Cognitive and attentional functioning in adolescents and young adults with Tetralogy of Fallot and d-transposition of the great arteries

The objective of this study is to investigate cognitive and attentional function in adolescents and young adults with operated congenital heart disease. Previous research has indicated that children with congenital heart disease have deficits in broad areas of cognitive function. However, less attention has been given to survivors as they grow into adolescence and early adulthood. The participants were 18 non-syndromic adolescents and young adults with tetralogy of Fallot and d-transposition of the great arteries that required cardiac surgery before the age of 5 years, and 18 healthy, unaffected siblings (11–22 years of age for both groups). Cases with congenital heart disease and their siblings were administered Wechsler Intelligence scales and reported attention problems using the Achenbach System of Empirically Based Assessments. Cases were compared to both healthy siblings and established norms. Cases performed significantly lower than siblings on full scale IQ and processing speed, and significantly lower than norms on perceptual reasoning. Cases also reported more attention problems compared to both siblings and norms. Effect sizes varied with medium-to-large effects for processing speed, perceptual reasoning, working memory, and attention problems. Findings suggest that neurocognitive function may continue to be affected for congenital heart disease survivors in adolescence and young adulthood, and that comparisons to established norms may underestimate neurocognitive vulnerabilities.     

Intellectual development of infants, children and adolescents with congenital heart disease

Cardiac disease is the most common congenital defect in children, affecting between 3 and 10 in every 1000 live births. While significant advances in medical and surgical management have resulted in increasing numbers of survivors, it is also recognized that there is a growing population of children living with neurological impairment and lowered intellectual function. The aetiology of such impairment is multi-factorial and hypoxia is one of the factors involved. This article provides an overview of the effects of hypoxia on the intellectual development of infants, children and adolescents with congenital heart disease.     

X线伤害:先天性心脏病介入治疗知情同意的盲点

随着先天性心脏病介入治疗快速发展,受X线照射的患儿日益增多。而X线照射的危害并未受到临床医生的重视,家属也没有充分的知情同意。     

Preattentive processing of heart cues and the perception of heart symptoms in congenital heart disease

The present study was aimed at clarifying whether preattentive processing of heart cues results in biased perception of heart sensations in patients with congenital heart disease (ConHD) who are also highly trait anxious. Twenty-six patients with ConHD and 22 healthy participants categorized heart-related (heart rate) or neutral sensations (constant vibration) as either heart or neutral. Both sensations were evoked using a bass speaker that was attached on the chest of the participant. Before each physical sensation, a subliminal heart-related or neutral prime was presented. Biased perception of heart-sensations would become evident by a delayed categorization of the heart-related sensations. In line with the prediction, a combination of high trait anxiety and ConHD resulted in slower responses after a heart-related sensation that was preceded by a subliminal heart cue. Preattentive processing of harmless heart cues may easily elicit overperception of heart symptoms in highly trait anxious patients with ConHD.     

Bicuspid Aortic Valve: a Review with Recommendations for Genetic Counseling

Bicuspid aortic valve (BAV) is the most common congenital heart defect and falls in the spectrum of left-sided heart defects, also known as left ventricular outflow tract obstructive (LVOTO) defects. BAV is often identified in otherwise healthy, asymptomatic individuals, but it is associated with serious long term health risks including progressive aortic valve disease (stenosis or regurgitation) and thoracic aortic aneurysm and dissection. BAV and other LVOTO defects have high heritability. Although recommendations for cardiac screening of BAV in at-risk relatives exist, there are no standard guidelines for providing genetic counseling to patients and families with BAV. This review describes current knowledge of BAV and associated aortopathy and provides guidance to genetic counselors involved in the care of patients and families with these malformations. The heritability of BAV and recommendations for screening are highlighted. While this review focuses specifically on BAV, the principles are applicable to counseling needs for other LVOTO defects.     

A Comparison of Drawings and Sentence Completion Responses of Congenital Heart Children with Normal Children

The House-Tree-Person, Sentence Completion and Inside-of-the-Body tests were administered to 20 congenital heart children and their matched controls. The stories of the H-T-P test of the congenital heart children tended to have more themes about illness-health, and life-death than those of the normal children. Similarly, more themes of illness-health were found in the congenital heart children on the Sentence Completion test (P < .001). Although the congenital heart children did not draw a greater number of hearts than the control children, they did reveal a tendency to draw relatively larger hearts and relatively fewer other internal organs (P < .05).

A major implication of the findings is that the body distortion of a congenital heart child pertains more to his perception of the inside of his body than to the view of his external body. This suggests that the Inside-of-the-Body test would be a useful addition to projective techniques aimed at investigating distortion of body image by the physically handicapped.     

