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

2017; Oxford University Press; Volume: 39; Issue: 12 Linguagem: Inglês

10.1093/eurheartj/ehx526

1522-9645

Vladimiro L. Vida, Alvise Guariento, Ornella Milanesi, Darío Gregori, Giovanni Stellin, Fabio Zucchetta, Lorenza Zanotto, Massimo A. Padalino, Biagio Castaldi, Sasa Bosiznik, R Crepaz, Joseph Stuefer, Flor de Maria Garcia Gonzales, Aldo R. Castañeda, Giancarlo Crupi, Gabriella Agnoletti, Sara Bondanza, Maurizio Marasini, L. Zannini, Gianfranco Butera, Alessandro Frigiola, Alessandro Varrica, Enrico Chiappa, Mara Pilati, Adriano Carotti, Matteo Trezzi, Daniela Prandstraller, Gaetano Gargiulo, Maria Giovanna Russo, Giuseppe Santoro, Giuseppe Caianiello, Isabella Spadoni, Bruno Murzi, Luigi Arcieri, Marco Pozzi, Giulio Porcedda, Håkan Berggren, Thierry Carrel, Alexander Kadner, Sertaç Çïçek, Yılmaz Zorman, José Fragata, Andreia Gordo, Mark G. Hazekamp, Vladimír Soják, Viktor Hraška, Boulos Asfour, Bohdan Maruszewski, Michał Kozłowski, D Métras, René Prêtre, Jean Rubay, Heikki Sairanen, George E. Sarris, Christian Schreiber, Masamichi Ono, Bart Meyns, Klaartje Van den Bossche, T Tláskal, Mauro Lo Rito, Shi Joon Yoo, Glen S. Van Arsdell, Christopher Calderone, Yoichi Iwamoto, Juan León-Wyss, Sylvie Di Filippo, C Leconte, Barbara J.M. Mulder, Tjark Ebels, Sara C. Arrigoni, Emanuela Valsangiacomo, Hitendu Dave, Igor E. Konstantinov, Andreas Gamillscheg, Doros Gabriela, Ulrike Herberg, Yves Dulac, Julio Edmerger, Alberto Zarate Fuentes, Juan Miguel Gil Jaúrena, Ilaria Bo, Olivier Ghez, Micheal L Rigby, Emile Bacha, David Kalfa, Simone Speggiorin, Frances Bu’Lock, Mamdouh Al‐Ahmadi, Giovanni Di Salvo, Rafał Surmacz, Illya Yemets, Yaroslav Mykychak, Ignacio Lugones, Duke E. Cameron, Luca A. Vricella, Carlos J. Troconis, Gaetano Thiene, Annalisa Angelini, Lucia Zanotto,

Congenital Heart Disease Studies

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. 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). 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.