Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: the 26-year Melbourne experience.

Autor:	Lee MG; Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., Brizard CP; Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., Galati JC; Clinical Epidemiology and Biostatistics Unit, Murdoch Childrens Research Institute; and the Department of Mathematics and Statistics, La Trobe University, Melbourne, Australia., Iyengar AJ; Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., Rakhra SS; Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., Konstantinov IE; Department of Cardiac Surgery, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., Pflaumer A; Department of Cardiology, The Royal Children's Hospital; the Department of Paediatrics, University of Melbourne; and the Murdoch Childrens Research Institute, Melbourne, Australia., d'Udekem Y; 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.
Jazyk:	angličtina
Zdroj:	The Journal of thoracic and cardiovascular surgery [J Thorac Cardiovasc Surg] 2014 Jul; Vol. 148 (1), pp. 194-201. Date of Electronic Publication: 2013 Sep 24.
DOI:	10.1016/j.jtcvs.2013.07.076
Abstrakt:	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. (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)
Databáze:	MEDLINE
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