Associated factors and trends in outcomes of interrupted aortic arch.
BACKGROUND
Interrupted aortic arch (IAA) continues to be associated with important mortality, both before and immediately after repair, with ongoing morbidity during follow-up. We sought to determine trends in presentation, management, outcomes and associated factors.


METHODS
We reviewed all consecutive patients (n = 119) presenting from 1975 to 1999, and data were collected regarding demographics, anatomy, management and outcomes.


RESULTS
Significant trends over time for patients born in three consecutive periods (1975 to 1984, 1985 to 1993, and 1994 to 1999) demonstrated a smaller proportion of patients with presentation with circulatory collapse (65%, 51%, and 25%, respectively), greater use of prostaglandins (72%, 90%, 100%), fewer deaths without IAA repair (49%, 15%, 13%) and greater use of one-stage repair (68%, 75%, 100%). Independent risk factors for death without IAA repair (p < 0.001) included absence of ventricular septal defect, and the presence of noncardiac anomaly, complex cardiac anomaly, episode of acidosis and earlier birth cohort. Overall survival after repair was 50% at age 1 month, 35% at 1 year, and 34% at 5 years. Early and constant-hazard phases were noted, with incremental risk factors for early phase mortality being cyanosis at presentation, presence of truncus arteriosus or aortic stenosis, an episode of circulatory collapse before repair, earlier date of repair, and lower weight at repair. Greatest survival occurred in those patients with uncomplicated IAA who had repair since 1993 (5 year survival, 83%). Freedom from reintervention for arch obstruction was 60% at 5 years.


CONCLUSIONS
While improving, outcomes of IAA remain of concern, especially in patients with associated lesions.
Oosterhof
Thomas
T
Department of Pediatrics, Division of Cardiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada.
Azakie
Anthony
A
Freedom
Robert M
RM
Williams
William G
WG
McCrindle
Brian W
BW
D000015:Abnormalities, Multiple / Q000453:epidemiology; D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000601:surgery; D001015:Aortic Arch Syndromes / Q000150:complications / Q000453:epidemiology / Q000601:surgery* / Q000628:therapy; D002404:Catheterization; D003490:Cyanosis / Q000209:etiology; D004374:Ductus Arteriosus, Patent / Q000150:complications; D005260:Female; D006330:Heart Defects, Congenital / Q000188:drug therapy / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015993:Life Tables; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology; D011453:Prostaglandins / Q000627:therapeutic use; D012189:Retrospective Studies; D012769:Shock / Q000209:etiology; D016019:Survival Analysis; D016896:Treatment Outcome; D014339:Truncus Arteriosus, Persistent / Q000150:complications / Q000601:surgery; D014694:Ventricular Outflow Obstruction / Q000150:complications / Q000601:surgery
D011453:Prostaglandins