Palliation Outcomes of Neonates Born With Single-Ventricle Anomalies Associated With Aortic Arch Obstruction.
BACKGROUND
The two most common surgical strategies for the treatment of neonates born with single-ventricle anomalies associated with aortic arch obstruction are the Norwood operation and pulmonary artery banding plus coarctation repair (PAB+COA). We reviewed characteristics and outcomes of neonates who underwent those two surgical strategies at our institution.


METHODS
Between 2002 and 2012, 94 neonates with a single ventricle and aortic arch obstruction (excluding hypoplastic left heart syndrome) underwent Norwood (n = 65) or PAB+COA (n = 29). Outcomes were parametrically modeled, and risk factors associated with early and late death were analyzed.


RESULTS
Competing-risks analysis showed that, at 2 years after the operation, 24% of patients had died or received transplantation and 75% had undergone a Glenn shunt. At 5 years after the Glenn shunt, 10% of patients had died or received transplantation, 62% had undergone Fontan, and 28% were alive awaiting Fontan. Overall 8-year survival was 70%. Outcomes after Norwood included extracorporeal membrane oxygenation use in 9 (14%), unplanned reoperation in 13 (20%), hospital death in 10 (15%), and interstage death in 8 (12%), with 8-year survival of 66%. Outcomes after PAB+COA included extracorporeal membrane oxygenation use in 1 (3%), unplanned reoperation in 9 (30%), hospital death in 1 (3%), and interstage death in 3 (10%), with 8-year survival of 76%. There was an association trend between underlying anatomy and survival (hazard ratio [HR], 2.1; 95% confidence interval [CI], 0.9 to 4.7; p = 0.087). On multivariable analysis, factors associated with death were extracorporeal membrane oxygenation use (HR, 5.5; 95% CI, 1.9 to 15.9; p = 0.002), genetic syndromes/extracardiac anomalies (HR, 3.5; 95% CI, 1.5 to 8.2; p = 0.003), and weight of 2.5 kg or less (HR, 3.0; 95% CI, 1.3 to 7.2; p = 0.012).


CONCLUSIONS
Anatomic and patient characteristics influence palliation outcomes in neonates born with single-ventricle anomalies associated with aortic arch obstruction. Although the Norwood operation is applicable in most of these patients, the PAB+COA strategy is a valid alternative in well-selected patients.
Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Georgia. Electronic address: balsoufi@hotmail.com.
Bahaaldin
B
Alsoufi
Division of Cardiology, Emory University School of Medicine, Atlanta, Georgia.
Courtney
C
McCracken
Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Georgia.
Subhadra
S
Shashidharan
Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Georgia.
Brian
B
Kogon
Division of Cardiology, Emory University School of Medicine, Atlanta, Georgia.
William
W
Border
Division of Cardiothoracic Surgery, Emory University School of Medicine, Atlanta, Georgia.
Kirk
K
Kanter
D000015:Abnormalities, Multiple; D001015:Aortic Arch Syndromes / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005845:Georgia / Q000453:epidemiology; D017052:Hospital Mortality / Q000639:trends; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D058327:Norwood Procedures / Q000379:methods*; D010166:Palliative Care / Q000379:methods*; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome