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

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 139; no. 2; pp. 359 - 365
• Main Authors: Furck, Anke Katharina, MD, Uebing, Anselm, MD, Hansen, Jan Hinnerk, Scheewe, Jens, MD, Jung, Olaf, MD, Fischer, Gunther, MD, Rickers, Carsten, MD, Holland-Letz, Tim, MSc, Kramer, Hans-Heiner, MD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-02-2010; AATS/WTSA; Elsevier
• Subjects: Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Cardiac Surgical Procedures; Cardiology. Vascular system; Cardiothoracic Surgery; Child; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Female; Fontan Procedure; Heart; Heart Arrest, Induced; Hospital Mortality; Humans; Hypoplastic Left Heart Syndrome - mortality; Hypoplastic Left Heart Syndrome - surgery; Male; Medical sciences; Pneumology; Postoperative Complications - epidemiology; Retrospective Studies; Risk Factors; Treatment Outcome; MA/AA; ASCP; mitral stenosis/aortic stenosis; OR; hypoplastic left heart syndrome; CI; 18; deep hypothermic circulatory arrest; mitral atresia/aortic stenosis; mitral stenosis/aortic atresia; MS/AA; odds ratio; HLHS; MA/AS; MS/AS; confidence interval; DHCA; mitral atresia/aortic atresia; 21; antegrade selective cerebral perfusion; Human; Center; Prognosis; Cardiovascular disease; Left Heart Hypoplasia; Patient; Operation; Congenital disease; Syndrome; Survey; Treatment; Heart disease; Surgery; Anesthesia; Circulatory system; Cardiology
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/j.jtcvs.2009.07.063

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.