch obstruction (AAO) and the management algorithm at Advocate Children’s Hospital. (AS ¼ aortic stenosis; AV ¼ aortic valve; CoA ¼ coarctation of aorta; HLHS ¼ hypoplastic left heart syndrome; IAA ¼ interrupted aortic arch; LV ¼ left ventricle; MV ¼ mitral valve; VSD ¼ ventricular septal defect.)
Illinois. All neonates undergoing the Norwood operation (or one of its modifications) as a first stage of a biventricular repair from January 2000 to January 2013 at our hospital were retrospectively analyzed. A combination of complex LVOTO, VSD, and AAO was present in all of the neonates in the study cohort (Fig 1). The mitral valve and left ventricular function and dimensions were normal or near normal (z scores 2 to 2). Genetic testing was performed as part of the routine assessment for neonates referred for cardiac surgery at our center. That includes testing for chromosome 22q11.2 deletion. Variables are expressed as mean SD or proportions, as appropriate. Kaplan-Meier survival curves were used for survival rates at different stages and freedom from reintervention. The Cox proportional hazards model was used to identify significant predictors of survival, with the purposeful model-building method. All statistical analysis was performed using Stata for Windows (Stata Corp, College Station, TX).
Results A total of 44 neonates were identified (Table 1). Aortic arch obstruction (interrupted aortic arch, 70%; CoA, 30%; and VSD 5 mm) was present in all patients. The mean aortic valve annulus size was 3.3 0.8 mm, with a mean z score of 5.9 1.9. Aortic atresia was present in 5 patients (11.4%). DiGeorge syndrome was present in 22 patients (50%), and trisomy 22 in 1 patient. The mean age at stage one was 11 9.6 days. Forty-four patients underwent the Norwood operation leaving a restrictive atrial septal defect (4 mm). The type of shunt was RV-PA in 21 neonates (48%) and a Blalock-Taussig shunt in 20 neonates (45%); 3 patients (7%) underwent a hybrid procedure. Our preference has been to use RV-PA shunt with reversed beveling, as described in a previous
report [7]. Stage one survival to hospital discharge was 91%. The overall survival for nonsyndromic and syndromic patients was 86% and 43%, respectively (p ¼ 0.01), and the interstage survival was 100% and 46%, respectively (p < 0.001; Figs 2, 3). Twenty-one patients (48%) required a catheter-based or surgical interstage procedure (Table 2).
Table 1. Stage One Characteristics Stage One Characteristics Preterm Female Weight, kg Body surface area, m2 Interrupted aortic arch type B Coarctation of aorta Ventricular septal defect Aortic stenosis Aortic atresia Aortic valve annulus, mm Aortic valve, z score Borderline left ventricle Genetic syndrome Age at stage 1, days Norwood Blalock-Taussig Norwood RV-PA shunt Hybrid Extracorporeal membrane oxygenation Ventilation, days Intensive care unit stay, days Hospital stay, days RV-PA ¼ right ventricle-to-pulmonary artery.
% (n) 23 (10) 43 (19) . . 70.5 (31) 29.5 (13) 100 (44) 88.7 (39) 11.3 (5) . . 16 (7) 52.2 (23) . 45 (20) 48 (21) 7 (3) 9.1 (4) . . .
Mean SD . . 2.9 0.19 . . . . . 3.3 5.9 . . 11.7 . . . . 6 11.9 36.3
0.63 0.03
0.8 1.9
9.6
1.5 4 56.7
1396
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Ann Thorac Surg 2014;98:1394–8
Table 2. Interstage Procedures % (n)
Mean SD
. 5 (2) 53 (21) 5 (2) 7.5 (3)
7.6 4.8 . . . .
Procedure Age, months Balloon pulmonary artery plasty Blalock-Taussig shunt Emergency Blalock-Taussig shunt Coarctation of aorta ballooning
CONGENITAL HEART
Fig 2. Comparison of interstage survival of the study cohort based on presence (dotted line) or absence (solid line) of genetic syndromes.
Twenty-four patients underwent biventricular completion repair at a mean age of 19.9 9 months with no deaths. Concomitant VSD enlargement was required in 38% (9 patients). Freedom from any reintervention after biventricular completion was 53% at 6 years. The types of reintervention procedures are listed in Table 3. The presence of genetic syndromes (adjusted hazard ratio for nonsyndromic, 0.06; p ¼ 0.02) and prematurity (adjusted hazard ratio for term neonates, 0.1; p ¼ 0.04) were significant predictors of interstage and overall mortality.
Comment This study was conducted to evaluate the outcomes of a challenging cohort of neonates presenting with AAO, VSD, and complex LVOTO. This combination is rather uncommon in that it represents less than 4% of the LVOTO cohort in the Congenital Heart Surgeons Society studies [8]. In spite of that, a number of small series have attempted to evaluate the best approach to managing this complex problem. It became clear from a number of reports that the Ross-Konno approach in combination with arch augmentation in the neonatal period is
associated with high mortality and failure rates. Our preference is to reserve the Ross-Konno procedure for patients with complex LVOTO who do not require arch augmentation. Whenever possible, this is deferred by various valvuloplasty techniques. Efforts in the more contemporary reports have been directed toward utilizing a Norwood-type procedure in combination with establishing RV-PA continuity in one stage (Yasui operation) or in a staged approach (Norwood-Rastelli). Each of these strategies has its advantages and drawbacks. The small number of patients in each of these studies makes it difficult to draw any solid conclusions. In our opinion, however, we believe that the staged approach is associated with less myocardial trauma and shorter ischemia times in the neonatal period. Furthermore, a staged approach may be necessary in cases with borderline left ventricle size because it allows for following a single ventricle pathway should that be indicated. An additional advantage would be the ability to implant a larger RV-PA conduit, delaying the need for subsequent interventions. The mean RV-PA conduit size at the time of biventricular completion in this cohort was 16 1.2 mm. The striking difference in the interstage mortality between the two groups is hard to attribute to a single cause as most of the deaths occurred out of the hospital. Two patients with 22q11.2 deletion required emergency readmission and extracorporeal support for shunt thrombosis. Both these patient, however, underwent successful biventricular completion and are alive and well to date. In an attempt to provide a collective comparative context, we summarized the approaches and commonly reported outcomes in all published series dealing with this subset of neonates from 1999 onward (Table 4). The decision to use a primary or staged approach in the summarized studies was largely based on the surgeon’s preference and to a lesser extent, the anatomic substrate. The patients in these studies had very similar anatomic substrates but varied on a number of Table 3. Reintervention Procedures After Biventricular Repair Reintervention Procedure
Fig 3. Comparison of overall survival of the study cohort based on presence (dotted line) or absence (solid line) of genetic syndromes.
Right ventricle–PA conduit replacement Patch PA plasty PA stent Ventricular septal defect enlargement Pacemaker PA ¼ pulmonary artery.
% (n) 45.8 8.3 4 8.3 4
(11) (2) (1) (2) (1)
Ann Thorac Surg 2014;98:1394–8
SHIHATA ET AL STAGED REPAIR FOR LVOTO, VSD, AND AAO
1397
Table 4. Summary of Recent Studies on Biventricular Repair of Left Ventricular Outflow Tract Obstruction, Ventricular Septal Defect, and Aortic Arch Obstruction From 1999 to 2013
AAO (%)
Ann Arbor [10], 1999
20
Cincinnati [11], 2003 Philadelphia [12], 2006 Boston [13], 2006 Birmingham, UK [14], 2007 Riyadh, Saudi Arabia [3], 2010 Atlanta [9], 2012 Current study
Series Review [Reference]
SYN (%)
Yasui P Versus S (n)
Early Mortality (%)
BiV (n)
90
NA
P 11, S 9
5%
19
8 21 17 16 14 21
87 29 80 75 79 81
NA 31 18 31 NA 48
S P P P P 13, S 1 P 6, S 15
0% 0% 18% 19% 21% 0%
6 21 17 16 14 21
44
100
52
S
9%
24
AAO ¼ aortic arch obstruction; BiV ¼ biventricular repair; repair (Norwood-Rastelli); SYN ¼ syndromic; y ¼ years.
