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

Scimitar Syndrome Study Group

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.

Original languageEnglish
Pages (from-to)1002-1011
Number of pages10
JournalEuropean Heart Journal
Volume39
Issue number12
DOIs
Publication statusPublished - Mar 21 2018

Fingerprint

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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

The natural history and surgical outcome of patients with scimitar syndrome : A multi-centre European study. / Scimitar Syndrome Study Group.

In: European Heart Journal, Vol. 39, No. 12, 21.03.2018, p. 1002-1011.

Research output: Contribution to journalArticle

@article{e9278b841791441497d4894b6ea25285,
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.",
keywords = "Congenital heart defect, Multi-centre study, Natural history, Surgery",
author = "{Scimitar Syndrome Study Group} and Vida, {Vladimiro L.} and Alvise Guariento and Ornella Milanesi and Dario Gregori and Giovanni Stellin and Fabio Zucchetta and Lorenza Zanotto and Padalino, {Massimo A.} and Biagio Castaldi and Sasa Bosiznik and Roberto Crepaz and Joseph Stuefer and {De Maria Garcia Gonzales}, Flor and Castaneda, {Aldo R.} and Giancarlo Crupi and Gabriella Agnoletti and Sara Bondanza and Maurizio Marasini and Lucio Zannini and Gianfranco Butera and Alessandro Frigiola and Alessandro Varrica and Enrico Chiappa and Mara Pilati and Adriano Carotti and Trezzi Matteo and Daniela Prandstraller and Gaetano Gargiulo and {Giovanna Russo}, Maria and Giuseppe Santoro and Giuseppe Caianiello and Isabella Spadoni and Bruno Murzi and Luigi Arcieri and Marco Pozzi and Giulio Porcedda and Hakan Berggren and Thierry Carrel and Alexander Kadner and Serta{\cc} {\cC}i{\cc}ek and Yilmaz Zorman and Jos{\'e} Fragata and Andreia Gordo and Mark Hazekamp and Vladimir Sojak and Viktor Hraska and Boulos Asfour and Bohdan Maruszewski and Michal Kozlowski and {Lo Rito}, Mauro",
year = "2018",
month = "3",
day = "21",
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",

}

TY - JOUR

T1 - The natural history and surgical outcome of patients with scimitar syndrome

T2 - A multi-centre European study

AU - Scimitar Syndrome Study Group

AU - Vida, Vladimiro L.

AU - Guariento, Alvise

AU - Milanesi, Ornella

AU - Gregori, Dario

AU - Stellin, Giovanni

AU - Zucchetta, Fabio

AU - Zanotto, Lorenza

AU - Padalino, Massimo A.

AU - Castaldi, Biagio

AU - Bosiznik, Sasa

AU - Crepaz, Roberto

AU - Stuefer, Joseph

AU - De Maria Garcia Gonzales, Flor

AU - Castaneda, Aldo R.

AU - Crupi, Giancarlo

AU - Agnoletti, Gabriella

AU - Bondanza, Sara

AU - Marasini, Maurizio

AU - Zannini, Lucio

AU - Butera, Gianfranco

AU - Frigiola, Alessandro

AU - Varrica, Alessandro

AU - Chiappa, Enrico

AU - Pilati, Mara

AU - Carotti, Adriano

AU - Matteo, Trezzi

AU - Prandstraller, Daniela

AU - Gargiulo, Gaetano

AU - Giovanna Russo, Maria

AU - Santoro, Giuseppe

AU - Caianiello, Giuseppe

AU - Spadoni, Isabella

AU - Murzi, Bruno

AU - Arcieri, Luigi

AU - Pozzi, Marco

AU - Porcedda, Giulio

AU - Berggren, Hakan

AU - Carrel, Thierry

AU - Kadner, Alexander

AU - Çiçek, Sertaç

AU - Zorman, Yilmaz

AU - Fragata, José

AU - Gordo, Andreia

AU - Hazekamp, Mark

AU - Sojak, Vladimir

AU - Hraska, Viktor

AU - Asfour, Boulos

AU - Maruszewski, Bohdan

AU - Kozlowski, Michal

AU - Lo Rito, Mauro

PY - 2018/3/21

Y1 - 2018/3/21

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.

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.

KW - Congenital heart defect

KW - Multi-centre study

KW - Natural history

KW - Surgery

UR - http://www.scopus.com/inward/record.url?scp=85044650013&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85044650013&partnerID=8YFLogxK

U2 - 10.1093/eurheartj/ehx526

DO - 10.1093/eurheartj/ehx526

M3 - Article

C2 - 29029134

AN - SCOPUS:85044650013

VL - 39

SP - 1002

EP - 1011

JO - European Heart Journal

JF - European Heart Journal

SN - 0195-668X

IS - 12

ER -