Perceived parenting style, self-esteem and psychological distress in adolescents with heart disease

The aim of the study was to assess the relationships between perceived parenting style, depressed mood, anxiety and self-esteem in adolescents with heart disease compared with healthy adolescents. Forty-five adolescents, aged 12-18 with congenital or acquired heart disease and 50 healthy age-matched adolescents answered perceived parental behaviour, self-esteem, depressed mood and anxiety questionnaires. The study group reported higher perceived acceptance and lower perceived parental control than healthy adolescents, but similar levels of depressed mood, anxiety and self-esteem. Fischer's r-to-z transformation and regression analyses showed different associations between perceived parenting style and depressed mood, anxiety and self esteem. In the study group, higher perceived parental acceptance was associated with lower depressed mood and higher self-esteem, whereas these associations were not significant in the control group. In the control, but not the study group, higher perceived parental control was associated with lower depressed mood and lower anxiety. Parenting style proved to exert a differential effect on adolescents with and without heart disease. For the former, perceived parental acceptance had a more substantial effect on psychological well-being than perceived parental control. Professionals caring for these adolescents should be aware of the special importance of parenting style on the well-being of adolescents with heart disease, and address this issue in the clinical setting with the patients and their parents.     

超声心动图与心血管造影X线引导下先心病介入封堵治疗的比较

目前先天性心脏病介入治疗已成为介入心脏病学的新热点,而先心病介入治疗离不开影像学引导,目前临床上常用的影像学引导方式主要有超声心动图和心血管造影两种,本文针对两者优缺点,从治疗方式、疗效、费用等方面进行比较,为临床治疗思路提供借鉴。     

婴幼儿喘息的分类和小儿哮喘的鉴别诊断

喘息是小儿时期常见的症状,尤其是婴幼儿。婴幼儿喘息分为三种类型：一过性的早期喘息,只发生在3岁以前的喘息。无家族的哮喘病史,与过敏原的致敏无关,与父母吸烟有关。持续性的早发的喘息,即非过敏的喘息,与病毒感染诱发有关。晚发的喘息/哮喘,即过敏性的喘息, 有湿疹和哮喘的气道病理特点。在诊断支气管哮喘前,还必须排除以下疾病：先天性气道和肺的发育异常,先天性心、血管异常,异物吸入,胃食管返流,支气管结核,纤毛不动综合征,免疫缺陷病等。     

先天性心脏病新技术——介入导管术

近20年间,伴随着科学技术的发展以及医疗水平的提高,介入导管术治疗先天性心脏病逐渐成为主要选择。该项技术微创、疗效好、安全、并发症少、住院时间短,深受患者青旺火。本文讨论该项技术的主要适应范围以及今后的发展趋势,同时指出医疗技术的创新必须遵守的哲学性原则。     

Factors Associated with Perceived Cognitive Problems in Children and Adolescents with Congenital Heart Disease

The objective of the present study was to assess demographic and disease-specific factors associated with perceived cognitive problems in children and adolescents with heart disease. 246 children with congenital heart disease ages 8–18 years and their parents completed the Cognitive Problems Scale of the Pediatric Quality of Life Inventory Cardiac Module. The 5-item Cognitive Problems Scale assesses school-related cognitive problems including attention and memory from the perspective of the child and parent. Multiple linear regression analysis was conducted with demographic variables and disease-specific factors entered as independent variables and perceived cognitive problems as the dependent variable. Socioeconomic status (SES) and cardiac disease severity were significantly associated with parent proxy-reported cognitive problems (p < .05). None of the demographic or disease-specific variables were significantly associated with child self-reported cognitive problems. In conclusion, our data suggest that assessment of perceived cognitive problems in children and adolescents with greater disease severity and from lower SES homes may facilitate early identification of children who need formal evaluation and interventions to address cognitive impairments.     

Multimethod study of clinical judgment

Expert and novice clinicians judged the likelihood of disease alternatives and gave thinking-aloud protocols as they evaluated simulated cases of congenital heart disease. Specific combinations of cues in the patient data were manipulated to create alternate versions of a single case so that the use of critical cues could be identified. Analyses of variance of subjects' disease judgments revealed differences between expert and novice clinicians in their use of critical cues and cue combinations. Analyses of the thinking-aloud protocols revealed that clinicians with different degrees of expertise employed different interpretations of patient data cues as well as qualitatively distinct "lines of reasoning" in reaching clinical judgments.     

Legal and Ethical Considerations in Allowing Parental Exemptions From Newborn Critical Congenital Heart Disease (CCHD) Screening

Critical congenital heart disease (CCHD) screening is rapidly becoming the standard of care in the United States after being added to the Recommended Uniform Screening Panel (RUSP) in 2011. Newborn screens typically do not require affirmative parental consent. In fact, most states allow parents to exempt their baby from receiving the required screen on the basis of religious or personally held beliefs. There are many ethical considerations implicated with allowing parents to exempt their child from newborn screening for CCHD. Considerations include the treatment of religious exemptions in our current legal system, as well as medical and ethical principles in relation to the rights of infants. Although there are significant benefits to screening newborns for CCHD, when a parent refuses for religious or personal beliefs, in the case of CCHD screening, the parental decision should stand.     

Alzheimer's disease in Down syndrome: neurobiology and risk

Down syndrome (DS) is characterized by increased mortality rates, both during early and later stages of life, and age-specific mortality risk remains higher in adults with DS compared with the overall population of people with mental retardation and with typically developing populations. Causes of increased mortality rates early in life are primarily due to the increased incidence of congenital heart disease and leukemia, while causes of higher mortality rates later in life may be due to a number of factors, two of which are an increased risk for Alzheimer's disease (AD) and an apparent tendency toward premature aging. In this article, we describe the increase in lifespan for people with DS that has occurred over the past 100 years, as well as advances in the understanding of the occurrence of AD in adults with DS. Aspects of the neurobiology of AD, including the role of amyloid, oxidative stress, Cu/ZN dismutase (SOD-1), as well as advances in neuroimaging are presented. The function of risk factors in the observed heterogeneity in the expression of AD dementia in adults with DS, as well as the need for sensitive and specific biomarkers of the clinical and pathological progressing of AD in adults with DS is considered.     

A Therapeutic Recreation Camp for Children with Congenital Heart Disease: Examining Impact on the Psychosocial Well-Being of Parents

Therapeutic recreation camps have been considered to be an effective context for improving the psychosocial functioning of children with congenital heart disease (CHD). However, there is limited research regarding their impact on parents. Nine parents participated in two semi-structured interviews before and after their children attended a camp for children with CHD. Parents described their perceptions of their children’s camp experiences and identified the psychosocial utility of camp for themselves. Camp enabled parents to let their children grow and be independent, and helped reduce parents’ restricting and overprotective behaviours. Camp also provided parents with respite care, which alleviated their need to be ‘cardiac’ parents. These findings strengthen the case for therapeutic recreation camps and underscore the importance of expanding camp access to more families affected by CHD.     

Neurodevelopmental functioning in children being evaluated for heart transplant prior to 2 years of age

Cardiac defects represent the most common type of birth defect and children with these abnormalities are known to experience a variety of developmental and neuropsychological delays. Children receiving heart transplants may be at the highest risk for delays as they tend to represent the most severely ill patients with heart disease. This study investigates neurocognitive and adaptive functioning in 20 children under 2 years of age who were undergoing evaluation for heart transplant, comparing their performance to that of normative samples. The results indicate delays across various domains of neurocognition, including general cognitive, receptive language, expressive language, fine motor, and gross motor skills. In addition, adaptive and social-emotional functioning fell significantly below that of normative samples. No significant differences were found when comparing the test performance of patients with congenital heart defects (n = 12) to patients with cardiomyopathy (n = 8). Secondary analyses investigating the impact of genetic conditions, premature birth, and non-English language dominance showed minor changes in results across some domains, although this may have been due, at least in part, to decreased power to detect differences. Overall, this study’s results support the need for early evaluation, intensive intervention (e.g., speech, occupational, and physical therapy), and continued monitoring of these patients’ neurodevelopmental/neuropsychological functioning over time. Future research should examine longitudinal changes in development using both pre- and post-transplant data and should investigate the impact of early intervention.     

老年舒张性心力衰竭诊治的思考

舒张性心力衰竭在老年高血压、冠心病患者中发病率较高,但临床症状、体征不特异,不典型,常容易漏诊。因此,临床工作中应注意发现舒张性心力衰竭相关的症状、体征,结合胸片有肺淤血、超声心动图左室射血分数(left ventriculare-jection fraction,LVEF)正常、血浆脑钠肽(brain natriuretic peptide,BNP)增高等,早期诊断及治疗,提高老年患者的生活质量,减少晚期混合型心力衰竭的发病率。     

血小板计数与肝素耐药的相关性研究

研究心脏手术中的血小板（PLT）计数与肝素耐药的相关性。随机将120例行心脏手术患者根据PLT计数分成仍血小板组（PLT〈150×10^9·L^-1）和高血小板组（PLT〉250×10^9·L^-1）,每组各60例。麻醉后抽取中心静脉血监测浏活凝血时间（ACT）基础值,肝素化后ACT值,肝素总用量和鱼精蛋白用量,并计算鱼精蛋白／肝素用量。首次肝素化石静脉血ACT值两组间差异无统计学意义。但两组ACT基础值差异有统计学意义（P〈0．05）。高血小板组肝素抗凝并足者有26例（43．3％）,低血小板组仅4例（6．67％）,两组间差异有统计学意义（P〈0．01）。肝素及鱼精蛋白用量均有朔计学意义,前者用量较大（P〈O．01）而后者用量较少（P〈O．05）。心脏手术时血小板计数高者易出现肝素抗凝不足。肝素需要量较大而鱼精蛋白用量较少。高血小板组以先心病患儿为多。     

Gender and cardiovascular disease: a psychobiological and integrative approach

Coronary artery disease is the most common cause of morbidity, mortality, and economic loss in all industrialized countries. Although there are gender differences in the prevalence, causes, symptoms, treatment, and outcome of heart disease, the differences are complex and often misunderstood. These gender differences are a function of psychological influences, physiological influences, and an interaction of the two. Understanding these complex interactions and how they differentially influence the development and progression of heart disease will ultimately contribute to a greater understanding of how to integrate information from medicine and epidemiology with that from psychology and behavioral medicine. Ultimately, it is this integrated approach that will allow us to better understand heart disease in both men and women.