Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: the 26-year Melbourne experience.
BACKGROUND
To review the long-term outcomes of patients born with single-ventricle physiology and aortic arch obstruction.


METHODS
Follow-up of 70 consecutive neonates undergoing single-ventricle palliation and arch repair, excluding hypoplastic left heart syndrome, between 1983 and 2008, was reviewed. Dominant arch anomalies were coarctation (n = 48), interrupted arch (n = 10), and hypoplastic arch alone (n = 12). Neonatal Damus procedure with arch repair and shunt became the dominant approach, being performed in 1 (10%) of 10 in 1983 to 1989, 9 (32%) of 28 in 1990 to 1999, and 23 (72%) of 32 in 2000 to 2008.


RESULTS
All patients underwent an initial procedure at a median of 6 days (range, 4-12 days): pulmonary artery banding and arch repair (n = 35); Damus, arch repair, and shunt (n = 33); and other (n = 2). Twenty-six patients died before Fontan completion. Of the 34 survivors of initial banding, 17 (50%) later required a Damus and 4 (12%) required subaortic stenosis relief. Forty patients underwent Fontan completion at a median age of 5 years (range, 4-7 years). After a mean of 5 ± 6 years after Fontan, there was 1 hospital death and 1 Fontan takedown. Overall survival was similar if patients initially underwent a Damus or pulmonary artery banding (P = .3). Overall survival at 10 years was 53% (95% confidence interval, 42%-67%).


CONCLUSIONS
Patients born with single-ventricle physiology and arch obstruction have a high risk of mortality in the first years of life. Their outcomes seem excellent once they reach Fontan status. It is likely that, in patients with single-ventricle and arch obstruction, strategies to avoid systemic outflow tract obstruction should be implemented in early life, and regular monitoring of blood pressure is warranted.
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Melissa G Y
MG
Lee
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Christian P
CP
Brizard
Clinical Epidemiology and Biostatistics Unit, Murdoch Childrens Research Institute; and the Department of Mathematics and Statistics, La Trobe University, Melbourne, Australia.
John C
JC
Galati
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Ajay J
AJ
Iyengar
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Sandeep S
SS
Rakhra
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Igor E
IE
Konstantinov
Department of Cardiology, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia.
Andreas
A
Pflaumer
Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au.
Yves
Y
d'Udekem
D001013:Aorta, Thoracic / Q000503:physiopathology / Q000601:surgery; D001017:Aortic Coarctation / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D001018:Aortic Diseases / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D001157:Arterial Occlusive Diseases / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003251:Constriction, Pathologic; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects / Q000401:mortality; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D006352:Heart Ventricles / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D017052:Hospital Mortality; D006801:Humans; D007226:Infant Mortality; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications / Q000401:mortality / Q000601:surgery; D016016:Proportional Hazards Models; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014739:Victoria