Here are just a few abstracts found on the PubMed database of recently published studies pertaining to HLHS. When researching articles, it is important to note the date, since even just a few year's time makes a huge difference in the outcomes. Noting the facility which published the study is also important, since some hospitals have much more experience with HLHS, and usually, much better outcomes as a result. The abstracts are free, but the full articles are usually offered for a fee. You can also look up the full articles at a medical library. These are listed merely as a guide to show what kinds of studies are being done, and to help you ask questions of the surgeons and cardiologists. |
J Pediatr 2000 Nov;137(5):646-652 Neurodevelopmental outcome of patients after the Fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions. Goldberg CS, Schwartz EM, Brunberg JA, Mosca RS, Bove EL, Schork MA, Stetz SP, Cheatham JP, Kulik TJ University of Michigan Congenital Heart Center and the Divisions of Pediatric Cardiology, Pediatric Cardiovascular Surgery, and Pediatric Psychology and the Departments of Pediatrics, Surgery, Radiology, and Biostatistics, University of Michigan, Ann Arbor, Michigan. OBJECTIVE: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV).Study design: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. RESULTS: The mean Full Scale Wechsler Intelligence score was 101.4 +/- 5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8 +/- 7.3, and for the non-HLHS subgroup it was 107.0 +/- 7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. CONCLUSION: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range. ------------------------------------------------------------------------------------------------------------------------------------------------ |
Pediatr Cardiol 19:308-315 (1998) Current Status of Staged Reconstruction for Hypoplastic Left Heart Syndrome, E.L. Bove Abstract Once considered a uniformly fatal condition, the outlook for newborns with hypoplastic left heart syndrome has been dramatically altered with staged reconstructive procedures. Refinements in operative technique and perioperative management have been largely responsible for this improved outlook. At the University of Michigan, 253 patients underwent the Norwood operation for classic hypoplastic left heart syndrome between January 1990 and November 1997. Hospital survival was 76%. Among patients considered at standard risk, survival was significantly higher (86%) than that for those patients with important risk factors (42%, p = 0.0001). Adverse survival was most strongly associated with significant associated noncardiac congenital conditions (p = 0.008) and severe preoperative obstruction to pulmonary venous return (p = 0.03). Survival following second-stage reconstruction with a hemi-Fontan or bidirectional Glenn procedure was 97%. The Fontan procedure has been completed in 94 of these patients with a hospital survival rate of 88%. Survival after the Fontan procedure improved significantly when the second stage of the reconstruction was completed with a hemi-Fontan procedure compared to a bidirectional Glenn (98% vs 81%, p = 0.05). Among the patients considered at standard risk, actuarial survival was 70% at 5 years. The largest decrease in survival occurred in the first month of life and late deaths affected primarily those patients in the high-risk group. Neurodevelopmental outcome studies demonstrated normal verbal and performance scores in the majority of patients. Staged reconstruction has significantly improved the intermediate-term outlook for patients with hypoplastic left heart syndrome. Factors addressing improvements in early first-stage survival would be expected to add significantly to an overall improved late outcome. ------------------------------------------------------------------------------------------------------------------------------------------------ |
Pediatrics 2000 May;105(5):1082-9 Neurodevelopmental outcome and lifestyle assessment in school-aged and adolescent children with hypoplastic left heart syndrome. Mahle WT, Clancy RR, Moss EM, Gerdes M, Jobes DR, Wernovsky G Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. OBJECTIVES: The purposes of this study are to describe the quality of life and cognitive function in school-aged children who have undergone staged palliation for hypoplastic left heart syndrome (HLHS), and to identify factors that are predictive of neurodevelopmental outcome in this population. METHODS: School-aged survivors with HLHS who had undergone palliative surgery at our institution were identified and mailed a questionnaire to assess subjectively quality of life, school performance, and incidence of medical complications. A subgroup of local patients underwent standardized testing of cognitive function and neurologic examination. These patients were compared with the larger (remote) group of questionnaire respondents to determine whether results may be generalizable to the entire HLHS population. Potential predictors of neurologic and cognitive outcome were tested for their association with test scores using multivariate regression analysis. RESULTS: Questionnaire results were obtained from 115 of 138 eligible children (83%; mean age: 9.0 +/- 2.0 years). Standardized testing was performed in 28 of 34 (82%) eligible local patients (mean age: 8.6 +/- 2.1 years). The majority of parents or guardians described their child's health as good (34%) or excellent (45%) and their academic performance as average (42%) or above average (42%). One third of the children, however, were receiving some form of special education. Chronic medication usage was common (64%); the incidence of medical complications was comparable to that previously reported in children with Fontan physiology. Cognitive testing of the local group demonstrated a median full scale IQ of 86 (range: 50-116). Mental retardation (IQ: <70) was noted in 18% of patients. In multivariate analysis, only the occurrence of preoperative seizures predicted lower full scale IQ. CONCLUSIONS: Although the majority of school-aged children with HLHS had IQ scores within the normal range, mean performance for this historical cohort of survivors was lower than that in the general population. ------------------------------------------------------------------------------------------------------------------------------------------------ |
Hypoplastic Left Heart Syndrome (HLHS) Information Page Selected Medical Journal Abstracts |
This web site is not intended as medical advice. Please consult your physician with any questions you may have regarding your child's condition. Copyright © 2001-2005 D.L. Hilton, all rights reserved. |
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:39-47. Neurodevelopmental outcomes in hypoplastic left heart syndrome. Mahle WT, Wernovsky G. Department of Pediatrics, Division of Cardiology, Children's Healthcare of Atlanta, Emory University School of Medicine, Atlanta, GA 30322-1062, USA. Infants with hypoplastic left heart syndrome (HLHS) are now surviving to school age and early adulthood because of surgical palliation with either staged reconstruction or transplantation. Although in some respects these patients are doing remarkably well, recent investigations have shown that they are at risk for cognitive, neuromotor, and psychosocial problems. This article examines the neurodevelopmental outcome for children and adolescents with HLHS. In addition, hemodynamic and genetic factors unique to this patient population that may impact longer-term outcome are explored. The continued evolution of the pre-, intra- and post-operative care of the patient with HLHS has significantly improved early survival. Concomitant changes in care, such as selective cerebral perfusion and neurologic monitoring, aimed at reducing neurologic insult may also favorably impact later neurodevelopmental status. -------------------------------------------------------------------------------------------------------------------------------------------------- |
Here are just a few abstracts found on the PubMed database of recently published studies pertaining to HLHS. When researching articles, it is important to note the date, since even just a few year's time makes a huge difference in the outcomes. Noting the facility which published the study is also important, since some hospitals have much more experience with HLHS, and usually, much better outcomes as a result. The abstracts are free, but the full articles are usually offered for a fee. You can also look up the full articles at a medical library. These are listed merely as a guide to show what kinds of studies are being done, and to help you ask questions of the surgeons and cardiologists. |
