Washington University Record, March 1, 2002

https://digitalcommons.wustl.edu/record/1925/thumbnail.jp

Disfunção vascular nos doentes com coarctação da aorta tratada

RESUMO: As cardiopatias congénitas (CC) afetam aproximadamente 1% dos recémnascidos e são responsáveis pela maior proporção de mortalidade infantil nos países desenvolvidos. A coarctação da aorta (CoA), a 6ª CC mais frequente, consiste numa estenose da aorta descendente proximal. Se não for tratada, tem uma história natural desfavorável. A cirurgia, dilatação com balão e a implantação de stent são atualmente técnicas que podem atingir o objetivo de uma remoção eficaz e duradoura da estenose ístmica, sendo a decisão baseada na idade doente, anatomia da CoA e preferência do operador ou da instituição. Contudo, um bom resultado anatómico não evita morbilidade e mortalidade de longo prazo, apresentando cerca de metade dos doentes hipertensão arterial (HTA), e registando-se mortalidade precoce, maioritariamente devido a complicações cardiovasculares e acidentes vasculares cerebrais. O perfil tensional anómalo sugere que os resultados subótimos possam ser secundários a disfunção vascular, cuja existência foi bem documentada em doentes com CoA tratada. Existem anomalias intrínsecas da estrutura arterial e função, alterações da sensibilidade neuro-hormonal ou da regulação endócrina, e fatores adquiridos, como a idade do tratamento, que contribuem para esta disfunção vascular. Os maus resultados a longo prazo podem resultar igualmente do tipo de tratamento efetuado, que provavelmente impactam de modo diverso a rigidez do istmo aórtico e potencialmente comprometem as funções da aorta. Este efeito da modalidade terapêutica não foi até ao momento estudado. A CoA não é uma simples doença mecânica que fica resolvida quando é removido o obstáculo. Objetivos e Hipóteses: O objetivo deste estudo é comparar a função vascular entre diferentes modalidades terapêuticas de CoA. A hipótese principal é a de que os doentes submetidos a dilatação com balão têm melhor função vascular que os doentes submetidos a cirurgia ou implantação de stent, pois aquela modalidade terapêutica tem menor potencial para danificar a integridade e propriedades biomecânicas da parede da aorta do que estas. Métodos: Avaliação prospetiva da função vascular usando múltiplas modalidades não invasivas, de modo a comparar os resultados de três grupos de doentes com CoA, tratados com dilatação com cirurgia, balão ou implantação de stent, após controle das variáveis de confusão. Em doentes com CoA tratada com sucesso, comparámos prospectivamente a rigidez da aorta com tonometria de aplanação e ressonância magnética cardíaca; função endotelial com tonometria arterial periférica endotelial; analise da onda de pulso com tonometria de aplanação e tonometria arterial periférica endotelial; massa ventricular esquerda e anatomia do arco aórtico com ressonância magnética cardíaca; marcadores séricos de função endotelial, inflamação, função da parede arterial e matriz extracelular; e saúde cardiovascular ideal. A análises estatística incluiu ajuste para as variáveis de confusão. Resultados: O estudo foi realizado em sete grandes centros, de Portugal e Estados Unidos da América. Foram incluídos 75 doentes, tratado por cirurgia (n=28), dilatação com balão (n=23) e implantação de stent (n=24). Os grupos tiveram idade semelhante à data de inclusão, gravidade da CoA, gradiente residual e perfil metabólico, mas eram diferentes quanto à idade à data do tratamento. A HTA, rigidez da aorta, função endotelial e massa ventricular eram semelhantes entre os grupos. Contudo, o grupo da dilatação com balão tinha mais distensibilidade regional da aorta ascendente, menor tensão arterial (TA) sistólica durante o exercício, menos alteração da variação noturna da TA, e dose menor de biomarcadores inflamatórios. Os resultados permaneceram inalterados após ajuste das potenciais variáveis de confusão, incluindo idade à data do tratamento. Conclusões: A modalidade terapêutica não estava associada à presença de HTA, rigidez arterial global e função endotelial. Contudo, os doentes com dilatação com balão tinham um perfil de função vascular mais favorável, caracterizado por maior distensibilidade da aorta ascendente, TA noturna mais baixa, menor resposta hipertensiva no esforço e menores marcadores séricos de inflamação. São necessários mais estudos para confirmar se os nossos resultados poderão contribuir para o refinamento do