Ten-year institutional experience with palliative surgery for hypoplastic left heart syndrome. Risk factors related to stage I mortality.
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.


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 pre-stage 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 pre-stage II attrition. These data may be useful in choosing initial management for patients with HLHS.
Forbess
J M
JM
Department of Cardiovascular Surgery, Children's Hospital of Boston, MA 02115, USA.
Cook
N
N
Roth
S J
SJ
Serraf
A
A
Mayer
J E
JE
Jonas
R A
RA
D000704:Analysis of Variance; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality* / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome