DOI: 10.1161/CIRCULATIONAHA.115.017865
The Atrial Level Switch Operation: Lessons Old and New
Running title: Tweddell; Lessons learned from the atrial switch operation
James S. Tweddell, MD
Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Address Address for Correspondence: C rr Co rresp ponden ncee: Jame mess S. me S Tweddell, Twedd dddel elll, MD MD James Cincinnati Children’s Hospital Medical Center 3333 Burnet Ave MLC 2013 Cincinnati, OH 45229 Tel: 513-803-8824 Fax: 513-636-3847 E-mail: [email protected] Journal Subject Code: Cardiovascular (CV) surgery:[41] Pediatric and congenital heart disease, including cardiovascular surgery
Key words: editorial, transposition of great vessels, congenital cardiac defect, transparency, quality improvement
1 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
The atrial level switch operation was a breakthrough in treatment of individuals with Dtransposition of the great arteries (D-TGA). The Senning and Mustard procedures were widely performed in the 1970s and into the 1980s, they relieved cyanosis and established an in-series circulation. It could be argued that the Mustard/Senning procedures heralded the era of radical correction of complex congenital heart disease. In this issue of Circulation, Vejlstrup and colleagues summarize the entire experience with atrial level correction of D-TGA in Sweden and Denmark1. The story is remarkable from an historic perspective and provides a window into the early years of congenital heart surgery but there are also important lessons for the current era not just related to transposition corrected with a Mustard/Senning procedure but also regarding congenital heart surgery in general. From this experience we can learn from the le ess sson onss of on of;; lessons access to care; transparency as well as the limits of physiologic correction of transposition of the grea at ve vess ssel ss elss andd th el the systemic right ventricles. great vessels Most sur urpr ur p isin pr ing was in was the the finding find fi ndin nd i g that in that att the thhe majority majjoriity individuals ind ndiv ivid iv id dua ualls ls with withh transposition traans nspo posi po siti si tionn did ti did not not surprising un nde derg rgo a Mu rg Musta ard/Seennning pr proc o ed dur u e. B ased ed on ep pidem emio em ologi gicc da gi dataa th he aut tho hors estimate esstim imatte thatt im undergo Mustard/Senning procedure. Based epidemiologic the authors ess than tha hann half half of of affected affe af fect fe cted ct ed in indi divi di vidu idual alss unde al un nde derw rwen entt surgical en surg su rgic rg ical ic al correction. corr co rrec rr ecti ec tion ti on. Th on Thee Mu M ust star st ard/ ar d/Se d/ Senn Se nnin nn ingg in less individuals underwent Mustard/Senning procedure was not performed in the neonatal period and enlargement of the atrial septal defect was necessary to improve saturations until corrective surgery was performed generally between 1 and 3 years of age. Certainly one could imagine that diagnosis and access to atrial septectomy/septostomy may be challenging in large countries with remote populations especially during the winter. The message for the current era is the benefit of prenatal diagnosis combined with immediate access to treatment for individuals with complex congenital heart disease. The mortality for the Mustard/Senning procedure in Denmark and Sweden was 20% in addition the authors found a high variability in outcome between centers. The mortality is higher
2 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
than single center series and due in part to publication bias. It is likely that the complete followup available in Denmark and Sweden provides a “real-world” picture of the learning curve and outcome of the Mustard/Senning procedures. The authors correctly point out that the high variability of outcome between centers would not be well tolerated today. In the current era multi-institutional registries such as the Society of Thoracic Surgeons Congenital Heart Database provide “real-world” results for our current surgical strategies2. Transparency of results combined with public reporting could provide realistic expectations as well as identify underperforming centers. Combined with a collaborative, non-punitive approach underperforming centers could benefit through knowledge transfer and