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PEDIATRICS Vol. 110 No. 2 August 2002, pp. 292-298

Clinical Management of Infants With Hypoplastic Left Heart Syndrome in the United States, 1988–1997

Ruey-Kang R. Chang, MD, MPH*, Alex Y. Chen, MD{ddagger} and Thomas S. Klitzner, MD, PhD{ddagger}

* Division of Cardiology, Department of Pediatrics, Harbor-UCLA Medical Center, Torrance, California
{ddagger} Department of Pediatrics, UCLA School of Medicine, Los Angeles, California

Objectives. To evaluate changes in the clinical management of infants with hypoplastic left heart syndrome (HLHS) over a 10-year period.

Background. Orthotopic heart transplantation (OHT) and the Norwood procedure have emerged as the treatment options for HLHS over the last 2 decades.

Methods. We used 1988–1997 hospital discharge data from the National Inpatient Sample dataset. Patients ≤30 days of age with a principal diagnosis of HLHS were identified. Clinical management included the Norwood procedure, OHT, in-hospital death without surgery, discharge home without surgery, and transfer to another hospital. Multivariate logistic regression was used to evaluate variables associated with the choice of management.

Results. There were 1986 cases of HLHS with 812 in-hospital deaths, yielding a mortality rate 40.9%. The in-hospital mortality rate decreased from 54.4% in 1988 to 38.1% in 1997. The proportion of patients treated with the Norwood procedure increased from 8% in 1988 to 34% in 1997. The proportion of patients who died in the hospital without surgery decreased over time while the percentage discharged from the hospital without surgery or transferred to another hospital remained relatively unchanged. The in-hospital mortality rate was significantly lower in the OHT group compared with the Norwood group (26.2% vs 46.0%). We found no differences in gender, race, type of insurance, or home income between patients treated with the Norwood procedure compared with those who received comfort care. Patients from a later era, in the South, and in teaching hospitals were more likely to undergo the Norwood procedure.

Conclusions. Between 1988 and 1997, the proportion of infants with HLHS treated with the Norwood procedure increased while the use of comfort care decreased. Gender, race/ethnicity, type of medical insurance, and home income did not correlate with treatment choices.

Key Words: hypoplastic left heart syndrome • Norwood procedure • heart transplant • outcomes

Abbreviations: HLHS, hypoplastic left heart syndrome • OHT, orthotopic heart transplant • NIS, National Inpatient Sample • ICD-9CM, International Classification of Diseases, Ninth Revision, Clinical Modification • OR, odds ratio • CI, confidence interval

Received for publication Jan 28, 2002; Accepted Mar 28, 2002.




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