The scimitar syndrome: an Italian multicenter study.
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.
Vida
Vladimiro L
VL
Pediatric and Congenital Cardiac Surgery Unit, University of Padua, Padua, Italy.
Speggiorin
Simone
S
Padalino
Massimo A
MA
Crupi
Giancarlo
G
Marcelletti
Carlo
C
Zannini
Lucio
L
Frigiola
Alessandro
A
Varrica
Alessandro
A
Di Carlo
Duccio
D
Di Donato
Roberto
R
Murzi
Bruno
B
Bernabei
Massimo
M
Boccuzzo
Giovanna
G
Stellin
Giovanni
G
D000293:Adolescent; D000328:Adult; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007558:Italy; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000453:epidemiology; D012587:Scimitar Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult