Karen Uzark

Neurodevelopmental Outcomes in Children With Congenital Heart Disease: Evaluation and Management A Scientific Statement From the American Heart Association

Background— The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population. Methods and Results— A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms. The reference lists of identified articles were also searched. The American College of Cardiology/American Heart Association classification of recommendations and levels of evidence for practice guidelines were used. A management algorithm was devised that stratified children with CHD on the basis of established risk factors. For those deemed to be at high risk for developmental disorder or disabilities or for developmental delay, formal, periodic developmental and medical evaluations are recommended. A CHD algorithm for surveillance, screening, evaluation, reevaluation, and management of developmental disorder or disability has been constructed to serve as a supplement to the 2006 American Academy of Pediatrics statement on developmental surveillance and screening. The proposed algorithm is designed to be carried out within the context of the medical home. This scientific statement is meant for medical providers within the medical home who care for patients with CHD. Conclusions— Children with CHD are at increased risk of developmental disorder or disabilities or developmental delay. Periodic developmental surveillance, screening, evaluation, and reevaluation throughout childhood may enhance identification of significant deficits, allowing for appropriate therapies and education to enhance later academic, behavioral, psychosocial, and adaptive functioning.

The PedsQL™ 4.0 Generic Core Scales: Sensitivity, Responsiveness, and Impact on Clinical Decision-Making

The PedsQLTM 4.0 (Pediatric Quality of Life InventoryTM) Generic Core Scales are child self-report and parent proxy-report scales developed to measure health-related quality of life (HRQOL) in children and adolescents ages 2–18. The PedsQLTM 4.0 Generic Core Scales consist of 23 items applicable for healthy school and community populations and pediatric populations with acute and chronic health conditions. The 4 PedsQLTM 4.0 Generic Core Scales (Physical, Emotional, Social, School) were administered to 209 children and 269 parents (289 subjects accrued overall) recruited from pediatric cardiology, orthopedics, and rheumatology clinics. Sensitivity, responsiveness, and the impact on clinical decision-making were determined. The PedsQLTM was differentially sensitive to increasing degrees of cardiac disease severity in the cardiology clinic setting and responsive to clinical change over time in the pediatric orthopedics clinic setting. In the pediatric rheumatology clinic setting, the PedsQLTM demonstrated an impact on clinical decision-making, resulting in subsequent increases in HRQOL.

Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: The Transition Process and Medical and Psychosocial Issues: A Scientific Statement From the American Heart Association

Many children born with complex childhood illnesses that historically caused early death are now surviving into adulthood with the expectation of leading meaningful and productive lives. They will ultimately need to transition their care from pediatric to adult-centered care. Unfortunately, in the absence of structured programs to guide this transition, there is often delayed or inappropriate care, improper timing of the transfer of care, and undue emotional and financial stress on the patients, their families, and the healthcare system. At its worst, and as frequently happens now, patients are lost to appropriate follow-up. In fact, the number of adults with congenital heart disease (CHD) in the United States is rising exponentially and now exceeds 1 000 000.1,–,7 At least half of these patients may have complex CHD. Fewer than 30% of adults with CHD are seen by appropriate specialized providers. Fewer than 15% of these patients, who are seen in specialty adult CHD (ACHD) clinics, have CHD that is classified as severe.8 Thus, adolescents with CHD constitute a growing population of individuals for whom a well-planned and well-executed “transition process” is essential. The goals of a formal transition program are to prepare young adults for transfer of care. It should provide uninterrupted health care that is patient centered, age and developmentally appropriate, flexible, and comprehensive. It should include age-appropriate education about medical conditions and promote skills in communication, decision making, self-care, and self-advocacy.9,–,13 It should foster greater personal and medical independence and a greater sense of control over health, healthcare decisions, and psychosocial environment. The ultimate goal of a transition program is to optimize the quality of life (QOL), life expectancy, and future productivity of young patients.14 We acknowledge that the development of ideal transition programs is a …

Quality of life in children with heart disease as perceived by children and parents

OBJECTIVES. The aims of this study were to evaluate and compare the parent-reported and self-reported quality of life of children who have cardiovascular disease with the healthy pediatric population across age groups and to determine the relationship between perceived quality of life and severity of cardiovascular disease. METHODS. The Pediatric Quality of Life Inventory (PedsQL) Generic Core Scales and Cardiac Module were administered to 475 families including 347 children with cardiovascular disease during a pediatric cardiology outpatient visit. The PedsQL scores reported by children with cardiovascular disease and their parents were compared with pediatric population norms. The relationship between Pediatric Quality of Life Inventory scores and patient characteristics was examined. RESULTS. By self-report, mean PedsQL scores for children with cardiovascular disease were significantly lower than healthy child norms for physical and psychosocial functioning. Psychosocial quality of life scores were classified as significantly impaired as reported by 21% of children ≥8 years of age. Even among children with less severe cardiovascular disease, 19.2% reported significantly impaired psychosocial quality of life. By parental report, overall PedsQL scores were not significantly different from healthy children except in the teenage group, and both the 8- to 12-year-old and teenage groups had lower mean psychosocial quality of life scores than healthy peers. Parent-reported mean PedsQL scores for both physical and psychosocial quality of life were significantly lower in children with more severe cardiovascular disease. Children with more severe cardiovascular disease reported lower mean scores for physical functioning, but smaller differences in psychosocial quality of life scores were observed related to disease severity. CONCLUSIONS. Overall, children with cardiovascular disease perceived lower quality of life than healthy children across all age groups. As perceived by parents, overall quality of life was not significantly different in young children with cardiovascular disease, but children with more severe cardiovascular disease have worse physical and psychosocial quality of life. One in 5 children with cardiovascular disease perceives impaired psychosocial functioning, including children with mild disease severity.

Cardiovascular Monitoring of Children and Adolescents With Heart Disease Receiving Medications for Attention Deficit/Hyperactivity Disorder A Scientific Statement From the American Heart Association Council on Cardiovascular Disease in the Young Congenital Cardiac Defects Committee and the Council on Cardiovascular Nursing

Over the past decade, concerns have been raised regarding the safety of a variety of psychotropic medications in children and adolescents, the appropriate selection of patients for therapy, and the indications for cardiovascular monitoring. In 1999, concerns over potential cardiovascular effects of psychotropic drugs, especially tricyclic antidepressants1,2 but including stimulants, prompted the American Heart Association (AHA) scientific statement “Cardiovascular Monitoring of Children and Adolescents Receiving Psychotropic Drugs.”3 At that time, no specific cardiovascular monitoring was recommended for the use of stimulant medications. Since that time, a constellation of circumstances have come together, necessitating a second look at this complicated issue. These circumstances include an increased awareness of the presence of attention deficit/hyperactivity disorder (ADHD) in the general population and in children with preexisting cardiac conditions; public concerns about the side effects and toxicities of medications, especially psychotropic medications in children; and regulatory factors and warnings issued by the US Food and Drug Administration (FDA) and by the pharmaceutical industry in response to the FDA. This writing group was convened in response to FDA concerns with regard to the safety of the ADHD drugs and with regard to the identification of children with underlying cardiovascular abnormalities. At a time when there is much discussion of the side effects of drugs and of the use of psychotropic drugs in children in the media and lay literature, it is particularly important for the medical profession to play a significant role in critically evaluating the use of stimulant medication in children, including those who may have undiagnosed heart disease and those who are known to have heart disease. The writing group for “Cardiovascular Monitoring of Children and Adolescents With Heart Disease Receiving Medications for Attention Deficit/Hyperactivity Disorder” reviewed the literature relevant to this topic since the last publication of the AHA scientific …

Task Force 2: special health care needs of adults with congenital heart disease

Transition into the adult health care system is crucial for patients with congenital heart disease (CHD), as well as for adolescents with many other chronic conditions. Indeed, “arranging efficient and caring transfer for adolescents from pediatric to adult care (is)…one of the great challenges

