The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study

V.L. Vida, A. Guariento, O. Milanesi, D. Gregori, G. Stellin, F. Zucchetta, L. Zanotto, M.A. Padalino, B. Castaldi, S. Bosiznik, R. Crepaz, J. Stuefer, F. De Maria Garcia Gonzales, A.R. Castaneda, G. Crupi, G. Agnoletti, S. Bondanza, M. Marasini, L. Zannini, G. Butera & 78 others A. Frigiola, A. Varrica, E. Chiappa, M. Pilati, A. Carotti, T. Matteo, D. Prandstraller, G. Gargiulo, M. Giovanna Russo, G. Santoro, G. Caianiello, I. Spadoni, B. Murzi, L. Arcieri, M. Pozzi, G. Porcedda, H. Berggren, T. Carrel, A. Kadner, S. Çiçek, Y. Zorman, J. Fragata, A. Gordo, M. Hazekamp, V. Sojak, V. Hraska, B. Asfour, B. Maruszewski, M. Kozlowski, D. Metras, R. Pretre, J. Rubay, H. Sairanen, G. Sarris, C. Schreiber, M. Ono, B. Meyns, K. Van Den Bossche, T. Tlaskal, M. Lo Rito, S. Joon Yoo, G.S. Van Arsdell, C. Calderone, Y. Iwamoto, J. Leon-Wyss, S. Di Filippo, C. Leconte, B.J. Mulder, T. Ebels, S. Arrigoni, E. Valsangiacomo, D. Hitendu, I.E. Konstantinov, A. Gamillscheg, D. Gabriela, U. Herberg, Y. Dulac, J. Edmerger, A. Zarate Fuentes, J. Miguel Gil Jaurena, I. Bo, O. Ghez, M.L. Rigby, E.A. Bacha, D. Kalfa, S. Speggiorin, F. Bu'Lock, M. Al-Ahmadi, G. Di Salvo, R. Surmacz, I.M. Yemets, Y.B. Mykychak, I. Lugones, D.E. Cameron, L.A. Vricella, C.J. Troconis, G. Thiene, A. Angelini

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P <0.001) and pulmonary hypertension (P <0.001). Most patients were asymptomatic at last follow-up (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P <0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02-0.28). Many STPs (63/254, 25%) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age. © The Author 2017.
Original languageEnglish
Pages (from-to)1002-1011
Number of pages10
JournalEuropean Heart Journal
Volume39
Issue number12
DOIs
Publication statusPublished - 2018

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Scimitar Syndrome
Natural History
Confidence Intervals
Cytidine Monophosphate
Survival
Odds Ratio
Pulmonary Hypertension
Drainage
Pathologic Constriction
Registries
Heart Diseases

Keywords

  • Congenital heart defect
  • Multi-centre study
  • Natural history
  • Surgery

Cite this

Vida, V. L., Guariento, A., Milanesi, O., Gregori, D., Stellin, G., Zucchetta, F., ... Angelini, A. (2018). The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study. European Heart Journal, 39(12), 1002-1011. https://doi.org/10.1093/eurheartj/ehx526
Vida, V.L. ; Guariento, A. ; Milanesi, O. ; Gregori, D. ; Stellin, G. ; Zucchetta, F. ; Zanotto, L. ; Padalino, M.A. ; Castaldi, B. ; Bosiznik, S. ; Crepaz, R. ; Stuefer, J. ; De Maria Garcia Gonzales, F. ; Castaneda, A.R. ; Crupi, G. ; Agnoletti, G. ; Bondanza, S. ; Marasini, M. ; Zannini, L. ; Butera, G. ; Frigiola, A. ; Varrica, A. ; Chiappa, E. ; Pilati, M. ; Carotti, A. ; Matteo, T. ; Prandstraller, D. ; Gargiulo, G. ; Giovanna Russo, M. ; Santoro, G. ; Caianiello, G. ; Spadoni, I. ; Murzi, B. ; Arcieri, L. ; Pozzi, M. ; Porcedda, G. ; Berggren, H. ; Carrel, T. ; Kadner, A. ; Çiçek, S. ; Zorman, Y. ; Fragata, J. ; Gordo, A. ; Hazekamp, M. ; Sojak, V. ; Hraska, V. ; Asfour, B. ; Maruszewski, B. ; Kozlowski, M. ; Metras, D. ; Pretre, R. ; Rubay, J. ; Sairanen, H. ; Sarris, G. ; Schreiber, C. ; Ono, M. ; Meyns, B. ; Van Den Bossche, K. ; Tlaskal, T. ; Lo Rito, M. ; Joon Yoo, S. ; Van Arsdell, G.S. ; Calderone, C. ; Iwamoto, Y. ; Leon-Wyss, J. ; Di Filippo, S. ; Leconte, C. ; Mulder, B.J. ; Ebels, T. ; Arrigoni, S. ; Valsangiacomo, E. ; Hitendu, D. ; Konstantinov, I.E. ; Gamillscheg, A. ; Gabriela, D. ; Herberg, U. ; Dulac, Y. ; Edmerger, J. ; Zarate Fuentes, A. ; Miguel Gil Jaurena, J. ; Bo, I. ; Ghez, O. ; Rigby, M.L. ; Bacha, E.A. ; Kalfa, D. ; Speggiorin, S. ; Bu'Lock, F. ; Al-Ahmadi, M. ; Di Salvo, G. ; Surmacz, R. ; Yemets, I.M. ; Mykychak, Y.B. ; Lugones, I. ; Cameron, D.E. ; Vricella, L.A. ; Troconis, C.J. ; Thiene, G. ; Angelini, A. / The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study. In: European Heart Journal. 2018 ; Vol. 39, No. 12. pp. 1002-1011.
@article{85cc0e73bc2b438d8d485627abfa0803,
title = "The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study",
abstract = "Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57{\%}) patients were treated surgically (STPs) and 206 (43{\%}) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88{\%} [85-92{\%} confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P <0.001) and pulmonary hypertension (P <0.001). Most patients were asymptomatic at last follow-up (279/451, 62{\%}); STPs were more frequently asymptomatic than CMPs (73{\%} vs. 47{\%}, P <0.001), with fewer cardiac [odds ratio (OR) 0.42, 95{\%} CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95{\%} CI 0.02-0.28). Many STPs (63/254, 25{\%}) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age. {\circledC} The Author 2017.",
keywords = "Congenital heart defect, Multi-centre study, Natural history, Surgery",
author = "V.L. Vida and A. Guariento and O. Milanesi and D. Gregori and G. Stellin and F. Zucchetta and L. Zanotto and M.A. Padalino and B. Castaldi and S. Bosiznik and R. Crepaz and J. Stuefer and {De Maria Garcia Gonzales}, F. and A.R. Castaneda and G. Crupi and G. Agnoletti and S. Bondanza and M. Marasini and L. Zannini and G. Butera and A. Frigiola and A. Varrica and E. Chiappa and M. Pilati and A. Carotti and T. Matteo and D. Prandstraller and G. Gargiulo and {Giovanna Russo}, M. and G. Santoro and G. Caianiello and I. Spadoni and B. Murzi and L. Arcieri and M. Pozzi and G. Porcedda and H. Berggren and T. Carrel and A. Kadner and S. {\cC}i{\cc}ek and Y. Zorman and J. Fragata and A. Gordo and M. Hazekamp and V. Sojak and V. Hraska and B. Asfour and B. Maruszewski and M. Kozlowski and D. Metras and R. Pretre and J. Rubay and H. Sairanen and G. Sarris and C. Schreiber and M. Ono and B. Meyns and {Van Den Bossche}, K. and T. Tlaskal and {Lo Rito}, M. and {Joon Yoo}, S. and {Van Arsdell}, G.S. and C. Calderone and Y. Iwamoto and J. Leon-Wyss and {Di Filippo}, S. and C. Leconte and B.J. Mulder and T. Ebels and S. Arrigoni and E. Valsangiacomo and D. Hitendu and I.E. Konstantinov and A. Gamillscheg and D. Gabriela and U. Herberg and Y. Dulac and J. Edmerger and {Zarate Fuentes}, A. and {Miguel Gil Jaurena}, J. and I. Bo and O. Ghez and M.L. Rigby and E.A. Bacha and D. Kalfa and S. Speggiorin and F. Bu'Lock and M. Al-Ahmadi and {Di Salvo}, G. and R. Surmacz and I.M. Yemets and Y.B. Mykychak and I. Lugones and D.E. Cameron and L.A. Vricella and C.J. Troconis and G. Thiene and A. Angelini",
note = "Cited By :2 Export Date: 22 June 2018 CODEN: EHJOD Correspondence Address: Vida, V.L.; Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Via Giustiniani 2, Italy; email: vladimiro.vida@unipd.it References: Vida, V.L., Padrini, M., Boccuzzo, G., Agnoletti, G., Bondanza, S., Butera, G., Chiappa, E., Milanesi, O., Natural history and clinical outcome of {"}uncorrected{"} scimitar syndrome patients: A multicenter study of the Italian society of pediatric cardiology (2013) Rev Esp Cardiol (Engl Ed), 66, pp. 556-560. , Italian Society of Pediatric Cardiology; Vida, V.L., Padalino, M.A., Boccuzzo, G., Tarja, E., Berggren, H., Carrel, T., Ci{\cc}ek, S., Stellin, G., Scimitar syndrome: A European Congenital Heart Surgeons Association (ECHSA) multicentric study (2010) Circulation, 122, pp. 1159-1166; Espinosa-Zavaleta, N., Jativa-Chavez, S., Munos-Castellanos, L., Zamora-Gonzales, C., Clinical and echocardiographic characteristics of scimitar syndrome (2006) Rev Esp Cardiol, 59, pp. 284-288; 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Vida, V.L., Speggiorin, S., Padalino, M.A., Crupi, G., Marcelletti, C., Zannini, L., Frigiola, A., Stellin, G., The scimitar syndrome: An Italian multicenter study (2009) Ann Thorac Surg, 88, pp. 440-444; Lugones, I., Garcia, R., A new surgical approach to scimitar syndrome (2014) Ann Thorac Surg, 97, pp. 353-355; Brown, J.W., Ruzmetov, M., Minnich, D.J., Vijay, P., Edwards, C.A., Uhlig, P.N., Fiore, A.C., Turrentine, M.W., Surgical management of scimitar syndrome: An alternative approach (2003) J Thorac Cardiovasc Surg, 125, pp. 238-245; Brink, J., Yong, M.S., D'Udekem, Y., Weintraub, R.G., Brizard, C.P., Konstantinov, I.E., Surgery for scimitar syndrome: The Melbourne experience (2015) Interact Cardiovasc Thorac Surg, 20, pp. 31-34; Mason, D.P., Mihaljevic, T., Mazzone, P.J., Murthy, S.C., Rice, T.W., Extrapleural pneumonectomy for scimitar syndrome (2006) J Thorac Cardiovasc Surg, 132, pp. 704-705; Kamiyama, M., Kamata, S., Usui, N., Scimitar syndrome treated with pneumonectomy: A case associated with bronchospastic attack (2004) Pediatr Surg Int, 20, pp. 65-66; 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year = "2018",
doi = "10.1093/eurheartj/ehx526",
language = "English",
volume = "39",
pages = "1002--1011",
journal = "European Heart Journal",
issn = "0195-668X",
publisher = "Oxford University Press",
number = "12",

