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(Circulation. 2002;106:I-82.)
© 2002 American Heart Association, Inc.

Surgery for Congenital Heart Disease

Improved Survival of Patients Undergoing Palliation of Hypoplastic Left Heart Syndrome: Lessons Learned From 115 Consecutive Patients

James S. Tweddell, MD; George M. Hoffman, MD; Kathleen A. Mussatto, RN; Raymond T. Fedderly, MD; Stuart Berger, MD; Robert D. B. Jaquiss, MD; Nancy S. Ghanayem, MD; Stephanie J. Frisbee, MSc; S. Bert Litwin, MD

From the Children’s Hospital of Wisconsin and the Medical College of Wisconsin, Milwaukee, Wis.

Correspondence to James S. Tweddell, MD, Cardiothoracic Surgery, Children’s Hospital of Wisconsin, 9000 W. Wisconsin Ave., MS 715, Milwaukee, WI 53226. E-mail jtweddell{at}chw.org

Abstract

Background Outcome of stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS) has improved coincident with application of treatment strategies including continuous superior vena cava oximetry (SvO₂), phenoxybenzamine (POB), strategies to minimize the duration of deep hypothermic circulatory arrest (DHCA) and efforts to ameliorate the inflammatory response to cardiopulmonary bypass (CPB) using aprotinin and modified ultrafiltration.

Methods and Results Analysis of a consecutive series of 115 patients undergoing S1P was done to identify the risk factors for mortality and the impact of new treatment strategies. For the current era, July 1996 to October 2001, hospital survival was 93% (75/81) compared with 53% (18/34) for the time period, January 1992 to June 1996, P<0.001. Survival to stage 2 palliation (S2P) was also significantly improved in the current era, 81% (66/81) versus 44% (15/34), P<0.01. Anti-inflammatory treatment strategies demonstrated improved survival by univariate analysis (P<0.001). Multivariate analysis identified continuous SvO₂ monitoring as a factor favoring S1P survival (P=0.02) and use of POB as a factor favoring survival to S2P (P=0.003). In the current era shorter duration of DHCA was associated with improved survival to S2P (P=0.02).

Conclusions–Improved survival following S1P can be achieved with strategies that allow for early identification of decreased systemic output and the use of afterload reduction to stabilize systemic vascular resistance and therefore the pulmonary to systemic flow ratio. Strategies to ameliorate the inflammatory response to CPB may decrease the degree and duration of postoperative support. Strategies to minimize duration of DHCA may improve intermediate survival and merit additional studies.

Key Words: heart defects, congenital • circulatory control • cardiac output • autonomic nervous system