Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome

Abstract

ABSTRACT. 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 <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. H ypoplastic left heart syndrome (HLHS) remains one of the most complex congenital heart defects to treat, both medically and surgically. Despite advances in treatment, HLHS continues to have the highest mortality rate, of all congenital heart defects, for infants Ͻ1 year of age. 1 HLHS is a combination of congenital cardiac anomalies involving hypoplasia of the ascending aorta, aortic valve atresia or stenosis, a small or absent left ventricle, and mitral atresia or hypoplasia. 2 Before 1980, HLHS was considered universally fatal in the newborn period, leaving the health care team with the sole option of providing compassionate care. [2][3][4][5][6] Currently, most infants diagnosed as having HLHS undergo a series of surgical palliations; a small percentage undergo orthotopic heart transplantation (OHT) as an initial surgical approach. [7][8][9][10][11][12][13][14][15][16][17][18] As the rates of death after surgical intervention for HLHS have decreased, morbidities resulting from both surgical palliation and transpla...

Discharge Disposition of Adolescents Admitted to Medical Hospitals After Attempting Suicide

“…The sample is drawn from a federally funded hospital discharge data set, which has previously been used to describe hospitalized pediatric patients and to identify variations in their care. 18,[20][21][22] Our objective is to describe the characteristics of hospital admissions of adolescents who attempted suicide and the patterns of discharge disposition, and specifically to test the hypothesis that disposition varies as a function of hospital type and geographic region.…”

Waiting Before Birth: Outcomes After Fetal Listing for Heart Transplantation

“…Potentially lethal congenital lesions with a high risk for mortality may be detected as early as 16 weeks gestation (1)(2)(3)(4). Routine prenatal screening has led to a reduction in the frequency of live births of infants with significant congenital defects such as hypoplastic left heart syndrome (HLHS), partly as a result of planned termination of pregnancy (5). However, alternatives to pregnancy termination, including neonatal surgical palliation or heart transplantation, (2) continue to offer improving outcomes.…”

Hypoplastic Left Heart Syndrome: Parent Support for Early Decision Making

“…A recent study of national data of 550 children with HLHS noted a 32% mortality rate for the Norwood procedure (Connor, Arons, Figueroa, & Geddie, 2004). Another study reported a much lower mortality rate of 8% (Sano, Huang, Kasahara, Yoshizumi, Kotani, & Ishino, 2009).…”

“…Mortality rates for the Glenn and Fontan are much lower, at 2 to 5.4% and less than 5% respectively (Connor, et al, 2004). Although surgical survival rates have improved greatly in the past 20 years, there is still a high possibility that children with HLHS are at risk for mental and physical abnormalities (Connor & Thiagarajan, 2007).…”

“…A heart transplant eliminates the need for several staged operations in early life, but is typically not an option because of lack of donor heart availability. Heart transplantation mortality rate is high, at around 41% (Connor, et al, 2004). Other drawbacks of heart transplantation include complications while waiting for an organ, early graft failure and the need for lifelong immunosuppressant administration (ClaxonMcKinney, 2001).…”