}

Vida, VL, Guariento, A, Milanesi, O, Gregori, D, Stellin, G, Zucchetta, F, Zanotto, L, Padalino, MA, Castaldi, B, Bosiznik, S, Crepaz, R, Stuefer, J, De Maria Garcia Gonzales, F, Castaneda, AR, Crupi, G, Agnoletti, G, Bondanza, S, Marasini, M, Zannini, L, Butera, G, Frigiola, A, Varrica, A, Chiappa, E, Pilati, M, Carotti, A, Matteo, T, Prandstraller, D, Gargiulo, G, Giovanna Russo, M, Santoro, G, Caianiello, G, Spadoni, I, Murzi, B, Arcieri, L, Pozzi, M, Porcedda, G, Berggren, H, Carrel, T, Kadner, A, Çiçek, S, Zorman, Y, Fragata, J, Gordo, A, Hazekamp, M, Sojak, V, Hraska, V, Asfour, B, Maruszewski, B, Kozlowski, M, Metras, D, Pretre, R, Rubay, J, Sairanen, H, Sarris, G, Schreiber, C, Ono, M, Meyns, B, Van Den Bossche, K, Tlaskal, T, Lo Rito, M, Joon Yoo, S, Van Arsdell, GS, Calderone, C, Iwamoto, Y, Leon-Wyss, J, Di Filippo, S, Leconte, C, Mulder, BJ, Ebels, T, Arrigoni, S, Valsangiacomo, E, Hitendu, D, Konstantinov, IE, Gamillscheg, A, Gabriela, D, Herberg, U, Dulac, Y, Edmerger, J, Zarate Fuentes, A, Miguel Gil Jaurena, J, Bo, I, Ghez, O, Rigby, ML, Bacha, EA, Kalfa, D, Speggiorin, S, Bu'Lock, F, Al-Ahmadi, M, Di Salvo, G, Surmacz, R, Yemets, IM, Mykychak, YB, Lugones, I, Cameron, DE, Vricella, LA, Troconis, CJ, Thiene, G & Angelini, A 2018, 'The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study', European Heart Journal, vol. 39, no. 12, pp. 1002-1011. https://doi.org/10.1093/eurheartj/ehx526

