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J Thorac Cardiovasc Surg 2005;129:343-350
© 2005 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Risk factors associated with mortality and interventions in 472 neonates with interrupted aortic arch: A Congenital Heart Surgeons Society study

^a Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
^b Division of Cardiovascular Surgery, Montreal Children's Hospital, Montreal, Quebec, Canada
^c Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
^d Division of Cardiothoracic Surgery, DeVos Children's Hospital, Grand Rapids, Mich
^e Division of Cardiothoracic Surgery, St Christopher's Hospital for Children, Philadelphia, Pa
^f Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic Foundation, Cleveland, Ohio

Read at the Eighty-fourth Annual Meeting of The American Association for Thoracic Surgery, Toronto, Ontario, Canada, April 25-28, 2004.

Received for publication April 23, 2004; revisions received September 4, 2004; accepted for publication October 1, 2004.

^* Address for reprints: William G. Williams, MD, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario, Canada M5G 1X8 (E-mail: bill.williams{at}sickkids.ca).

OBJECTIVE: We sought to determine the prevalence of outcomes and associated patient and management factors for neonates with interrupted aortic arch.

METHODS: From 1987 to 1997, a total of 472 neonates were enrolled prospectively from 33 institutions. Competing risks methodology was used to determine simultaneous risk and associated incremental risk factors for death, initial and subsequent left ventricular outflow tract procedures, and arch reinterventions.

RESULTS: Overall survival was 59% at 16 years after study entry but improved with successive birth cohort. In general, risk factors for death in each of the competing risks analyses included lower birth weight, younger age at study entry, type B interrupted aortic arch, and major associated cardiac anomalies. Of 453 patients who had interrupted aortic arch repair, after 16 years 33% had died and 28% had undergone an arch reintervention. Reintervention was more likely for those who had truncus arteriosus repair, interrupted aortic arch repair by a method other than direct anastomosis with patch augmentation, and the use of polytetrafluoroethylene as either an interposition graft or a patch. From study entry, competing risks after 16 years showed that 28% had died and 34% had undergone an initial left ventricular outflow tract procedure. Initial left ventricular outflow tract procedure was more likely for those with single ventricle, type B interrupted aortic arch, bicuspid aortic valve, or anomalous right subclavian artery. Among those who had undergone an initial left ventricular outflow tract procedure, after 16 years 37% had died and 28% had undergone a second procedure.

CONCLUSION: Anatomic features affect mortality and initial left ventricular outflow tract procedures, whereas characteristics of the arch repair affect arch reintervention.

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