(Circulation. 1995;92:262-266.)
© 1995 American Heart Association, Inc.
Articles |
From the Departments of Cardiovascular Surgery (J.M.F., N.C., A.S., J.E.M., R.A.J.) and Cardiology (S.J.R.), Children's Hospital of Boston, and the Departments of Surgery and Pediatrics, Harvard Medical School, Boston, Mass.
Correspondence to Richard A. Jonas, MD, Department of Cardiovascular Surgery, Children's Hospital, 300 Longwood Ave, Boston, MA 02115.
Background We reviewed 212 consecutive patients who underwent stage I palliative surgery for hypoplastic left heart syndrome (HLHS) at our institution between January 1983 and June 1993.
Methods and Results Six surgeons participated in the care of these patients. Follow-up is 97% complete. Preoperative anatomic and physiological factors and procedural features of the stage I operation were analyzed for impact on stage I mortality, survival to stage II palliation, and actuarial survival. Hospital mortality was not significantly lower during the second half of the study period (P=.242). Operative mortality was 46.2%. Multivariate analysis revealed improved stage I operative survival in patients with mitral stenosis (MS) and aortic stenosis (AS; P=.006). Additional risk factors for stage I mortality were a lower immediately prestage I pH (P=.034) and weight <3 kg (P=.015). Overall first-year actuarial survival for MS/AS was 59%, and it was 33% for all others (P=.001). Among stage I survivors, patients with MS/AS were more likely to survive to stage II palliation (P=.031). Analysis of actuarial survival of stage I survivors showed that a smaller ascending aorta (P<.001), aortic atresia (P<.001), and mitral atresia (P=.002) were all risk factors for intermediate death.
Conclusions Preoperative anatomic and physiological state are predictors of stage I mortality. HLHS anatomic subtype also influences intermediate outcome, most notably prestage II attrition. These data may be useful in choosing initial management for patients with HLHS.
Key Words: surgery mortality hypoplastic left heart syndrome survival
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