The Pediatric Quality of Life Inventory™ in children with heart disease

Abstract Objective : To determine the feasibility, reliability and validity of the pediatric quality of life inventory (PedsQL™) in children with heart disease. Participants and setting: The study sample was comprised of parents of children with heart disease between the ages of 2 and 18 years including 250 child-respondents 5–18 years of age followed at a large, hospital-based pediatric cardiology clinic. Procedure/design : Parents and children completed the PedsQL™ 4.0 Generic Core Scales and the newly developed PedsQL™ 3.0 Cardiac Module during a pediatric cardiology outpatient visit. Child and parent scores on the PedsQL™ Generic Core Scales were compared to known healthy children/parents. Measurement properties were computed for the PedsQL™ Cardiac Module Scales. Cross-informant variance between children and parents was assessed. Results : The percentage of missing item responses was less than 2% for both parent and child-respondents, supporting the feasibility of PedsQL™ administration in the outpatient setting. Comparisons between all PedsQL™ Generic Core Scales for healthy children and children with heart disease revealed a significant difference ( P P =0.114). There was a significant difference in self-reported physical functioning between children with complex heart disease and the healthy sample ( P Conclusions : Results support the feasibility, reliability and validity of the PedsQL™ Generic Core Scales and Cardiac Module to assess health-related quality of life in children with heart disease.

Aortopulmonary collateral vessels and prolonged pleural effusions after modified Fontan procedures

Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.

Lower weight-for-age z score adversely affects hospital length of stay after the bidirectional Glenn procedure in 100 infants with a single ventricle

OBJECTIVE Poor growth has been described in infants with a single ventricle; however, little is known regarding its effect on surgical outcomes. We sought to assess the effect of nutritional status at the time of the bidirectional Glenn procedure on short-term outcomes. METHODS We performed a retrospective case series of children who underwent the bidirectional Glenn procedure at our institution between January 2001 and December 2007. Anthropometric measurements were recorded at the time of neonatal admission and the bidirectional Glenn procedure. Data from preoperative echocardiograms and cardiac catheterization were recorded. The primary outcome variable was length of hospital stay. RESULTS Data on 100 infants were included for analysis. Age at the time of the bidirectional Glenn procedure was 5.1 months (range, 2.4-10 months). The median weight-for-age z score at birth was -0.4 (range, -2.6 to 3.2), and by the time of the bidirectional Glenn procedure, it had decreased to -1.3 (range, -3.9 to 0.6). In multivariable modeling longer postoperative hospital stays were predicted by lower weight-for-age z score (P = .02), younger age (P < .001), being fed through a gastrostomy tube (P = .01), and undergoing concomitant aortic arch reconstruction (P < .001) at the time of the bidirectional Glenn procedure. CONCLUSIONS There is suboptimal weight gain between neonatal discharge and the bidirectional Glenn procedure. A lower weight-for-age z score and younger age at the time of the bidirectional Glenn procedure affects length of hospital stay independent of hemodynamic or echocardiographic variables.

Neurodevelopmental outcomes in children with Fontan repair of functional single ventricle

Objectives. The purpose of this study was to assess the neurodevelopmental status of children after Fontan repair of functional single ventricle and to examine the relationship between cognitive function and selected patient characteristics. Study Design. Neurodevelopmental tests including the Stanford-Binet Intelligence (IQ) scale and the Developmental Test of Visual Motor Integration (VMI) were administered to 32 children (26 months to 16 years of age) with complex single ventricle. The mean and distribution of IQ and VMI scores were compared with population norms. The relationship between test scores and patient characteristics was examined utilizing analysis of variance and correlational methods. Results. The majority of children had intellectual function within the normal range (mean, 97.5 ± 12.1). Below average VMI scores were found in 21.4% of children. There were no significant correlations between intellectual function or visual motor integration ability and preoperative oxygen saturation or age at Fontan. Children who had deep hypothermic circulatory arrest during a prior Norwood procedure tended to have a lower IQ score. Conclusions. Intellectual development in children with Fontan repair of complex heart defects is essentially within the normal range. Visual motor integration deficits may be more prevalent in these children. In our population, the duration and degree of preoperative hypoxemia had no apparent effect on cognitive function.