Clinical outcomes and secondary diagnoses for infants born with hypoplastic left heart syndrome.

Objective: To explore clinical outcomes and secondary diagnoses present at discharge for infants born with hypoplastic left heart syndrome (HLHS), from a national perspective.

Methods: We examined hospitalizations for infants < or =30 days of age who were born with HLHS, using hospital discharge data from the 1997 Kids Inpatient Database. To explore treatment choices, clinical outcomes, and resource use, we used International Classification of Diseases, 9th Revision, Clinical Modification diagnostic and procedure codes to classify discharges according to type of surgical intervention versus no surgical intervention. To investigate outcomes in more detail, we identified secondary diagnoses noted at discharge, using International Classification of Diseases, 9th Revision, Clinical Modification codes, and stratified results according to type of surgical intervention.

Results: Of a total of 550 patients with HLHS, 234 underwent the Norwood procedure, 17 underwent orthotopic heart transplantation, and 106 died in the hospital with no reported surgical intervention. Although we found no demographic variables to be significantly associated with the type of treatment received, discharged patients who died without surgical intervention were significantly more likely to have received care in hospitals identified as small (odds ratio [OR]: 1.5; 95% confidence interval [CI]: 1.03-3.1) or not children's hospitals (OR: 2.02; 95% CI: 1.13-3.6). Secondary diagnoses of cardiac arrest (OR: 2.0; 95% CI: 1.1-3.4) and seizures (OR: 2.6; 95% CI: 1.2-5.5) occurred more frequently in orthotopic heart transplantation cases than in Norwood procedure cases.

Conclusions: These data from a national perspective reflect outcomes of infants with HLHS during a time when rates of initial survival after surgical intervention were considered to be improved. These findings may be useful to clinicians when they are considering and recommending initial medical and surgical strategies currently being proposed for the treatment of HLHS.