paradigma de tratamento da CoA, ao adicionar ao objetivo de remoção da estenose, a preservação da função vascular, quando dois ou mais tratamentos são aplicáveis.ABSTRACT: Introduction: Congenital heart disease (CHD) affects approximately 1% of liveborns and accounts for the largest proportion of infant mortality in developed countries. Coarctation of the aorta (CoA), the 6th most common CHD, consists of a narrowing of the proximal descending aorta. If left untreated, it has an unfavorable natural history. Surgery, balloon dilation (BD) or stent implantation are all current treatments that can achieve a successful long-term removal of the stenosis, and the choice is based on age, CoA anatomy, and personal or institutional preference. Coarctation is not a mere mechanical disease that is treated by removing the increased afterload. In fact, a good anatomic result does not avoid long-term cardiovascular (CV) morbidity and mortality, with late systemic hypertension (HTN) in approximately half of the patients, and reduced life expectancy, mostly due to CV complications and stroke. The abnormal blood pressure (BP) phenotype suggests that the suboptimal results are likely due to abnormal vascular function, which has been well documented in patients with repaired CoA. There are inherent changes in the arterial structure and function, impaired neuronal sensitivity or endocrinal auto-regulation, and acquired features, such as age at treatment, that contribute to vascular dysfunction in CoA. The poor long-term vascular outcome may also be impacted by the different types of repair, which likely have differing effects on the stiffness of the repaired segment and potentially compromise both the conduit and cushioning functions of the aorta. The effects of treatment modality on long-term vascular function remain uncharacterized. Aims and Hypothesis: The goal of this study is to assess vascular function in this patient population for comparison among the treatment modalities. The central hypothesis of this study was that patients who have undergone successful BD will have better vascular function than patients who have undergone successful surgical repair or stenting since this modality is least likely to damage the integrity and biomechanical properties of the aortic wall. Methods: Prospective assessment of vascular function using multiple non-invasive modalities, and compare the results among the three groups of CoA patients previously treated using surgery, BD or stent implantation after frequency matching for confounding variables. In successfully repaired CoA patients, we prospectively compared aortic stiffness by applanation tonometry and cardiac magnetic resonance (CMR); endothelial function by endothelial pulse amplitude testing; pulse waveform analysis by applanation tonometry and endothelial pulse amplitude testing; BP phenotype by office BP, ambulatory BP monitoring, and BP response to exercise; left ventricular (LV) mass and aortic morphometrics by CMR; blood biomarkers of endothelial function, inflammation, vascular wall function, and extracellular matrix; and ideal cardiovascular health. In the statistical analysis, we adjusted for potential confounders. Results: This study was done in seven, large volume centers from Portugal and the United States of America. Participants included 75 patients treated with surgery (n=28), BD (n=23), or stent (n=24). Groups had similar age at enrollment, CoA severity, residual gradient, and metabolic profile but differed by age at treatment. Systemic HTN, aortic stiffness, endothelial function, and LV mass were similar among groups. However, BD had more distensible ascending aortas, lower peak systolic BP during exercise, less impairment in diurnal BP variation, and lower inflammatory biomarkers. The results were unchanged after adjustment for potential confounders, including age at treatment. Conclusions: Treatment modality was not associated with major vascular outcomes such as systemic HTN, global aortic stiffness, and endothelial function. However, BD patients had a better vascular phenotype profile characterized by higher ascending aorta distensibility, lower night-time BP, lower peak exercise BP and lower levels of inflammatory markers. Further studies are required to confirm if our results may contribute to refining the CoA treatment paradigm by adding to the goals of therapy the preservation of vascular function when two or more treatment techniques are applicable