identification of nadequate resource allocation. inadequate This is the largest series of long-term follow-up of the Mustard/Senning procedure. In cont tra rast st to to other othe ot h r large la proce cedu ce dures there does not seem du seeem to be a survival contrast series off atrial switch procedures advvantage to the ad he Sen nning ning ooperation pera pe rati ra tion ti on3. Th T The he au authors uth hors fo found ounnd th that a eera raa ooff ssurgery urggery aand ur nd tthe he ppresence reese senc ncee of nc advantage Senning associated as sso soci c ated defects def efectts impacted imp paccted early earl ea r y survival rl surv su viv ival a but butt afte after er thee early earlly po pos postoperative stop oper op e attiv er ve pe per period ri tthe riod he survival suurvvivaal ccurves cu urv rves es aare re rremarkably emar em arka ar kabl ka bly pa bl para parallel. rall ra llel ll el. On el Only ly iimplantation mpla mp lant la ntat nt atiion ooff a pa at pace pacemaker cema ce make ma kerr was ke waas associated asso as soci so ciat ci ated at ed with wit ithh decreased it decr de crea cr ease ea sedd se late survival and is probably a reflection of worsening systemic right ventricular function. The lack of additional factors impacting survival itself demonstrates that long-term outcome of individuals undergoing the Mustard/Senning procedure is dependent on systemic right ventricular function and reflects the limits of physiologic correction. The survivors of the Mustard/Senning procedure will disappear in the next decades but we will continue to care for individuals with systemic right ventricles including those with corrected transposition and an increasing single ventricle population. This analysis by Vejlstrup and colleagues shows us that our efforts should be targeted at preserving systemic ventricular function4,5. We can continue to
3 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
learn from this population, particularly the relationship between systemic right ventricular function and the development of tricuspid regurgitation as well as the trajectory of right ventricular failure compared to left ventricular failure. The paper by Vejlstrup and colleagues provides an historic look at early efforts at the treatment of individuals with transposition. Within this experience are lessons and challenges that apply to us today and into the future.
Conflict of Interest Disclosures: None.
References: 1. Vejlstrup N SK, Mattsson E, Iversen K, Thilen U, Kvidal P, Joahnsson B, Soendergaard Soen ndeerg gaa aard rd L. L. Long-term outcome of Mustard/Senning correction for Transposition of the Great Arteries Arteriies iin Ar n Sweden and Denmark. Circulation. 2015;132:XX-XXX. O'Brien Tchervenkov 2. JJacobs acob ac obs JP ob JP, O'Br Briien SM, Pasquali SK, Jacobs ML, Br ML,, Lacour-Gayet et FG, T chervenkov CI, Austin C,, P Pourmoghadam Scholl FG, Welke Gaynor Clarke Mayer EH,, Pizarro C EH ourm ou mog ogha hada ha d m KK KK,, Sc Scho h lll F G We G, elke KF KF,, Ga G ynor yn or JJW, W, C lark ke DR DR,, Ma aye yerr JE JE,, Mavroudis Variation operations: M avr v oudis C. Va ariiatio on in in outcomes out utccome mess for me foor risk-stratified riskk-sstrattiffiedd pedi ppediatric ediiat a ric cardiac ca c surgical surg giccal ope pera pe r tiion ns: aan n analysis the STS Congenital Heart Surgery Database. 2012;94:564-571; an nallys y is of th he ST TS Co ongeniital al Hea art r S urgery ry Dat tab basse. An Ann Thorac Thor orac Surg. or Sur urgg. 20 2012;94 4:5564-5571; discussion disc sccus ussi sion si on 771-2. 1-2. 2.. 33. Moons P, P Gewillig M, M Sluysmans T, T Verhaaren H, H Viart P, P Massin M, M Suys B, B Budts W, W Pasquet A, De Wolf D, Vliers A. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart. 2004;90:307-313. 4. Di Salvo G, Pacileo G, Rea A, Limongelli G, Baldini L, D'Andrea A, D’Alto M, Sarubbi B, Russo MG, Calabro R. Transverse strain predicts exercise capacity in systemic right ventricle patients. Int J Cardiol. 2010;145:193-196. 5. Szymanski P, Klisiewicz A, Lubiszewska B, Lipczynska M, Michalek P, Janas J, Hoffman P. Application of classic heart failure definitions of asymptomatic and symptomatic ventricular dysfunction and heart failure symptoms with preserved ejection fraction to patients with systemic right ventricles. Am J Cardiol. 2009;104:414-418.
