What do we really know about the management of patients with congenitally corrected transposition of the great arteries?
2017; Elsevier BV; Volume: 154; Issue: 3 Linguagem: Inglês
10.1016/j.jtcvs.2017.01.055
ISSN1097-685X
Autores Tópico(s)Vascular anomalies and interventions
ResumoCentral MessageDespite innovative surgical procedures, decision making for congenitally corrected transposition of the great arteries remains a challenge.See Article page 1019. Despite innovative surgical procedures, decision making for congenitally corrected transposition of the great arteries remains a challenge. See Article page 1019. Decision making concerning the treatment of congenitally corrected transposition of the great arteries (ccTGA) is challenging because of the rarity of the lesion, combined with wide anatomic variability and the fact that many of the outcomes occur in the distant future. Initially, it was thought that the morphologically right ventricle (RV) could sustain systemic work; indeed, the finding of apparently healthy older individuals with ccTGA without associated lesions was reassuring justification that atrial-level correction of dextrotransposed great arteries would yield an acceptable long-term outcome. Ultimately, however, more systematic analysis of the outcomes of individuals with ccTGA, even those without associated lesions, demonstrated high incidences of heart failure and premature death.1Graham Jr., T.P. Bernard Y.D. Mellen B.G. Celermajer D. Baumgartner H. Cetta F. et al.Long-term outcome in congenitally corrected transposition of the great arteries: a multi-institutional study.J Am Coll Cardiol. 2000; 36: 255-261Crossref PubMed Scopus (502) Google Scholar Results of several large series have demonstrated a 10-year survival between 68% and 88% after physiologic repair (PR) of ccTGA.2Yeh Jr., T. Connelly M.S. Coles J.G. Webb G.D. McLaughlin P.R. Freedom R.M. et al.Atrioventricular discordance: results of repair in 127 patients.J Thorac Cardiovasc Surg. 1999; 117: 1190-1203Abstract Full Text Full Text PDF PubMed Scopus (66) Google Scholar, 3Hraska V. Duncan B.W. Mayer Jr., J.E. Freed M. del Nido P.J. Jonas R.A. Long-term outcome of surgically treated patients with corrected transposition of the great arteries.J Thorac Cardiovasc Surg. 2005; 129: 182-191Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar, 4Shin'oka T. Kurosawa H. Imai Y. Aoki M. Ishiyama M. Sakamoto T. et al.Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients.J Thorac Cardiovasc Surg. 2007; 133 (1328.e1-4): 1318-1328Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar, 5Bogers A.J. Head S.J. de Jong P.L. Witsenburg M. Kappetein A.P. Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation.J Cardiothorac Surg. 2010; 5: 74Crossref PubMed Scopus (28) Google Scholar, 6Lim H.G. Lee J.R. Kim Y.J. Park Y.H. Jun T.G. Kim W.H. et al.Outcomes of biventricular repair for congenitally corrected transposition of the great arteries.Ann Thorac Surg. 2010; 89: 159-167Abstract Full Text Full Text PDF PubMed Scopus (39) Google Scholar Reoperation-free survival has been between 50% and 80%. After PR, patients remain at risk for RV dysfunction with associated tricuspid regurgitation. By 20 years after PR, most patients will have both tricuspid regurgitation and RV dysfunction. Furthermore, survivors of PR have markedly reduced exercise capacity.7Fredriksen P.M. Chen A. Veldtman G. Hechter S. Therrien J. Webb G. Exercise capacity in adult patients with congenitally corrected transposition of the great arteries.Heart. 2001; 85: 191-195Crossref PubMed Scopus (59) Google Scholar In 1990, Ilbawi and colleagues8Ilbawi M.N. DeLeon S.Y. Backer C.L. Duffy C.E. Muster A.J. Zales V.R. et al.An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.J Thorac Cardiovasc Surg. 1990; 100: 410-415Abstract Full Text PDF PubMed Google Scholar first reported anatomic correction of ccTGA with a combination of atrial-level switch operation and the Rastelli procedure to restore the left ventricle (LV) and mitral valve into the systemic circulation. Shortly after the report by Ilbawi and colleagues,8Ilbawi M.N. DeLeon S.Y. Backer C.L. Duffy C.E. Muster A.J. Zales V.R. et al.An alternative approach to the surgical management of physiologically corrected transposition with ventricular septal defect and pulmonary stenosis or atresia.J Thorac Cardiovasc Surg. 1990; 100: 410-415Abstract Full Text PDF PubMed Google Scholar the combination of the atrial-level switch and the arterial switch operations to achieve anatomic repair (AR) of ccTGA was reported by groups in Japan, the United Kingdom, and Australia.9Yamagishi M. Imai Y. Hoshino S. Ishihara K. Koh Y. Nagatsu