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(Circulation. 2008;118:1410-1418.)
© 2008 American Heart Association, Inc.

Congenital Heart Disease

Two-Year Survival and Mental and Psychomotor Outcomes After the Norwood Procedure

An Analysis of the Modified Blalock-Taussig Shunt and Right Ventricle–to–Pulmonary Artery Shunt Surgical Eras

Joseph Atallah, MD; Irina A. Dinu, PhD; Ari R. Joffe, MD; Charlene M.T. Robertson, MD; Reg S. Sauve, MD; John D. Dyck, MD; David B. Ross, MD; Ivan M. Rebeyka, MD; the Western Canadian Complex Pediatric Therapies Follow-Up Group

From the Department of Pediatrics (J.A., A.R.J., C.M.T.R., J.D.D., I.M.R.), School of Public Health (I.A.D.), and Department of Surgery (D.B.R., I.M.R.), University of Alberta, Edmonton, Alberta, Canada; Pediatric Rehabilitation Outcomes Evaluation and Research Unit (C.M.T.R.), Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada; and Department of Pediatrics (R.S.S.), University of Calgary, Calgary, Alberta, Canada.

Correspondence to Ari Joffe, MD, FRCP(C), Department of Pediatrics, 3A3.07 Stollery Children’s Hospital, 8440 112 St, Edmonton, Alberta, Canada T6G 2B7. E-mail ajoffe{at}cha.ab.ca

Received September 24, 2007; accepted July 25, 2008.

Background— The Norwood procedure for stage 1 palliation of hypoplastic left heart syndrome is performed with either the modified Blalock-Taussig (MBTS) or the right ventricle–to–pulmonary artery (RVPA) shunt. In our institution, surgical practice changed from use of the MBTS to use of the RVPA shunt in 2002. We analyzed survival and mental and psychomotor outcomes of the 2 consecutive surgical eras.

Methods and Results— Between September 1996 and July 2005, 94 neonates with hypoplastic left heart syndrome underwent the Norwood procedure. Patients were recruited as neonates and followed up prospectively. Health, mental, and psychomotor outcomes (Bayley Scales of Infant Development-II) were assessed at 2 years. The study subjects were from the Norwood-MBTS era (n=62; 1996 to 2002) or the Norwood-RVPA era (n=32; 2002 to 2005). In the MBTS era, early and 2-year mortality rates were 23% (14/62) and 52% (32/62); the mean (SD) mental and psychomotor developmental indices were 79 (18) and 67 (19). In the RVPA era, early and 2-year mortality rates were 6% (2/32) and 19% (6/32); the mean (SD) mental and psychomotor developmental indices were 85 (18) and 78 (18). The 2-year mortality rate (P=0.002) and the psychomotor developmental index (P=0.029) were improved in the more recent surgical era. On multivariable Cox regression analysis, postoperative highest serum lactate independently predicted 2-year mortality in the MBTS and RVPA eras.

Conclusions— Analysis of 2 consecutive surgical eras of hypoplastic left heart syndrome patients undergoing the Norwood procedure showed a significant improvement in 2-year survival and psychomotor development in the more recent era. Adverse neurodevelopmental outcome in this patient population remains a concern.

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Circulation 2008 118: 1403-1404. [Extract] [Full Text]