The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study. / Vida, V.L.; Guariento, A.; Milanesi, O.; Gregori, D.; Stellin, G.; Zucchetta, F.; Zanotto, L.; Padalino, M.A.; Castaldi, B.; Bosiznik, S.; Crepaz, R.; Stuefer, J.; De Maria Garcia Gonzales, F.; Castaneda, A.R.; Crupi, G.; Agnoletti, G.; Bondanza, S.; Marasini, M.; Zannini, L.; Butera, G.; Frigiola, A.; Varrica, A.; Chiappa, E.; Pilati, M.; Carotti, A.; Matteo, T.; Prandstraller, D.; Gargiulo, G.; Giovanna Russo, M.; Santoro, G.; Caianiello, G.; Spadoni, I.; Murzi, B.; Arcieri, L.; Pozzi, M.; Porcedda, G.; Berggren, H.; Carrel, T.; Kadner, A.; Çiçek, S.; Zorman, Y.; Fragata, J.; Gordo, A.; Hazekamp, M.; Sojak, V.; Hraska, V.; Asfour, B.; Maruszewski, B.; Kozlowski, M.; Metras, D.; Pretre, R.; Rubay, J.; Sairanen, H.; Sarris, G.; Schreiber, C.; Ono, M.; Meyns, B.; Van Den Bossche, K.; Tlaskal, T.; Lo Rito, M.; Joon Yoo, S.; Van Arsdell, G.S.; Calderone, C.; Iwamoto, Y.; Leon-Wyss, J.; Di Filippo, S.; Leconte, C.; Mulder, B.J.; Ebels, T.; Arrigoni, S.; Valsangiacomo, E.; Hitendu, D.; Konstantinov, I.E.; Gamillscheg, A.; Gabriela, D.; Herberg, U.; Dulac, Y.; Edmerger, J.; Zarate Fuentes, A.; Miguel Gil Jaurena, J.; Bo, I.; Ghez, O.; Rigby, M.L.; Bacha, E.A.; Kalfa, D.; Speggiorin, S.; Bu'Lock, F.; Al-Ahmadi, M.; Di Salvo, G.; Surmacz, R.; Yemets, I.M.; Mykychak, Y.B.; Lugones, I.; Cameron, D.E.; Vricella, L.A.; Troconis, C.J.; Thiene, G.; Angelini, A.

In: European Heart Journal, Vol. 39, No. 12, 2018, p. 1002-1011.

Research output: Contribution to journalArticle

TY - JOUR

T1 - The natural history and surgical outcome of patients with scimitar syndrome: A multi-centre European study

AU - Vida, V.L.

AU - Guariento, A.

AU - Milanesi, O.

AU - Gregori, D.

AU - Stellin, G.

AU - Zucchetta, F.

AU - Zanotto, L.

AU - Padalino, M.A.

AU - Castaldi, B.

AU - Bosiznik, S.

AU - Crepaz, R.

AU - Stuefer, J.

AU - De Maria Garcia Gonzales, F.

AU - Castaneda, A.R.

AU - Crupi, G.

AU - Agnoletti, G.

AU - Bondanza, S.

AU - Marasini, M.

AU - Zannini, L.