Blue Foundation for a Healthy Florida 10-Year Anniversary proposed keynote speaker information

Blue Foundation for a Healthy Florida 10-Year Anniversary proposed keynote speaker information

Child Health Supervision: Analytical Studies in the Financing, Delivery, and Cost-Effectiveness of Preventive and Health Promotion Services for Infants, Children, and Adolescents

Contents: Financing and Delivery of Child Health Supervision Services (An Overview of Health Insurance Coverage and Access to Child Health Supervision Services, Private Health Insurance Coverage of Preventive Benefits for Children, A 20-Year Retrospective of Child Health Supervision in Ambulatory Pediatric Settings, Ensuring Adequate Health Care Benefits for Children and Adolescents); Child Health Supervision Services and Medicaid (Informing State Medicaid Providers about EPSDT, Barriers to Full Participation in EPSDT and Possible Strategies for the Maternal and Child Bureau, Medicaid Managed Care: A Briefing Book on Issues for Children and Adolescents; State Implementation of OBRA \u2789 EPSDT Amendments within Medicaid Managed Care Arrangements)

Neurodevelopment, quality of life and burden of care of young children who have undergone cardiac interventions in central South Africa: three-month and six-month post cardiac intervention outcomes

A thesis submitted to the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, in fulfilment of the requirements for the degree of Doctor of Philosophy. Johannesburg, 2017.Over recent decades medical and surgical advances have significantly lowered the mortality rate for children born with congenital heart defects. Congenital heart disease (CHD) survivors are at high-risk of growth retardation and developmental morbidity that negatively affect their health-related quality of life (HRQOL). In addition, caring for a child with a chronic health condition such as CHD places a considerable financial and emotional burden on parents, putting them at risk of ongoing stress and psychological morbidity including anxiety and depression. The outcomes of children living with CHD and their families in South Africa (SA) are unknown. Outcomes for children with CHD in SA are likely to be further complicated by social disadvantage and Human Immunodeficiency Virus (HIV) co-infection. The aim of this observational descriptive study (Phase I and II) was to determine the pre-cardiac intervention, and three-month and six-month post-cardiac intervention development, growth, HRQOL and parenting stress outcomes of young children with CHD in central SA. Outcomes were compared over time, and variables associated with development, HRQOL and parenting stress outcomes determined. In addition, the developmental needs of young children living with CHD in central SA were to be identified. In order to meet the Phase I and II objectives, forty-eight consecutive children, 30 months and younger, and their parents were recruited into this study at the Universitas Academic Hospital in Bloemfontein. Children who had previous or emergency cardiac surgery were excluded. Development was assessed using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III), HRQOL using the Paediatric Quality of Life Inventory (PedsQLTM) and levels of parenting stress using the Parenting Stress Index Short-Form (PSI-SF). Growth outcomes were determined by z-scores calculated for growth parameters. Medical severity of the cardiac disease was rated by a paediatric cardiologist using the Cardiologists Perception of Medical Severity Scale. Baseline data was collected for 40 children. The majority of children (n=26) underwent open-heart surgery in infancy with cardiopulmonary bypass. Most children (n=30) had moderate disease severity, with twenty percent (n=8) having cyanotic heart defects. A quarter of the children (n=10) had Down syndrome (DS). Surgical outcomes were comparable to those reported in developed countries, with a mortality rate of 15%. There was a high attrition rate during Phase II of this study, with 47.5% of children and their families missing one or more follow-up visit. Mothers fulfilled the role of primary caregiver, and carried most of the burden of care. The majority of families were from a low socioeconomic backgrounds (87.5%) and mothers had low levels of education, with only 40% having graduated high school. The majority (68%) of children had suboptimal growth prior to cardiac intervention. There was significant growth catch-up for both weight (p=0.04) and head circumference (p= 0.02) by the six-month post-cardiac intervention. Complete catchup growth had not yet taken place by the six-month post-cardiac intervention, with 40.9% of the children still presenting with malnutrition. The growth trends of children with CHD with DS were found to be similar to those of children with CHD without DS. Growth in children with cyanotic heart defects tended to be poorer both before and after cardiac intervention. There was a high prevalence of moderate developmental delay across all development domains. Motor delays (27.5%) were most prevalent prior to cardiac intervention. Motor performance improved with age and post cardiac intervention, but language and cognitive performance declined with age and increasing skill complexity. There was not a significant change in the developmental outcome of the children over the timespan of this study. The developmental outcome for children with cyanotic heart defects tended to be similar to those with acyanotic