Hypoplastic left heart syndrome---outcome and management

Ap, Salmon

doi:10.1136/adc.85.6.450

Cited by 14 publications

(11 citation statements)

References 9 publications

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“…These results are consistent with other available data and confirm the trend in improved immediate survival rates reported in the past 20 years by individual institutions. 15,17,36,37 We are unsure whether discharges recorded as "died during hospitalization" (n ϭ 106) represent patients who were waiting for surgical intervention or patients who were recommended for compassionate care. We observed the median LOS to be 4 days (range: 1-148 days).…”

Section: General Findingsmentioning

confidence: 99%

“…[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 transplantation have been reported. [19][20][21] Neurologic complications have been noted to occur among infants with HLHS not only as a result of the surgical procedure but also during the preoperative and postoperative periods.…”

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confidence: 99%

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

et al. 2004

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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...

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Section: General Findingsmentioning

confidence: 99%

mentioning

confidence: 99%

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

et al. 2004

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“…Some remain in Edmonton for weeks or months during pregnancy, separated from their family. Other geographic areas with available LST for HLHS have much lower rates of NSS compared to our center [6,11,22,23]. Mothers who chose palliative comfort care for HLHS have been reported more often to work in healthcare and to have more education than mothers who chose surgery, suggesting that they were more knowledgeable about the consequences and serious risks of LST described above [3,7,11,22,23].…”

mentioning

confidence: 85%

“…Outside of specialist perinatal cardiac centers, NSS and HT are not considered as the only treatment for HLHS [9,11,12,20]. However, as survival rates for HT and NSS improve, their use becomes the "standard of care" [6,7,10,21,22]. The increasing rate of prenatal diagnosis of HLHS now results in mothers being transferred from a western Canadian catchment area of over 1 million square kilometers to deliver electively in Edmonton, as has been reported from other centers [22,23].…”

mentioning

confidence: 99%

“…However, as survival rates for HT and NSS improve, their use becomes the "standard of care" [6,7,10,21,22]. The increasing rate of prenatal diagnosis of HLHS now results in mothers being transferred from a western Canadian catchment area of over 1 million square kilometers to deliver electively in Edmonton, as has been reported from other centers [22,23]. Despite this trend of increased LST use, both NSS and HT are associated with serious acute complications, with considerable pain and suffering, with repeated prolonged hospitalizations, and with significant risks of long-term physical and mental disability [8,21,22,24].…”

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confidence: 99%

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