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Original Articles: General Thoracic

The Scimitar Syndrome: An Italian Multicenter Study

^a Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Padua, Italy
^b Ospedali Riuniti di Bergamo, Centro Diagnosi e Trattamento delle Cardiopatie Congenite, Bergamo, Italy
^c Unità Operativa Cardiochirurgia Pediatrica, ARNAS Ospedale Civico e "Benfratelli," Palermo, Italy
^d Cardiochirurgia Pediatrica, IRCS Gaslini, Genova, Italy
^e Policlinico San Donato Milanese, Divisione di Cardiochirurgia, San Donato Milanese, Italy
^f Ospedale Pediatrico Bambino Gesù, Servizio di Cardiochirurgia Pediatrica, Rome, Italy
^g Unità Operativa di Cardiochirurgia Pediatrica, Ospedale "Pasquinucci," Massa, Italy
^h Department of Statistics, University of Padua, Padua, Italy

Accepted for publication April 24, 2009.

^_* Address correspondence to Dr Stellin, Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Via Giustiniani, 2, Padua, 35100, Italy (Email: giovanni.stellin{at}unipd.it).

Presented at the Poster Session of the Forty-fifth Annual Meeting of The Society of Thoracic Surgeons, San Francisco, CA, Jan 26–28, 2009.

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.