CONGENITAL
HEART DISEASE
Determinants of Outcome in Hospitalized Infants with Congenital Heart Disease Gail D. Pearson, MD, ScD, Catherine A. Neil, MD, Timothy M. Beittel, and Langford Kidd, MD
To identify fbtermhumts of adverse outcome in this era of early, definitive treatment, retrospective data were analyzed for 1966 on infants aged 26 days and 29% (31 of 166) dbd dving the first year. Univariate analysis showed that complex disease (i.e., severe ventricular hypopksia, ventriwlar septal malatlgnment or outfbw atresia), extracardiac anomalies, early initial presentation, and prdonged stay in the intensive care unit were significantly assodated with infant death, whereas surgery was assodated with a significantly increased rate of survival. The findings for compbx disease and surgery persisted In multlpb b&tic regresslon analysis. It is conch&d that outcome In most infants with congenital cardiac defects ls now extremely favorable, and that major research and preventive efforts should focus on complex WngeLilital cardiac defects. (Am JCardld 1991;68:1665-1669)
nfants with symptomatic heart diseasearc being treated at younger agesthan ever before. At least 50% of definitive pediatric cardiac surgical proccdures are now being performed before I year of age, with sharp declinesin operative mortality since the early 1970s.’ The newborn with transposition of the great arteries that is repaired early and who is discharged 7 days) were significantly associated with a higher risk of mortality (chi-square 8.5, p = 0.004; chi-square 4.3, p = 0.04). The multiple logistic regressionmodel did not differ significantly from a “perfect” model, as measured by log likelihood (chi-square 86.8, degrees of difference 94, p = 0.69) and goodnessof fit (chi-square 89.4, degreesof difference 94, p = 0.62) statistics, and demonstrated a correct classification rate of 81%. Complex disease was significantly associated with death by 1 year of age (p = 0.003, odds ratio = 6, 95% confidence OUTCOME IN CONGENITAL HEART DISEASE 1057
interval 2 to 19), whereas surgery (L 1 procedure) was significantly associated with survival to 1 year (p = 0.02, odds ratio = 8, 95% confidence interval 1 to 42). Extracardiac anomalies, highly significant in the univariate analyses,only approachedsignificance in the multivariate model (p = 0.12, odds ratio 3, 95% confidenceinterval 0.8 to 8). The remaining variables (race, sex, birthweight, age at diagnosis,insurance type, medical admission, prolonged intensive care unit stay [>7 days], prolonged hospital stay [>28 days]) were not significantly associatedwith outcome.
Both the disproportionate occurrence and effect on mortality of extracardiac anomalies in children with cardiac diseaseare well known. The incidence of such anomalies in our study, 33%, is consistent with other studies.*J***l The combined effect of complex disease and major extracardiac anomalies has not been investigated systematically. In our study, infants with both conditions had a mortality rate of 58%, compared with a rate of just 6% for infants with neither condition. Determinants of mortality for infants between these extremes require further study. Like Fyler et al9 we found that surgery exerts a significant influence on mortality rates in a multivariate model. The positive relation of surgery to successful outcome is illustrated most clearly by the changes in mortality for tetralogy and transposition. Unlike Fyler et aL9 we found no significant association of birthweight with mortality. It is likely that improved management for low birthweight infants over the past 20 years is responsiblefor most of this difference. In addition, our study excluded preterm infants with the sole diagnosis of persistent ductus arteriosus, and only 2 study infants weighed
HEART DISEASE
Determinants of Outcome in Hospitalized Infants with Congenital Heart Disease Gail D. Pearson, MD, ScD, Catherine A. Neil, MD, Timothy M. Beittel, and Langford Kidd, MD
To identify fbtermhumts of adverse outcome in this era of early, definitive treatment, retrospective data were analyzed for 1966 on infants aged 26 days and 29% (31 of 166) dbd dving the first year. Univariate analysis showed that complex disease (i.e., severe ventricular hypopksia, ventriwlar septal malatlgnment or outfbw atresia), extracardiac anomalies, early initial presentation, and prdonged stay in the intensive care unit were significantly assodated with infant death, whereas surgery was assodated with a significantly increased rate of survival. The findings for compbx disease and surgery persisted In multlpb b&tic regresslon analysis. It is conch&d that outcome In most infants with congenital cardiac defects ls now extremely favorable, and that major research and preventive efforts should focus on complex WngeLilital cardiac defects. (Am JCardld 1991;68:1665-1669)
nfants with symptomatic heart diseasearc being treated at younger agesthan ever before. At least 50% of definitive pediatric cardiac surgical proccdures are now being performed before I year of age, with sharp declinesin operative mortality since the early 1970s.’ The newborn with transposition of the great arteries that is repaired early and who is discharged 7 days) were significantly associated with a higher risk of mortality (chi-square 8.5, p = 0.004; chi-square 4.3, p = 0.04). The multiple logistic regressionmodel did not differ significantly from a “perfect” model, as measured by log likelihood (chi-square 86.8, degrees of difference 94, p = 0.69) and goodnessof fit (chi-square 89.4, degreesof difference 94, p = 0.62) statistics, and demonstrated a correct classification rate of 81%. Complex disease was significantly associated with death by 1 year of age (p = 0.003, odds ratio = 6, 95% confidence OUTCOME IN CONGENITAL HEART DISEASE 1057
interval 2 to 19), whereas surgery (L 1 procedure) was significantly associated with survival to 1 year (p = 0.02, odds ratio = 8, 95% confidence interval 1 to 42). Extracardiac anomalies, highly significant in the univariate analyses,only approachedsignificance in the multivariate model (p = 0.12, odds ratio 3, 95% confidenceinterval 0.8 to 8). The remaining variables (race, sex, birthweight, age at diagnosis,insurance type, medical admission, prolonged intensive care unit stay [>7 days], prolonged hospital stay [>28 days]) were not significantly associatedwith outcome.
Both the disproportionate occurrence and effect on mortality of extracardiac anomalies in children with cardiac diseaseare well known. The incidence of such anomalies in our study, 33%, is consistent with other studies.*J***l The combined effect of complex disease and major extracardiac anomalies has not been investigated systematically. In our study, infants with both conditions had a mortality rate of 58%, compared with a rate of just 6% for infants with neither condition. Determinants of mortality for infants between these extremes require further study. Like Fyler et al9 we found that surgery exerts a significant influence on mortality rates in a multivariate model. The positive relation of surgery to successful outcome is illustrated most clearly by the changes in mortality for tetralogy and transposition. Unlike Fyler et aL9 we found no significant association of birthweight with mortality. It is likely that improved management for low birthweight infants over the past 20 years is responsiblefor most of this difference. In addition, our study excluded preterm infants with the sole diagnosis of persistent ductus arteriosus, and only 2 study infants weighed