NA ¼ not applicable;
characteristics, including the prevalence of genetic syndromes and prematurity. Both were found to be significant negative predictors of survival in our analysis, consistent with a recent report by Kanter and coworkers [9]. The lack of a control group makes it difficult to reach conclusions about the superiority of the staged approach in our study. In comparison to previously published reports, however, it seems that the staged approach allows for performing the biventricular completion on larger babies. In conclusion, staged biventricular repair for complex LVOTO, VSD, and AAO is safe, reproducible, and sometimes necessary. It allows for a larger RV-PA conduit at the time of completion, delaying the need for subsequent interventions. Genetic syndromes and prematurity are significant negative predictors of long-term survival.
References 1. Hickey EJ, Caldarone CA, McCrindle BW. Left ventricular hypoplasia: a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. J Am Coll Cardiol 2012;59(Suppl):43–54. 2. Yasui H, Kado H, Nakano E, et al. Primary repair of interrupted aortic arch and severe aortic stenosis in neonates. J Thorac Cardiovasc Surg 1987;93:539–45. 3. Alsoufi B, Al-Halees Z, Manlhiot C, et al. Intermediate results following complex biventricular repair of left ventricular outflow tract obstruction in neonates and infants. Eur J Cardiothorac Surg 2010;38:431–8. 4. Hickey EJ, Caldarone CA, Blackstone EH, et al. Biventricular strategies for neonatal critical aortic stenosis: high mortality associated with early reintervention. J Thorac Cardiovasc Surg 2012;144:409–17.
Overall Survival (%)
nSYN ¼ nonsyndromic;
P (73%), S (89%) 100 95 82 46 79 nSYN 100, SYN 65 nSYN 86, SYN 43
Reintervention After BiV repair (%) NA 33 (3 y) 67 (10 y) 63 (3 y) 80 (5 y) Surgery 43 (5 y) 79% (3 y)
P ¼ primary;
46% (6 y) S ¼ staged
5. Erez E, Tam VK, Kanter KR, Fyfe DA. Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction. Ann Thorac Surg 2001;71:1974–7. 6. Hickey EJ, Caldarone CA, Blackstone EH, et al. Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 2007;134:1429–37. 7. Polimenakos AC, Sathanandam SK, Blair C, El-Zein CF, Husayni TS, Ilbawi MN. Shunt reintervention and timerelated events after Norwood operation: impact of shunt strategy. Ann Thorac Surg 2012;94:1551–61. 8. Alsoufi B, Karamlou T, McCrindle BW, Caldarone CA. Management options in neonates and infants with critical left ventricular outflow tract obstruction. Eur J Cardiothorac Surg 2007;31:1013–21. 9. Kanter KR, Kirshbom PM, Kogon BE. Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles. Ann Thorac Surg 2012;93:1999–2006. 10. Ohye RG, Kagisaki K, Lee LA, Mosca RS, Goldberg CS, Bove EL. Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect. J Thorac Cardiovasc Surg 1999;118:648–53. 11. Pearl JM, Cripe LW, Manning PB. Biventricular repair after Norwood palliation. Ann Thorac Surg 2003;75:132–7. 12. Gruber PJ, Fuller S, Cleaver KM, et al. Early results of singlestage biventricular repair of severe aortic hypoplasia or atresia with ventricular septal defect and normal left ventricle. J Thorac Cardiovasc Surg 2006;132:260–3. 13. Nathan M, Rimmer D, del Nido PJ, et al. Aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect: results of primary biventricular repair in neonates. Ann Thorac Surg 2006;82:2227–32. 14. Moorthy PS, McGuirk SP, Jones TJ, Brawn WJ, Barron DJ. Damus-Rastelli procedure for biventricular repair of aortic atresia and hypoplasia. Ann Thorac Surg 2007; 84:142–6.
INVITED COMMENTARY The constellation of left ventricular outflow tract obstruction (LVOTO), ventricular septal defect (VSD), and aortic arch obstruction (AAO) is a rare anomaly Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
presenting a complex surgical decision in both timing and execution. Both primary and staged repair have been used with success for over 15 years with small series of 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.07.002
CONGENITAL HEART
No. of Cases
Madinah Cardiac Center, Taibah University, Madinah, Saudi Arabia; and Heart Institute for Children, Advocate Children’s Hospital, Oak Lawn, Illinois
Background. The purpose of this study is to evaluate clinical outcomes of neonates who underwent a Norwood operation as a first step of a planned biventricular repair and the impact of associated risk factors. Methods. A retrospective cohort study was performed on all neonates (n [ 44) undergoing the Norwood operation as the first stage of a biventricular (Norwood-Rastelli) repair from January 2000 to December 2012 at a single center. Multivariable analysis was performed to identify predictors of survival. Results. Stage one mortality was 9%. The interstage survival for nonsyndromic and syndromic patients was 100% versus 46%, respectively (p < 0.001). Twenty-four patients (55%) underwent biventricular completion repair with no mortality. Freedom from reintervention after
biventricular completion was 53% at 6 years. The overall survival for nonsyndromic patients versus syndromic patients was 86% versus 43%, respectively (p [ 0.01). Genetic syndromes and prematurity were significant predictors of interstage mortality on multivariable analysis. Conclusions. Staged biventricular repair for patients with complex left ventricular outflow tract obstruction, ventricular septal defect, and aortic arch obstruction can be achieved with excellent outcomes for neonates without genetic syndromes. The staged approach is associated with longer time to reintervention after the biventricular completion.
N
the Yasui operation can be performed as a single stage or as a staged repair (Norwood-Rastelli). In the staged approach, a Norwood type repair is performed followed at a later stage by complete septation, channeling the VSD to the native pulmonary valve and establishing right ventricle to pulmonary artery (RV-PA) continuity [5]. Both the anatomic substrate and institutional preference influence the type of surgical repair. The superiority of one approach over the other remains an unanswered question. Complete repair has the advantage of avoiding a shunt type physiology and hypoxemia during early development stages. The staged approach carries less surgical trauma initially and is particularly helpful in borderline left-side heart structures for it preserves the fallback option to a single ventricle repair, should the left ventricle or mitral valve become clearly inadequate on subsequent follow-up. Conversion of biventricular repair to single ventricle repair has been notoriously associated with high mortality [6]. The purpose of this study is to evaluate outcomes of neonates who underwent a Norwood procedure as a first step of a planned biventricular repair and to identify factors that affect these outcomes.
eonates presenting with critical left ventricular outflow tract obstruction (LVOTO), aortic arch obstruction (AAO [interruption or coarctation]), and ventricular septal defect (VSD) can vary in their presentation [1]. These patients are invariably dependent on a patent ductus arteriosus to maintain adequate systemic circulation. Some present with hypoplastic left heart syndrome or one of its variants and are triaged down a single ventricle repair pathway utilizing the Norwood procedure or one of its modifications. Others have a normal left ventricle and mitral valve and are suitable for biventricular repair. Some neonates have borderline left cardiac structures and can present a true challenge in deciding which surgical pathway to follow. If the native left ventricular outflow tract cannot be used as the sole systemic outflow, it needs to be replaced by a Ross or a Ross-Konno operation or augmented with a Damus-Kaye-Stansel procedure, as in the Yasui operation if a VSD is present [2]. The neonatal Ross operation is associated with high mortality, especially when associated with arch repair [3, 4]. Furthermore,
Accepted for publication May 27, 2014. Presented at the Poster Session of the Fiftieth Annual Meeting of The Society of Thoracic Surgeons, Orlando, FL, Jan 25–29, 2014. Address correspondence to Dr Shihata, Madinah Cardiac Center, Taibah University, PO Box 6167, Madinah 41311, Saudi Arabia; e-mail: mshihata@ gmail.com.
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
(Ann Thorac Surg 2014;98:1394–8) Ó 2014 by The Society of Thoracic Surgeons
Patients and Methods The study was approved by the Institutional Review Board at Advocate Children’s Hospital, Oak Lawn, 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.05.077
Ann Thorac Surg 2014;98:1394–8
SHIHATA ET AL STAGED REPAIR FOR LVOTO, VSD, AND AAO
1395
CONGENITAL HEART
Fig 1. The spectrum of complex left ventricular outflow tract obstruction (LVOTO) and aortic arch obstruction (AAO) and the management algorithm at Advocate Children’s Hospital. (AS ¼ aortic stenosis; AV ¼ aortic valve; CoA ¼ coarctation of aorta; HLHS ¼ hypoplastic left heart syndrome; IAA ¼ interrupted aortic arch; LV ¼ left ventricle; MV ¼ mitral valve; VSD ¼ ventricular septal defect.)
Illinois. All neonates undergoing the Norwood operation (or one of its modifications) as a first stage of a biventricular repair from January 2000 to January 2013 at our hospital were retrospectively analyzed. A combination of complex LVOTO, VSD, and AAO was present in all of the neonates in the study cohort (Fig 1). The mitral valve and left ventricular function and dimensions were normal or near normal (z scores 2 to 2). Genetic testing was performed as part of the routine assessment for neonates referred for cardiac surgery at our center. That includes testing for chromosome 22q11.2 deletion. Variables are expressed as mean SD or proportions, as appropriate. Kaplan-Meier survival curves were used for survival rates at different stages and freedom from reintervention. The Cox proportional hazards model was used to identify significant predictors of survival, with the purposeful model-building method. All statistical analysis was performed using Stata for Windows (Stata Corp, College Station, TX).
Results A total of 44 neonates were identified (Table 1). Aortic arch obstruction (interrupted aortic arch, 70%; CoA, 30%; and VSD 5 mm) was present in all patients. The mean aortic valve annulus size was 3.3 0.8 mm, with a mean z score of 5.9 1.9. Aortic atresia was present in 5 patients (11.4%). DiGeorge syndrome was present in 22 patients (50%), and trisomy 22 in 1 patient. The mean age at stage one was 11 9.6 days. Forty-four patients underwent the Norwood operation leaving a restrictive atrial septal defect (4 mm). The type of shunt was RV-PA in 21 neonates (48%) and a Blalock-Taussig shunt in 20 neonates (45%); 3 patients (7%) underwent a hybrid procedure. Our preference has been to use RV-PA shunt with reversed beveling, as described in a previous
report [7]. Stage one survival to hospital discharge was 91%. The overall survival for nonsyndromic and syndromic patients was 86% and 43%, respectively (p ¼ 0.01), and the interstage survival was 100% and 46%, respectively (p < 0.001; Figs 2, 3). Twenty-one patients (48%) required a catheter-based or surgical interstage procedure (Table 2).
Table 1. Stage One Characteristics Stage One Characteristics Preterm Female Weight, kg Body surface area, m2 Interrupted aortic arch type B Coarctation of aorta Ventricular septal defect Aortic stenosis Aortic atresia Aortic valve annulus, mm Aortic valve, z score Borderline left ventricle Genetic syndrome Age at stage 1, days Norwood Blalock-Taussig Norwood RV-PA shunt Hybrid Extracorporeal membrane oxygenation Ventilation, days Intensive care unit stay, days Hospital stay, days RV-PA ¼ right ventricle-to-pulmonary artery.
% (n) 23 (10) 43 (19) . . 70.5 (31) 29.5 (13) 100 (44) 88.7 (39) 11.3 (5) . . 16 (7) 52.2 (23) . 45 (20) 48 (21) 7 (3) 9.1 (4) . . .
Mean SD . . 2.9 0.19 . . . . . 3.3 5.9 . . 11.7 . . . . 6 11.9 36.3
0.63 0.03
0.8 1.9
9.6
1.5 4 56.7
1396
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Ann Thorac Surg 2014;98:1394–8
Table 2. Interstage Procedures % (n)
Mean SD
. 5 (2) 53 (21) 5 (2) 7.5 (3)
7.6 4.8 . . . .
Procedure Age, months Balloon pulmonary artery plasty Blalock-Taussig shunt Emergency Blalock-Taussig shunt Coarctation of aorta ballooning
CONGENITAL HEART
Fig 2. Comparison of interstage survival of the study cohort based on presence (dotted line) or absence (solid line) of genetic syndromes.
Twenty-four patients underwent biventricular completion repair at a mean age of 19.9 9 months with no deaths. Concomitant VSD enlargement was required in 38% (9 patients). Freedom from any reintervention after biventricular completion was 53% at 6 years. The types of reintervention procedures are listed in Table 3. The presence of genetic syndromes (adjusted hazard ratio for nonsyndromic, 0.06; p ¼ 0.02) and prematurity (adjusted hazard ratio for term neonates, 0.1; p ¼ 0.04) were significant predictors of interstage and overall mortality.
Comment This study was conducted to evaluate the outcomes of a challenging cohort of neonates presenting with AAO, VSD, and complex LVOTO. This combination is rather uncommon in that it represents less than 4% of the LVOTO cohort in the Congenital Heart Surgeons Society studies [8]. In spite of that, a number of small series have attempted to evaluate the best approach to managing this complex problem. It became clear from a number of reports that the Ross-Konno approach in combination with arch augmentation in the neonatal period is
associated with high mortality and failure rates. Our preference is to reserve the Ross-Konno procedure for patients with complex LVOTO who do not require arch augmentation. Whenever possible, this is deferred by various valvuloplasty techniques. Efforts in the more contemporary reports have been directed toward utilizing a Norwood-type procedure in combination with establishing RV-PA continuity in one stage (Yasui operation) or in a staged approach (Norwood-Rastelli). Each of these strategies has its advantages and drawbacks. The small number of patients in each of these studies makes it difficult to draw any solid conclusions. In our opinion, however, we believe that the staged approach is associated with less myocardial trauma and shorter ischemia times in the neonatal period. Furthermore, a staged approach may be necessary in cases with borderline left ventricle size because it allows for following a single ventricle pathway should that be indicated. An additional advantage would be the ability to implant a larger RV-PA conduit, delaying the need for subsequent interventions. The mean RV-PA conduit size at the time of biventricular completion in this cohort was 16 1.2 mm. The striking difference in the interstage mortality between the two groups is hard to attribute to a single cause as most of the deaths occurred out of the hospital. Two patients with 22q11.2 deletion required emergency readmission and extracorporeal support for shunt thrombosis. Both these patient, however, underwent successful biventricular completion and are alive and well to date. In an attempt to provide a collective comparative context, we summarized the approaches and commonly reported outcomes in all published series dealing with this subset of neonates from 1999 onward (Table 4). The decision to use a primary or staged approach in the summarized studies was largely based on the surgeon’s preference and to a lesser extent, the anatomic substrate. The patients in these studies had very similar anatomic substrates but varied on a number of Table 3. Reintervention Procedures After Biventricular Repair Reintervention Procedure
Fig 3. Comparison of overall survival of the study cohort based on presence (dotted line) or absence (solid line) of genetic syndromes.
Right ventricle–PA conduit replacement Patch PA plasty PA stent Ventricular septal defect enlargement Pacemaker PA ¼ pulmonary artery.
% (n) 45.8 8.3 4 8.3 4
(11) (2) (1) (2) (1)
Ann Thorac Surg 2014;98:1394–8
SHIHATA ET AL STAGED REPAIR FOR LVOTO, VSD, AND AAO
1397
Table 4. Summary of Recent Studies on Biventricular Repair of Left Ventricular Outflow Tract Obstruction, Ventricular Septal Defect, and Aortic Arch Obstruction From 1999 to 2013
AAO (%)
Ann Arbor [10], 1999
20
Cincinnati [11], 2003 Philadelphia [12], 2006 Boston [13], 2006 Birmingham, UK [14], 2007 Riyadh, Saudi Arabia [3], 2010 Atlanta [9], 2012 Current study
Series Review [Reference]
SYN (%)
Yasui P Versus S (n)
Early Mortality (%)
BiV (n)
90
NA
P 11, S 9
5%
19
8 21 17 16 14 21
87 29 80 75 79 81
NA 31 18 31 NA 48
S P P P P 13, S 1 P 6, S 15
0% 0% 18% 19% 21% 0%
6 21 17 16 14 21
44
100
52
S
9%
24
AAO ¼ aortic arch obstruction; BiV ¼ biventricular repair; repair (Norwood-Rastelli); SYN ¼ syndromic; y ¼ years.
