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J Thorac Cardiovasc Surg 2010;139:359-365
© 2010 The American Association for Thoracic Surgery


Congenital Heart Disease

Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: A 12-year single-center survey

Anke Katharina Furck, MDa,*, Anselm Uebing, MDa, Jan Hinnerk Hansena, Jens Scheewe, MDb, Olaf Jung, MDa, Gunther Fischer, MDa, Carsten Rickers, MDa, Tim Holland-Letz, MScc, Hans-Heiner Kramer, MDa

a Department of Pediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany
b Department of Cardiovascular Surgery, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany
c Department of Medical Informatics, Biometry and Epidemiology, Bochum, Germany

Received for publication December 16, 2008; revisions received June 23, 2009; accepted for publication July 23, 2009.

* Address for reprints: Anke Katharina Furck, MD, Department Pediatric Cardiology, University Hospital of Schleswig-Holstein, Campus Kiel, Arnold-Heller Strasse 3, House 9, 24105 Kiel, Germany. (Email: akfurck{at}aol.com).

Objective: Recent advances in perioperative care have led to a decrease in mortality of children with hypoplastic left heart syndrome undergoing the Norwood operation. This study aimed to evaluate the outcome of the Norwood operation in a single center over 12 years and to identify clinical and anatomic risk factors for adverse early and longer term outcome.

Methods: Full data on all 157 patients treated between 1996 and 2007 were analyzed.

Results: Thirty-day mortality of the Norwood operation decreased from 21% in the first 3 years to 2.5% in the last 3 years. The estimated exponentially weighted moving average of early mortality after 157 Norwood operations was 2.3%. Risk factors were an aberrant right subclavian artery, the use and duration of circulatory arrest, and the duration of total support time. The anatomic subgroup mitral stenosis/aortic atresia and female gender tended to show an increased early mortality. In the group of patients who required postoperative cardiopulmonary resuscitation, the ascending aorta was significantly smaller than in the remainder (3.03 ± 1.05 vs 3.63 ± 1.41 mm). Interstage mortality was 15% until the initiation of a home surveillance program in 2005, which has zeroed it so far. It was significantly higher in the mitral stenosis/aortic atresia subgroup and tended to be higher in patients who required cardiopulmonary resuscitation after the Norwood operation. The best actuarial survival was observed in the mitral atresia/aortic atresia subgroup.

Conclusion: The Norwood operation can now be performed with low mortality. Patients with mitral stenosis/aortic atresia still constitute the most challenging subgroup.



Abbreviations and Acronyms ASCP = antegrade selective cerebral perfusion; CI = confidence interval; DHCA = deep hypothermic circulatory arrest; HLHS = hypoplastic left heart syndrome; MA/AA = mitral atresia/aortic atresia; MA/AS = mitral atresia/aortic stenosis; MS/AA = mitral stenosis/aortic atresia; MS/AS = mitral stenosis/aortic stenosis; OR = odds ratio



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