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J Thorac Cardiovasc Surg 2003;125:1070-1082
© 2003 The American Association for Thoracic Surgery

Surgery for Congenital Heart Disease

Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia

From the Division of Cardiovascular Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada^a; the Department of Surgery, Wake Forest University School of Medicine, Winston-Salem, NC^b; the Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada^c; the Division of Cardiovascular Surgery, Montreal Children's Hospital, Montreal, Quebec, Canada^d; the Division of Cardiothoracic Surgery, St Christopher's Hospital for Children, Philadelphia, Pa^e; the Section of Cardiac Surgery, Children's Mercy Hospital, Kansas City, Mo^f; the Section of Cardiac Surgery, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, Mich^g; the Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, Philadelphia, Pa^h; the Department of Thoracic and Cardiovascular Surgery, Cleveland Clinic Foundation, Cleveland, Ohioⁱ; and the Department of Cardiovascular Surgery, University of Toronto, Toronto, Ontario, Canada.^j

The Congenital Heart Surgeons Society Data Center is supported by the member surgeons and the Hospital for Sick Children (Toronto). Dr Ashburn was supported by the Bradshaw Fellowship in Surgical Research from the Department of Surgery at Wake Forest University School of Medicine.

Received for publication May 29, 2002; revisions requested July 12, 2002; revisions received Sept 17, 2002. Accepted for publication Sept 24, 2002. Address for reprints: William G. Williams, MD, Hospital for Sick Children, Division of Cardiovascular Surgery, 555 University Ave, Toronto, Ontario M5G 1X8, Canada (E-mail: bill.williams{at}sickkids.ca).

Objective: This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation.
Methods: A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates. Competing risks analyses were constructed for outcomes after Norwood operation and after cavopulmonary shunt. Incremental risk factors for outcome events were sought.
Results: Overall survivals after the Norwood operation were 72%, 60%, and 54% at 1 month, 1 year, and 5 years, respectively. According to competing risks analysis, 97% of neonates reached a subsequent transition state by 18 months after Norwood operation, consisting of death (37%), cavopulmonary shunt (58%), or other state (2%, cardiac transplantation, biventricular repair, or Fontan operation). Risk factors for death occurring before subsequent transition included patient-specific variables (lower birth weight, smaller ascending aorta, older age at Norwood operation), institutional variables (institutions enrolling 10 neonates, two institutions enrolling 40 neonates), and procedural variables (shunt originating from aorta, longer circulatory arrest time, and management of the ascending aorta). Of neonates undergoing cavopulmonary shunt, 91% had reached a subsequent transition state by 6 years after cavopulmonary shunt, consisting of Fontan operation (79%), death (9%), or cardiac transplantation (3%). Risk factors for death occurring before subsequent transition included younger age at cavopulmonary shunt and need for right atrioventricular valve repair.
Conclusions: Competing risks analysis defines the prevalence of the various outcomes after Norwood operation and predicts improved outcomes with successful modification of controllable risk factors.