AU - Butera, G.

AU - Frigiola, A.

AU - Varrica, A.

AU - Chiappa, E.

AU - Pilati, M.

AU - Carotti, A.

AU - Matteo, T.

AU - Prandstraller, D.

AU - Gargiulo, G.

AU - Giovanna Russo, M.

AU - Santoro, G.

AU - Caianiello, G.

AU - Spadoni, I.

AU - Murzi, B.

AU - Arcieri, L.

AU - Pozzi, M.

AU - Porcedda, G.

AU - Berggren, H.

AU - Carrel, T.

AU - Kadner, A.

AU - Çiçek, S.

AU - Zorman, Y.

AU - Fragata, J.

AU - Gordo, A.

AU - Hazekamp, M.

AU - Sojak, V.

AU - Hraska, V.

AU - Asfour, B.

AU - Maruszewski, B.

AU - Kozlowski, M.

AU - Metras, D.

AU - Pretre, R.

AU - Rubay, J.

AU - Sairanen, H.

AU - Sarris, G.

AU - Schreiber, C.

AU - Ono, M.

AU - Meyns, B.

AU - Van Den Bossche, K.

AU - Tlaskal, T.

AU - Lo Rito, M.

AU - Joon Yoo, S.

AU - Van Arsdell, G.S.

AU - Calderone, C.

AU - Iwamoto, Y.

AU - Leon-Wyss, J.

AU - Di Filippo, S.

AU - Leconte, C.

AU - Mulder, B.J.

AU - Ebels, T.

AU - Arrigoni, S.

AU - Valsangiacomo, E.

AU - Hitendu, D.

AU - Konstantinov, I.E.

AU - Gamillscheg, A.

AU - Gabriela, D.

AU - Herberg, U.

AU - Dulac, Y.

AU - Edmerger, J.

AU - Zarate Fuentes, A.

AU - Miguel Gil Jaurena, J.

AU - Bo, I.

AU - Ghez, O.

AU - Rigby, M.L.

AU - Bacha, E.A.

AU - Kalfa, D.

AU - Speggiorin, S.

AU - Bu'Lock, F.

AU - Al-Ahmadi, M.

AU - Di Salvo, G.

AU - Surmacz, R.

AU - Yemets, I.M.

AU - Mykychak, Y.B.

AU - Lugones, I.

AU - Cameron, D.E.

AU - Vricella, L.A.

AU - Troconis, C.J.

AU - Thiene, G.

AU - Angelini, A.