4 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
The Atrial Level Switch Operation: Lessons Old and New James S. Tweddell Circulation. published online July 16, 2015; Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2015 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/early/2015/07/16/CIRCULATIONAHA.115.017865
Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/
Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
The Atrial Level Switch Operation: Lessons Old and New
Running title: Tweddell; Lessons learned from the atrial switch operation
James S. Tweddell, MD
Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Address Address for Correspondence: C rr Co rresp ponden ncee: Jame mess S. me S Tweddell, Twedd dddel elll, MD MD James Cincinnati Children’s Hospital Medical Center 3333 Burnet Ave MLC 2013 Cincinnati, OH 45229 Tel: 513-803-8824 Fax: 513-636-3847 E-mail: [email protected] Journal Subject Code: Cardiovascular (CV) surgery:[41] Pediatric and congenital heart disease, including cardiovascular surgery
Key words: editorial, transposition of great vessels, congenital cardiac defect, transparency, quality improvement
1 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
The atrial level switch operation was a breakthrough in treatment of individuals with Dtransposition of the great arteries (D-TGA). The Senning and Mustard procedures were widely performed in the 1970s and into the 1980s, they relieved cyanosis and established an in-series circulation. It could be argued that the Mustard/Senning procedures heralded the era of radical correction of complex congenital heart disease. In this issue of Circulation, Vejlstrup and colleagues summarize the entire experience with atrial level correction of D-TGA in Sweden and Denmark1. The story is remarkable from an historic perspective and provides a window into the early years of congenital heart surgery but there are also important lessons for the current era not just related to transposition corrected with a Mustard/Senning procedure but also regarding congenital heart surgery in general. From this experience we can learn from the le ess sson onss of on of;; lessons access to care; transparency as well as the limits of physiologic correction of transposition of the grea at ve vess ssel ss elss andd th el the systemic right ventricles. great vessels Most sur urpr ur p isin pr ing was in was the the finding find fi ndin nd i g that in that att the thhe majority majjoriity individuals ind ndiv ivid iv id dua ualls ls with withh transposition traans nspo posi po siti si tionn did ti did not not surprising un nde derg rgo a Mu rg Musta ard/Seennning pr proc o ed dur u e. B ased ed on ep pidem emio em ologi gicc da gi dataa th he aut tho hors estimate esstim imatte thatt im undergo Mustard/Senning procedure. Based epidemiologic the authors ess than tha hann half half of of affected affe af fect fe cted ct ed in indi divi di vidu idual alss unde al un nde derw rwen entt surgical en surg su rgic rg ical ic al correction. corr co rrec rr ecti ec tion ti on. Th on Thee Mu M ust star st ard/ ar d/Se d/ Senn Se nnin nn ingg in less individuals underwent Mustard/Senning procedure was not performed in the neonatal period and enlargement of the atrial septal defect was necessary to improve saturations until corrective surgery was performed generally between 1 and 3 years of age. Certainly one could imagine that diagnosis and access to atrial septectomy/septostomy may be challenging in large countries with remote populations especially during the winter. The message for the current era is the benefit of prenatal diagnosis combined with immediate access to treatment for individuals with complex congenital heart disease. The mortality for the Mustard/Senning procedure in Denmark and Sweden was 20% in addition the authors found a high variability in outcome between centers. The mortality is higher
2 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
than single center series and due in part to publication bias. It is likely that the complete followup available in Denmark and Sweden provides a “real-world” picture of the learning curve and outcome of the Mustard/Senning procedures. The authors correctly point out that the high variability of outcome between centers would not be well tolerated today. In the current era multi-institutional registries such as the Society of Thoracic Surgeons Congenital Heart Database provide “real-world” results for our current surgical strategies2. Transparency of results combined with public reporting could provide realistic expectations as well as identify underperforming centers. Combined with a collaborative, non-punitive approach underperforming centers could benefit through knowledge transfer and identification of nadequate resource allocation. inadequate This is the largest series of