M. et al.Anatomic correction of atrioventricular discordance.J Thorac Cardiovasc Surg. 1993; 105: 1067-1076Abstract Full Text PDF PubMed Google Scholar, 10Karl T.R. Weintraub R.G. Brizard C.P. Cochrane A.D. Mee R.B. Senning plus arterial switch operation for discordant (congenitally corrected) transposition.Ann Thorac Surg. 1997; 64: 495-502Abstract Full Text PDF PubMed Scopus (85) Google Scholar, 11Stümper O. Wright J.G. De Giovanni J.V. Silove E.D. Sethia B. Brawn W.J. Combined atrial and arterial switch procedure for congenital corrected transposition with ventricular septal defect.Br Heart J. 1995; 73: 479-482Crossref PubMed Scopus (33) Google Scholar Several single-center experiences demonstrated 10-year survivals of 78% to 100% for patients undergoing the atrial plus arterial switch and 60% to 96% for the atrial switch plus the Rastelli procedure.12Duncan B.W. Mee R.B. Mesia C.I. Qureshi A. Rosenthal G.L. Seshadri S.G. et al.Results of the double switch operation for congenitally corrected transposition of the great arteries.Eur J Cardiothorac Surg. 2003; 24 (discussion 19-20): 11-19Crossref PubMed Scopus (78) Google Scholar, 13Sharma R. Talwar S. Marwah A. Shah S. Maheshwari S. Suresh P. et al.Anatomic repair for congenitally corrected transposition of the great arteries.J Thorac Cardiovasc Surg. 2009; 137: 404-412.e4Abstract Full Text Full Text PDF PubMed Scopus (41) Google Scholar, 14Gaies M.G. Goldberg C.S. Ohye R.G. Devaney E.J. Hirsch J.C. Bove E.L. Early and intermediate outcome after anatomic repair of congenitally corrected transposition of the great arteries.Ann Thorac Surg. 2009; 88: 1952-1960Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar, 15Malhotra S.P. Reddy V.M. Qiu M. Pirolli T.J. Barboza L. Reinhartz O. et al.The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: rationale and midterm results.J Thorac Cardiovasc Surg. 2011; 141: 162-170Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar, 16Hraška V. Mattes A. Haun C. Blaschczok H.C. Photiadis J. Murin P. et al.Functional outcome of anatomic correction of corrected transposition of the great arteries.Eur J Cardiothorac Surg. 2011; 40: 1227-1234PubMed Google Scholar, 17Murtuza B. Barron D.J. Stumper O. Stickley J. Eaton D. Jones T.J. et al.Anatomic repair for congenitally corrected transposition of the great arteries: a single-institution 19-year experience.J Thorac Cardiovasc Surg. 2011; 142: 1348-1357.e1Abstract Full Text Full Text PDF PubMed Scopus (95) Google Scholar, 18Hiramatsu T. Matsumura G. Konuma T. Yamazaki K. Kurosawa H. Imai Y. Long-term prognosis of double-switch operation for congenitally corrected transposition of the great arteries.Eur J Cardiothorac Surg. 2012; 42: 1004-1008Crossref PubMed Scopus (42) Google Scholar Ten-year freedom from reoperation after the double switch is 75% to 86%, and that for the atrial switch plus the Rastelli procedure is 57% to 91%. At least 4% of patients will require a pacemaker within 5 years of AR, and pacemaker implantation is strongly associated with LV dysfunction. In addition, sinus node dysfunction is common after the atrial switch operation, occurring in as many as 40% of patients after 20 years, with tachyarrhythmias occurring in 20% by 10 years.19Vetter V.L. Tanner C.S. Horowitz L.N. Electrophysiologic consequences of the Mustard repair of d-transposition of the great arteries.J Am Coll Cardiol. 1987; 10: 1265-1273Crossref PubMed Scopus (73) Google Scholar, 20Dos L. Teruel L. Ferreira I.J. Rodriguez-Larrea J. Miro L. Girona J. et al.Late outcome of Senning and Mustard procedures for correction of transposition of the great arteries.Heart. 2005; 91: 652-656Crossref PubMed Scopus (208) Google Scholar Baffle obstruction also remains a challenge, and the risk of baffle complications is 12% at 25 years.21Hörer J. Haas F. Cleuziou J. Schreiber C. Kostolny M. Vogt M. et al.Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle.Cardiol Young. 2007; 17: 158-165Crossref PubMed Scopus (4) Google Scholar, 22Wells W.J. Blackstone E. Intermediate outcome after Mustard and Senning procedures: a study by the Congenital Heart Surgeons Society.Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2000; 3: 186-197Abstract Full Text PDF PubMed Google Scholar, 23Hörer J. Karl E. Theodoratou G. Schreiber C. Cleuziou J. Prodan Z. et al.Incidence and results of reoperations following the Senning operation: 27 years of follow-up in 314 patients at a single center.Eur J Cardiothorac Surg. 2008; 33 (discussion 1067-8): 1061-1067Crossref PubMed Scopus (42) Google Scholar Reoperation and need for a pacemaker affect the quality of life of survivors of AR.24Gaies M.G. Watnick C.S. Gurney J.G. Bove E.L. Goldberg C.S. Health-related quality of life in patients with congenitally corrected transposition of the great arteries.J Thorac Cardiovasc Surg. 2011; 142: 136-141Abstract Full Text Full Text PDF PubMed Scopus (17) Google Scholar Retraining of the morphologic LV has been proposed for patients with ccTGA to make them candidates for AR. This strategy has been applied not only to individuals who have failing PR but also prophylactically to individuals with ccTGA without associated lesions. LV retraining has achieved a "proof of concept" status, but as yet we do not know the best strategies or who is a candidate. Despite a variety of strategies, LV retraining remains a risk factor for long-term outcome.25Quinn D.W. McGuirk S.P. Metha C. Nightingale P. de Giovanni J.V. Dhillon R. et al.The morphologic left ventricle that requires training by means of pulmonary artery banding before the double-switch procedure for congenitally corrected transposition of the great arteries is at risk of late dysfunction.J Thorac Cardiovasc Surg. 2008; 135 (1144.e1-2): 1137-1144Abstract Full Text Full Text PDF PubMed Scopus (81) Google Scholar Certainly, the published single-center series represent some of the best efforts at AR; however, there appear to be some real challenges with this strategy. In this issue of The Journal of Thoracic and Cardiovascular Surgery, Alomair and colleagues26Alomair M, Morgan C, Al Jughiman M, Caldarone C, Mertens L, Van Arsdell G. Should all patients with congenitally corrected transposition of great arteries undergo anatomic repair? J Thorac Cardiovasc Surg. [In review].Google Scholar from Toronto summarize a 34-year experience with this challenging patient population. They report on the outcomes of 89 patients undergoing PR and 38 patients undergoing AR. The survival trend for AR was not encouraging, with a 20-year survival of 53.6% compared with 65.7% in the PR group. Indeed, for the entire cohort, AR was the only predictor of death or transplant, although AR has not been a predictor of mortality since 2000. Furthermore, there appears to be some advantage to AR with regard to sustained systemic ventricular dysfunction and atrioventricular valve regurgitation. The largest experience comparing the outcomes of PR and AR comes from Japan and includes 189 patients.4Shin'oka T. Kurosawa H. Imai Y. Aoki M. Ishiyama M. Sakamoto T. et al.Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients.J Thorac Cardiovasc Surg. 2007; 133 (1328.e1-4): 1318-1328Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar Similar to the Toronto experience, the series from Tokyo Women's Medical University did not find a survival advantage to the AR strategy except among patients with tricuspid regurgitation. In this issue of the Journal, Hraška and colleagues,27Hraška V. Mitchell M.E. Woods R.K. Frommelt M.A. What surgical improvements are needed to prove that anatomical repair is superior to physiologic repair in the majority of patients with corrected transposition of great arteries?.J Thorac Cardiovasc Surg. 2017; 154: 1019-1022Abstract Full Text Full Text PDF Scopus (7) Google Scholar my friends and colleagues from the Medical College of Wisconsin, have provided a comment on the article by Alomair and colleagues26Alomair M, Morgan C, Al Jughiman M, Caldarone C, Mertens L, Van Arsdell G. Should all patients with congenitally corrected transposition of great arteries undergo anatomic repair? J Thorac Cardiovasc Surg. [In review].Google Scholar from the University of Toronto. Certainly, Hraška and colleagues27Hraška V. Mitchell M.E. Woods R.K. Frommelt M.A. What surgical improvements are needed to prove that anatomical repair is superior to physiologic repair in the majority of patients with corrected transposition of great arteries?.J Thorac Cardiovasc Surg. 2017; 154: 1019-1022Abstract Full Text Full Text PDF Scopus (7) Google Scholar are experts in the management of this challenging group of patients and have advocated for AR. They have offered some well–thought out suggestions that they believe will improve the outcome of AR. Among these are improvements in the atrial switch operation aimed at limiting superior vena cava (SVC) and pulmonary venous baffle obstruction. SVC baffle obstruction remains a challenge because of the unique anatomy of ccTGA. Some have advocated for a bidirectional superior caval connection as part of the correction of ccTGA, but I agree with Hraška and colleagues that this is less than optimal because it limits SVC access for transvenous pacemaker placement and it subjects the patients to a chronically elevated SVC pressure, potentially with venovenous collaterals and potentially