NA ¼ not applicable;
characteristics, including the prevalence of genetic syndromes and prematurity. Both were found to be significant negative predictors of survival in our analysis, consistent with a recent report by Kanter and coworkers [9]. The lack of a control group makes it difficult to reach conclusions about the superiority of the staged approach in our study. In comparison to previously published reports, however, it seems that the staged approach allows for performing the biventricular completion on larger babies. In conclusion, staged biventricular repair for complex LVOTO, VSD, and AAO is safe, reproducible, and sometimes necessary. It allows for a larger RV-PA conduit at the time of completion, delaying the need for subsequent interventions. Genetic syndromes and prematurity are significant negative predictors of long-term survival.
References 1. Hickey EJ, Caldarone CA, McCrindle BW. Left ventricular hypoplasia: a spectrum of disease involving the left ventricular outflow tract, aortic valve, and aorta. J Am Coll Cardiol 2012;59(Suppl):43–54. 2. Yasui H, Kado H, Nakano E, et al. Primary repair of interrupted aortic arch and severe aortic stenosis in neonates. J Thorac Cardiovasc Surg 1987;93:539–45. 3. Alsoufi B, Al-Halees Z, Manlhiot C, et al. Intermediate results following complex biventricular repair of left ventricular outflow tract obstruction in neonates and infants. Eur J Cardiothorac Surg 2010;38:431–8. 4. Hickey EJ, Caldarone CA, Blackstone EH, et al. Biventricular strategies for neonatal critical aortic stenosis: high mortality associated with early reintervention. J Thorac Cardiovasc Surg 2012;144:409–17.
Overall Survival (%)
nSYN ¼ nonsyndromic;
P (73%), S (89%) 100 95 82 46 79 nSYN 100, SYN 65 nSYN 86, SYN 43
Reintervention After BiV repair (%) NA 33 (3 y) 67 (10 y) 63 (3 y) 80 (5 y) Surgery 43 (5 y) 79% (3 y)
P ¼ primary;
46% (6 y) S ¼ staged
5. Erez E, Tam VK, Kanter KR, Fyfe DA. Successful biventricular repair after initial Norwood operation for interrupted aortic arch with severe left ventricular outflow tract obstruction. Ann Thorac Surg 2001;71:1974–7. 6. Hickey EJ, Caldarone CA, Blackstone EH, et al. Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 2007;134:1429–37. 7. Polimenakos AC, Sathanandam SK, Blair C, El-Zein CF, Husayni TS, Ilbawi MN. Shunt reintervention and timerelated events after Norwood operation: impact of shunt strategy. Ann Thorac Surg 2012;94:1551–61. 8. Alsoufi B, Karamlou T, McCrindle BW, Caldarone CA. Management options in neonates and infants with critical left ventricular outflow tract obstruction. Eur J Cardiothorac Surg 2007;31:1013–21. 9. Kanter KR, Kirshbom PM, Kogon BE. Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles. Ann Thorac Surg 2012;93:1999–2006. 10. Ohye RG, Kagisaki K, Lee LA, Mosca RS, Goldberg CS, Bove EL. Biventricular repair for aortic atresia or hypoplasia and ventricular septal defect. J Thorac Cardiovasc Surg 1999;118:648–53. 11. Pearl JM, Cripe LW, Manning PB. Biventricular repair after Norwood palliation. Ann Thorac Surg 2003;75:132–7. 12. Gruber PJ, Fuller S, Cleaver KM, et al. Early results of singlestage biventricular repair of severe aortic hypoplasia or atresia with ventricular septal defect and normal left ventricle. J Thorac Cardiovasc Surg 2006;132:260–3. 13. Nathan M, Rimmer D, del Nido PJ, et al. Aortic atresia or severe left ventricular outflow tract obstruction with ventricular septal defect: results of primary biventricular repair in neonates. Ann Thorac Surg 2006;82:2227–32. 14. Moorthy PS, McGuirk SP, Jones TJ, Brawn WJ, Barron DJ. Damus-Rastelli procedure for biventricular repair of aortic atresia and hypoplasia. Ann Thorac Surg 2007; 84:142–6.
INVITED COMMENTARY The constellation of left ventricular outflow tract obstruction (LVOTO), ventricular septal defect (VSD), and aortic arch obstruction (AAO) is a rare anomaly Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
presenting a complex surgical decision in both timing and execution. Both primary and staged repair have been used with success for over 15 years with small series of 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.07.002
CONGENITAL HEART
No. of Cases