long-term follow-up of the Mustard/Senning procedure. In cont tra rast st to to other othe ot h r large la proce cedu ce dures there does not seem du seeem to be a survival contrast series off atrial switch procedures advvantage to the ad he Sen nning ning ooperation pera pe rati ra tion ti on3. Th T The he au authors uth hors fo found ounnd th that a eera raa ooff ssurgery urggery aand ur nd tthe he ppresence reese senc ncee of nc advantage Senning associated as sso soci c ated defects def efectts impacted imp paccted early earl ea r y survival rl surv su viv ival a but butt afte after er thee early earlly po pos postoperative stop oper op e attiv er ve pe per period ri tthe riod he survival suurvvivaal ccurves cu urv rves es aare re rremarkably emar em arka ar kabl ka bly pa bl para parallel. rall ra llel ll el. On el Only ly iimplantation mpla mp lant la ntat nt atiion ooff a pa at pace pacemaker cema ce make ma kerr was ke waas associated asso as soci so ciat ci ated at ed with wit ithh decreased it decr de crea cr ease ea sedd se late survival and is probably a reflection of worsening systemic right ventricular function. The lack of additional factors impacting survival itself demonstrates that long-term outcome of individuals undergoing the Mustard/Senning procedure is dependent on systemic right ventricular function and reflects the limits of physiologic correction. The survivors of the Mustard/Senning procedure will disappear in the next decades but we will continue to care for individuals with systemic right ventricles including those with corrected transposition and an increasing single ventricle population. This analysis by Vejlstrup and colleagues shows us that our efforts should be targeted at preserving systemic ventricular function4,5. We can continue to
3 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
DOI: 10.1161/CIRCULATIONAHA.115.017865
learn from this population, particularly the relationship between systemic right ventricular function and the development of tricuspid regurgitation as well as the trajectory of right ventricular failure compared to left ventricular failure. The paper by Vejlstrup and colleagues provides an historic look at early efforts at the treatment of individuals with transposition. Within this experience are lessons and challenges that apply to us today and into the future.
Conflict of Interest Disclosures: None.
References: 1. Vejlstrup N SK, Mattsson E, Iversen K, Thilen U, Kvidal P, Joahnsson B, Soendergaard Soen ndeerg gaa aard rd L. L. Long-term outcome of Mustard/Senning correction for Transposition of the Great Arteries Arteriies iin Ar n Sweden and Denmark. Circulation. 2015;132:XX-XXX. O'Brien Tchervenkov 2. JJacobs acob ac obs JP ob JP, O'Br Briien SM, Pasquali SK, Jacobs ML, Br ML,, Lacour-Gayet et FG, T chervenkov CI, Austin C,, P Pourmoghadam Scholl FG, Welke Gaynor Clarke Mayer EH,, Pizarro C EH ourm ou mog ogha hada ha d m KK KK,, Sc Scho h lll F G We G, elke KF KF,, Ga G ynor yn or JJW, W, C lark ke DR DR,, Ma aye yerr JE JE,, Mavroudis Variation operations: M avr v oudis C. Va ariiatio on in in outcomes out utccome mess for me foor risk-stratified riskk-sstrattiffiedd pedi ppediatric ediiat a ric cardiac ca c surgical surg giccal ope pera pe r tiion ns: aan n analysis the STS Congenital Heart Surgery Database. 2012;94:564-571; an nallys y is of th he ST TS Co ongeniital al Hea art r S urgery ry Dat tab basse. An Ann Thorac Thor orac Surg. or Sur urgg. 20 2012;94 4:5564-5571; discussion disc sccus ussi sion si on 771-2. 1-2. 2.. 33. Moons P, P Gewillig M, M Sluysmans T, T Verhaaren H, H Viart P, P Massin M, M Suys B, B Budts W, W Pasquet A, De Wolf D, Vliers A. Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium. Heart. 2004;90:307-313. 4. Di Salvo G, Pacileo G, Rea A, Limongelli G, Baldini L, D'Andrea A, D’Alto M, Sarubbi B, Russo MG, Calabro R. Transverse strain predicts exercise capacity in systemic right ventricle patients. Int J Cardiol. 2010;145:193-196. 5. Szymanski P, Klisiewicz A, Lubiszewska B, Lipczynska M, Michalek P, Janas J, Hoffman P. Application of classic heart failure definitions of asymptomatic and symptomatic ventricular dysfunction and heart failure symptoms with preserved ejection fraction to patients with systemic right ventricles. Am J Cardiol. 2009;104:414-418.
4 Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
The Atrial Level Switch Operation: Lessons Old and New James S. Tweddell Circulation. published online July 16, 2015; Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 2015 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539
The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/early/2015/07/16/CIRCULATIONAHA.115.017865
Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/
Downloaded from http://circ.ahajournals.org/ at University of Toronto on July 20, 2015