with a volume-loading shunt to the RV. Although patch augmentation of the SVC-RV junction might limit the potential for obstruction, other groups have reported very good results without this addition. Hraška and colleagues27Hraška V. Mitchell M.E. Woods R.K. Frommelt M.A. What surgical improvements are needed to prove that anatomical repair is superior to physiologic repair in the majority of patients with corrected transposition of great arteries?.J Thorac Cardiovasc Surg. 2017; 154: 1019-1022Abstract Full Text Full Text PDF Scopus (7) Google Scholar have suggested an improved method for LV retraining. Although this well-reasoned strategy, which combines both pressure and volume loading seems to make sense, there are as yet inadequate data to recommend any specific LV-retraining strategy. Although heart block among patients with normal ventricular looping is rarely associated with LV dysfunction, the association between heart block and LV dysfunction among patients with ccTGA is a troubling reality. Hraška and colleagues27Hraška V. Mitchell M.E. Woods R.K. Frommelt M.A. What surgical improvements are needed to prove that anatomical repair is superior to physiologic repair in the majority of patients with corrected transposition of great arteries?.J Thorac Cardiovasc Surg. 2017; 154: 1019-1022Abstract Full Text Full Text PDF Scopus (7) Google Scholar are correct that every effort should be made to avoid surgical heart block. While the role of biventricular pacing for congenital heart disease in general and for ccTGA in particular, has not been compelety defined the addition of biventricular pacing for patients with ccTGA and heart block would seem to add little additional risk and should be considered. The addition of a Damus-Kaye-Stansel repair as part of the atrial switch Rastelli procedure is an innovative strategy to limit residual or recurrent LV outflow tract obstruction and should be considered as a routine part of the procedure. The combination of the Damus-Kaye-Stansel repair with the Rastelli procedure may limit the need for more radical and higher risk procedures, such as aortic root translocation, that carry a high risk of surgical heart block. Despite all the these well-reasoned and thoughtful modifications to the AR, however, it is unclear whether such change can improve results or extend the use of AR among patients with ccTGA. To summarize, treatment of ccTGA remains a challenge because it is rare, there is wide anatomic variability, and we are most concerned with late outcomes. What we know comes from single-institution retrospective studies that span a long period and in which the surgical strategies were often used in different eras. Interinstitutional outcomes are likely to vary widely because of differences in patient selection and surgical technique. Finally, the LV and perhaps the remaining myocardium of patients with ccTGA may have additional intrinsic abnormalities that render them more prone to development of dysfunction, accelerated by development of conduction abnormalities and complete heart block.28Wallis G.A. Debich-Spicer D. Anderson R.H. Congenitally corrected transposition.Orphanet J Rare Dis. 2011; 6: 22Crossref PubMed Scopus (78) Google Scholar Can we make evidence-based recommendations on the management of patients with ccTGA on the basis of the reported worldwide experience? I think that we can say that for the patient with ccTGA with a nonrestrictive VSD, good RV function and without tricuspid regurgitation, PR or a Fontan procedure is not a wrong choice. For those with tricuspid valve regurgitation or RV dysfunction with good LV function at systemic pressure, an AR is the best choice. The role of LV retraining remains to be defined. What surgical improvements are needed to prove that anatomic repair is superior to physiologic repair in the majority of patients with corrected transposition of the great arteries?The Journal of Thoracic and Cardiovascular SurgeryVol. 154Issue 3PreviewAlomair and colleagues deserve our admiration and respect for their very careful longer-term follow-up of a large cohort of patients with corrected transposition of the great arteries (ccTGA) undergoing physiologic or anatomic repair and their effort to address the oft-debated issue of which approach provides superior long-term outcomes. The cohort included 165 patients amenable to biventricular management spanning the interval from 1982 to 2015. Somewhat less-than-hoped-for results with a conversion to anatomic repair in the 1990s led to a transition back to physiologic repair, again with imperfect results. Full-Text PDF Open Archive
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