heart defects. The presence of DS was significantly (p<0.001) associated with developmental outcome across all developmental domains at all time-points of assessment. Children with CHD with DS tended to have considerably poorer developmental outcomes compared to children with CHD without DS. Disease severity (p=0.02) and maternal age (p=0.01) were significantly associated with cognitive development. Age at first cardiac surgery was found to be significantly associated with language development both before cardiac intervention (p<0.01) and at three-month post-cardiac intervention (p=0.04). Suboptimal growth prior to cardiac intervention (p=0.04) and maternal age (p<0.001) were significantly associated with motor development. Developmental performance was well below the test mean on all subscales of the BSID-III at all the time points of assessment. Although the patterns of development and the prevalence of developmental delays in the current study were similar to those reported in developed countries, children living with CHD in central SA performed below the expected developmental levels for children with CHD when assessed on the BSID-III. Hypotonia was the most significant abnormal neurological finding, with 45% of the children presenting with hypotonia prior to cardiac intervention. The hypotonia tended to resolve in the children without DS by the six-month post-cardiac intervention. Overall parents’ perceived their children’s HRQOL as being relatively good, and similar to that of their healthy same-aged peers and other children with CHD in developed countries. Parents’ perception of their children’s HRQOL improved significantly after cardiac intervention (p= 0.04). Perceived HRQOL tended to be similar for children with cyanotic and acyanotic heart defects. Parents of children with CHD with DS tended to perceive their children’s HRQOL as poorer when compared with parents of children with CHD without DS. Motor development (p=0.01) and levels of parenting stress (p=0.02) were significantly associated with parents’ perceptions of their children’s HRQOL prior to cardiac intervention. The majority of parents’ (60%) experienced clinically significant levels of stress prior to their children undergoing cardiac intervention. Parenting stress decreased significantly from pre-cardiac intervention levels at both three-month (p<0.001) and six-month (p<0.001) post-cardiac intervention as the child’s cardiac symptoms resolved or decreased, and their health status improved. Parents of children with cyanotic and acyanotic heart defects tended to experience similar levels of stress. Parents of children with CHD with DS tended to experience higher levels of ongoing stress when compared with parents of children with CHD without DS. Parenting stress prior to cardiac intervention was significantly associated with parents’ perception of their child’s HRQOL (p=0.02) and language development (p=0.04). Parenting stress at threemonth post-cardiac intervention was significantly associated with age at first cardiac surgery (p=0.03), language development (p=0.03) and level of maternal education (p=0.04). HRQOL and parenting stress outcomes were closely linked before cardiac intervention. Parents perceiving their child as having a poor ability to function in everyday situations experienced increased stress levels. Based on developmental performance on the BSID-III 59% of the children in the current study would qualify for referral to early intervention (EI) services including physiotherapy, occupational therapy and speech therapy, with many children requiring access to more than one service. A home-based parent-driven developmental activity programme would likely be best suited to meet the developmental needs of children with CHD living in central SA taking into account the geography of the area and service delivery challenges in the public healthcare sector. Phase III of the study resulted in the development of a home-based developmental activity programme to meet the identified developmental needs of children with CHD in central SA. Qualitative methods, including an expert panel of rehabilitation professionals and a focus group of parents, were used to gain consensus on the content of the developmental activity programme. In conclusion, it is encouraging that the longer-term outcomes of children with CHD in central SA were not vastly different from those of children in developed countries. The greater extent of the growth retardation and developmental delay of the children in the current study is however of concern. The findings in this study strongly support the implementation of a cardiac neurodevelopmental programme as part standard cardiac care in SA. Early developmental intervention and psychosocial support services are indicated to optimise the outcome for both children living with CHD and their families. A home-based parent-driven developmental stimulation programme provides an innovative approach to meeting the developmental needs of young children living with CHD. Keywords Congenital heart disease, neurodevelopment, growth, parenting stress, health-related quality of life, home-based developmental activity programme, Bayley Scales of Infant and Toddler Development, Third Edition, Paediatric Quality of Life Inventory, Parenting Stress Index-Short Form.LG201

Advisory Committee on Immunization Practices (ACIP) summary report : October 23-24, 2019, Atlanta, Georgia

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