The Scimitar Syndrome: An Italian Multicenter Study

Vladimiro L. Vida, Simone Speggiorin, Massimo A. Padalino, Giancarlo Crupi, Carlo Marcelletti, Lucio Zannini, Alessandro Frigiola, Alessandro Varrica, Duccio Di Carlo, Roberto Di Donato, Bruno Murzi, Massimo Bernabei, Giovanna Boccuzzo, Giovanni Stellin

Research output: Contribution to journalArticlepeer-review


Background: Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. Methods: From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. Results: Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). Conclusions: The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Original languageEnglish
Pages (from-to)440-444
Number of pages5
JournalAnnals of Thoracic Surgery
Issue number2
Publication statusPublished - Aug 2009

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Surgery
  • Pulmonary and Respiratory Medicine


Dive into the research topics of 'The Scimitar Syndrome: An Italian Multicenter Study'. Together they form a unique fingerprint.

Cite this