Abstract
| Original language | English |
|---|---|
| Pages (from-to) | 1002-1011 |
| Number of pages | 10 |
| Journal | European Heart Journal |
| Volume | 39 |
| Issue number | 12 |
| DOIs | |
| Publication status | Published - 2018 |
Keywords
- Congenital heart defect
- Multi-centre study
- Natural history
- Surgery
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In: European Heart Journal, Vol. 39, No. 12, 2018, p. 1002-1011.
Research output: Contribution to journal › Article › peer-review
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: [email protected] 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; Schramel, F.M., Westermann, C.J., Knaepen, P.J., Vanden Bosch, J.M., The scimitar syndrome: Clinical spectrum and surgical treatment (1995) Eur Respir J, 8, pp. 196-201; Snellen, H.A., Vanngen, H.C., Hoefsmit, E.C., Patterns of anomalous pulmonary venous drainage (1968) Circulation, 38, pp. 45-63; Gao, Y.A., Burrows, P.E., Benson, L.N., Rabinovitch, M., Freedom, R.M., Scimitar syndrome in infancy (1993) J Am Coll Cardiol, 22, pp. 873-882; Dupuis, C., Charaf, L.A., Breviere, G.M., Abou, P., Infantile form of the scimitar syndrome with pulmonary hypertension (1993) Am J Cardiol, 71, pp. 1326-1330; Dupuis, C., Charaf, L.A., Breviere, G.M., Abou, P., Remy-Jardin, M., Helmius, G., The "adult" form of the scimitar syndrome (1992) Am J Cardiol, 70, pp. 502-507; Tjang, Y.S., Blanz, U., Kirana, S., Korfer, R., Scimitar syndrome presenting in adults (2008) J Card Surg, 23, pp. 71-72; Canter, C.E., Martin, T.C., Spray, T.L., Weldon, C.S., Strauss, A.W., Scimitar syndrome in childhood (1986) Am J Cardiol, 58, pp. 652-654; 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); 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; Uthaman, B., Abushaban, L., Al-Qbandi, M., Rathinasamy, J., The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy (2008) Catheter Cardiovasc Interv, 71, pp. 671-678
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
SN - 0195-668X
VL - 39
SP - 1002
EP - 1011
JO - European Heart Journal
JF - European Heart Journal
IS - 12
ER -