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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.status.
The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study / 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.. - In: EUROPEAN HEART JOURNAL. - ISSN 0195-668X. - STAMPA. - 39:12(2018), pp. 1002-1011. [10.1093/eurheartj/ehx526]
The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study
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.status.
The natural history and surgical outcome of patients with scimitar syndrome: a multi-centre European study / 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.. - In: EUROPEAN HEART JOURNAL. - ISSN 0195-668X. - STAMPA. - 39:12(2018), pp. 1002-1011. [10.1093/eurheartj/ehx526]
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.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/664043
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simulazione ASN
Il report seguente simula gli indicatori relativi alla propria produzione scientifica in relazione alle soglie ASN 2023-2025 del proprio SC/SSD. Si ricorda che il superamento dei valori soglia (almeno 2 su 3) è requisito necessario ma non sufficiente al conseguimento dell'abilitazione. La simulazione si basa sui dati IRIS e sugli indicatori bibliometrici alla data indicata e non tiene conto di eventuali periodi di congedo obbligatorio, che in sede di domanda ASN danno diritto a incrementi percentuali dei valori. La simulazione può differire dall'esito di un’eventuale domanda ASN sia per errori di catalogazione e/o dati mancanti in IRIS, sia per la variabilità dei dati bibliometrici nel tempo. Si consideri che Anvur calcola i valori degli indicatori all'ultima data utile per la presentazione delle domande.
La presente simulazione è stata realizzata sulla base delle specifiche raccolte sul tavolo ER del Focus Group IRIS coordinato dall’Università di Modena e Reggio Emilia e delle regole riportate nel DM 589/2018 e allegata Tabella A. Cineca, l’Università di Modena e Reggio Emilia e il Focus Group IRIS non si assumono alcuna responsabilità in merito all’uso che il diretto interessato o terzi faranno della simulazione. Si specifica inoltre che la simulazione contiene calcoli effettuati con dati e algoritmi di pubblico dominio e deve quindi essere considerata come un mero ausilio al calcolo svolgibile manualmente o con strumenti equivalenti.