1. The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study
- Author
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Vida, Vladimiro L, Guariento, Alvise, Milanesi, Ornella, Gregori, Dario, Stellin, Giovanni, 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, Zanotto L., Vida, Vladimiro L, Guariento, Alvise, Milanesi, Ornella, Gregori, Dario, Stellin, Giovanni, 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, Zanotto L., Cardiology, APH - Personalized Medicine, APH - Aging & Later Life, ACS - Heart failure & arrhythmias, Cardiothoracic Surgery, 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, LUIGI PIERO, 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, 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, Zanotto, L., and University of Zurich
- Subjects
Male ,Pediatrics ,medicine.medical_specialty ,Time Factors ,Natural history ,610 Medicine & health ,Congenital heart defect ,Multi-centre study ,Surgery ,030204 cardiovascular system & hematology ,Asymptomatic ,2705 Cardiology and Cardiovascular Medicine ,03 medical and health sciences ,0302 clinical medicine ,Interquartile range ,Scimitar syndrome ,medicine ,Humans ,Registries ,Cardiac Surgical Procedures ,Retrospective Studies ,business.industry ,Incidence (epidemiology) ,Incidence ,Scimitar Syndrome ,Infant, Newborn ,Infant ,Retrospective cohort study ,Odds ratio ,medicine.disease ,Pulmonary hypertension ,Echocardiography, Doppler ,Europe ,Survival Rate ,Stenosis ,Treatment Outcome ,030228 respiratory system ,10036 Medical Clinic ,Pulmonary Veins ,Child, Preschool ,Female ,medicine.symptom ,Cardiology and Cardiovascular Medicine ,business ,Follow-Up Studies - 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
- Published
- 2018
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