N1 - Cited By :2 Export Date: 22 June 2018 CODEN: EHJOD Correspondence Address: Vida, V.L.; Pediatric and Congenital Cardiac Surgery Unit, Department of Thoracic, Cardiac and Vascular Sciences, University of Padua, Via Giustiniani 2, Italy; email: vladimiro.vida@unipd.it References: Vida, V.L., Padrini, M., Boccuzzo, G., Agnoletti, G., Bondanza, S., Butera, G., Chiappa, E., Milanesi, O., Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: A multicenter study of the Italian society of pediatric cardiology (2013) Rev Esp Cardiol (Engl Ed), 66, pp. 556-560. , Italian Society of Pediatric Cardiology; Vida, V.L., Padalino, M.A., Boccuzzo, G., Tarja, E., Berggren, H., Carrel, T., Ciçek, S., Stellin, G., Scimitar syndrome: A European Congenital Heart Surgeons Association (ECHSA) multicentric study (2010) Circulation, 122, pp. 1159-1166; Espinosa-Zavaleta, N., Jativa-Chavez, S., Munos-Castellanos, L., Zamora-Gonzales, C., Clinical and echocardiographic characteristics of scimitar syndrome (2006) Rev Esp Cardiol, 59, pp. 284-288; 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Huddleston, C.B., Exil, V., Canter, C.E., Mendeloff, E.N., Scimitar syndrome presenting in infancy (1999) Ann Thorac Surg, 67, pp. 154-159. , discussion 160; Hoffman, J.I.E., Chapter 16: Scimitar syndrome (2009) The Natural and Unnatural History of Congenital Heart Disease, pp. 161-166. , Oxford, UK: Wiley-Blackwell; Galie, N., Humbert, M., Vachiery, J.-L., Gibbs, S., Lang, I., Torbicki, A., Simonneau, G., Zamorano, J.L., 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension (2016) Rev Esp Cardiol (Engl Ed), 69, p. 177; Barst, R.J., McGoon, M., Torbicki, A., Sitbon, O., Krowka, M.J., Olschewski, H., Gaine, S., Diagnosis and differential assessment of pulmonary arterial hypertension (2004) J Am Coll Cardiol, 43, pp. 40S-47S; Rubin, D.B., On principles for modeling propensity scores in medical research (2004) Pharmacoepidemiol Drug Saf, 13, pp. 855-857; Diamond, A., Sekhon, J.S., Genetic matching for estimating causal effects: A general multivariable matching method for achieving balance in observational studies (2013) Rev Econ and Stat, 95, pp. 932-945; Luo, Z., Gardiner, J.C., Bradley, C.J., Applying propensity score methods in medical research: Pitfalls and prospects (2010) Med Care Res Rev, 67, pp. 528-554; White, H., A heteroskedasticity-consistent covariance matrix estimator and a direct test for heteroskedasticity (1980) Econometrica, 48, pp. 817-838; Benjamini, Y., Yekutieli, D., The control of the false discovery rate in multiple testing under dependency (2001) Ann Stat, 29, pp. 1165-1188; (2015) R: A Language and Environment for Statistical Computing, , http://www.R-project.org, Vienna, Austria: R Foundation for Statistical Computing; (02 January 2017); Sekhon, J.S., Multivariable and propensity score matching software with automated balance optimization: The Matching Package for R (2011) J Stat Software, 42, pp. 1-52; Harrell, F.E., Jr., RMS: Regression Modelling Strategies, , http://biostat.mc.vanderbilt.edu/wiki/pub/Main/RmS/rms.pdf, (02 January 2017); Bojanowski, M., Edwards, R., (2016) Alluvial: R Package for Creating Alluvial Diagrams, , https://github.com/mbojan/alluvial, R package version: 0.1-2, (02 January 2017); 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PY - 2018

Y1 - 2018

N2 - Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P <0.001) and pulmonary hypertension (P <0.001). Most patients were asymptomatic at last follow-up (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P <0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02-0.28). Many STPs (63/254, 25%) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age. © The Author 2017.

AB - Aims Treatment decisions in patients with scimitar syndrome (SS) are often challenging, especially in patients with isolated SS who are often asymptomatic and who might be diagnosed accidentally. We queried a large multi-institutional registry of SS patients to evaluate the natural history of this condition and to determine the efficacy of surgical treatment in terms of survival and clinical status. Methods and results We collected data on 485 SS patients from 51 institutions; 279 (57%) patients were treated surgically (STPs) and 206 (43%) were clinically monitored (CMPs). Median age at last follow-up was 11.6 years (interquartile range 4-22 years). Overall survival probability at 30 years of age was 88% [85-92% confidence intervals (CI)] and was lower in patients with associated congenital heart disease (CHD) (P <0.001) and pulmonary hypertension (P <0.001). Most patients were asymptomatic at last follow-up (279/451, 62%); STPs were more frequently asymptomatic than CMPs (73% vs. 47%, P <0.001), with fewer cardiac [odds ratio (OR) 0.42, 95% CI 0.22-0.82] and respiratory symptoms (OR 0.08, 95% CI 0.02-0.28). Many STPs (63/254, 25%) had stenosis/occlusion of the scimitar drainage, and this was associated with a younger age at surgery (OR 0.4, CI 0.21-0.78). Conclusion Patients with SS have a high overall survival. Survival probability was lower in patients with associated CHDs and in patients with pulmonary hypertension. Surgical treatment of SS is beneficial in reducing symptoms, however, given the significant risk of post-operative scimitar drainage stenosis/occlusion, it should be tailored to a comprehensive haemodynamic evaluation and to the patient's age. © The Author 2017.

KW - Congenital heart defect

KW - Multi-centre study

KW - Natural history

KW - Surgery

U2 - 10.1093/eurheartj/ehx526

DO - 10.1093/eurheartj/ehx526

M3 - Article

VL - 39

SP - 1002

EP - 1011

JO - European Heart Journal

JF - European Heart Journal

SN - 0195-668X

IS - 12

ER -