Respiratory Syncytial Virus (RSV) infection is common among young children. Congenital Heart Disease (CHD) is a risk factor of severe illness and hospitalization. Palivizumab prophylaxis reduces the severity of RSV infection and reduces the risk of hospitalization for children at high risk of severe illness, such as children born premature or with CHD. The aim of this thesis was to evaluate compliance with national guidelines for prophylactic treatment and to study the Relative Risk (RR) of hospitalization due to RSV and unspecified Respiratory Tract Infection (RTI) for children with CHD. In a prospective study, questionnaires were sent to all paediatric cardiology centres in Sweden with questions about prophylactic treatment. Hospitalization rates were retrieved from the national inpatient registry. Heart defects were grouped according to type and the relative risk of hospitalization was calculated for each group and for summer and winter seasons. Half of the patients received prophylactic treatment later than recommended in the guidelines. The risk of hospitalization due to RSV infection was increased (RR=2.06 95% CI 1.6-2.6; p < 0.0001) for children with CHD compared to children without CHD. The RR of hospitalization was also increased for all CHD subgroups, and was further increased during summer for children with the more severe CHD. We conclude that guidelines for prophylactic treatment were not followed and that the risk of hospitalization due to RSV and unspecified RTI was increased for all subgroups of CHD. The risk was increased both during winter and summer and we therefore argue that information to health personnel and parents should include that the risk of severe RTI is present all year round for children with CHD. / Respiratoriskt syncytialvirus (RSV) är det vanligaste förkylningsviruset och de allra flesta barn drabbas före två års ålder. RSV kan leda till allvarlig luftvägsinfektion hos alla barn, men speciellt hos dem med medfött hjärtfel. Någon botande läkemedelsbehandling finns inte för RSV, utan de medicinska insatserna får inriktas mot att mildra sjukdomsförloppet och för svårt sjuka barn krävs sjukhusvård för att exempelvis erhålla syrgasbehandling. Det finns inget vaccin mot RSV, men barn som riskerar att bli svårt sjuka kan behandlas profylaktiskt med en monoklonal antikropp (Palivizumab) som ges som injektion en gång per månad under vintersäsong. Vissa barn med svårt hjärtfel får denna profylaktiska behandling enligt nationella riktlinjer. Vår första studie visade att ungefär hälften av barnen med medfött hjärtfel, aktuella för profylax mot RSV, fick behandlingen senare än vad de nationella riktlinjerna rekommenderade. Denna studie genomfördes via en enkät till alla landets barnkliniker under två vintersäsonger. Vi såg även att något fler barn än förväntat (4.6%) fick RSV-infektion trots profylaktisk behandling och för cirka en tredjedel av dessa barn fördröjdes tiden till hjärtoperation. Behovet av sjukhusvård kan användas som mått på hur svårt ett sjukdomsförlopp är, och baserat på Socialstyrelsens slutenvårdsregister studerade vi alla barn under två års ålder och fann att den relativa risken för sjukhusvård på grund av RSV var högre för barn med hjärtfel än för barn utan hjärtfel (RR=2.06 95% CI 1.6-2.6; p < 0.0001). I vår andra studie, baserad på slutenvårdsregistret, beräknade vi den relativa risken för sjukhusvård på grund av RSV, för barn med olika former av hjärtfel och uppdelat i sommar- och vintersäsong. Risken för sjukhusvård var ökad för alla barn oavsett typ av hjärtfel, och detta gällde såväl under vintern som under sommaren. Barn med de allvarligaste formerna av hjärtfel hade högre risk för sjukhusvård under sommaren jämfört med deras risk under vintern, medan barn med vad som anses vara lättare hjärtfel hade ökad risk för sjukhusvård under hela året, utan någon större skillnad i risk mellan vinter och sommar. Att barn med hjärtfel riskerar att bli svårt sjuka i RSV är väl känt, men våra resultat visar att denna risk även existerar under sommarhalvåret, då det inte är RSV-säsong och då profylax inte ges. Vi fann också att barn med vad som anses vara lättare hjärtfel löper lika stor risk att drabbas av svårare sjukdomsförlopp med sjukhusvård under vintern, som barn med svårare hjärtfel. Att denna information sprids till såväl sjukvårdspersonal som arbetar med denna patientgrupp som till föräldrar med hjärtsjuka barn är viktigt, för att belysa att även dessa barn behöver skyddas, och detta inte bara under vintern och RSV-säsongen.

abstract: The advent of medical imaging has enabled significant advances in pre-procedural planning, allowing cardiovascular anatomy to be visualized noninvasively before a procedure. However, absolute scale and tactile information are not conveyed in traditional pre-procedural planning based on images alone. This information deficit fails to completely prepare clinicians for complex heart repair, where surgeons must consider the varied presentations of cardiac morphology and malformations. Three-dimensional (3D) visualization and 3D printing provide a mechanism to construct patient-specific, scale models of cardiovascular anatomy that surgeons and interventionalists can examine prior to a procedure. In addition, the same patient-specific models provide a valuable resource for educating future medical professionals. Instead of looking at idealized images on a computer screen or pages from medical textbooks, medical students can review a life-like model of patient anatomy. In cases where surgical repair is insufficient to return the heart to normal function, a patient may proceed to advanced heart failure, and a heart transplant may be required. Unfortunately, a finite number of available donor hearts are available. A mechanical circulatory support (MCS) device can be used to bridge the time between heart failure and reception of a donor heart. These MCS devices are typically constructed for the adult population. Accordingly, the size associated to the device is a limiting factor for small adults or pediatric patients who often have smaller thoracic measurements. While current eligibility criteria are based on correlative measurements, the aforementioned 3D visualization capabilities can be leveraged to accomplish patient-specific fit analysis. The main objectives of the work presented in this dissertation were 1) to develop and evaluate an optimized process for 3D printing cardiovascular anatomy for surgical planning and medical education and 2) to develop and evaluate computational tools to assess MCS device fit in specific patients. The evaluations for objectives 1 and 2 were completed with a collection of qualitative and quantitative validations. These validations include case studies to illustrate meaningful, qualitative results as well as quantitative results from surgical outcomes. The latter results present the first quantitative supporting evidence, beyond anecdotal case studies, regarding the efficacy of 3D printing for pre-procedural planning; this data is suitable as pilot data for clinical trials. The products of this work were used to plan 200 cardiovascular procedures (including 79 cardiothoracic surgeries at Phoenix Children's Hospital), via 3D printed heart models and assess MCS device fit in 29 patients across 6 countries. / Dissertation/Thesis / Doctoral Dissertation Bioengineering 2015

No cuidado cotidiano a crianças portadoras de cardiopatias congênitas percebe-se que os pais têm dificuldade para lidar, no domicílio, com as situações decorrentes da própria situação clínica, tais como cianose, prevenção da endocardite infecciosa, administração dos fármacos, etc. Com a finalidade de conhecer melhor os determinantes e possíveis soluções para tais situações, buscou-se identificar trabalhos científicos que abordassem as necessidades de informação e suporte a pais de criança cardiopatas, de 0 a 12 anos de idade, quanto à sete pontos: cardiopatia congênita propriamente dita; como lidar com a cianose; promoção de atividade física; promoção da alimentação; promoção da saúde bucal; prevenção da endocardite infecciosa e administração de fármacos. A busca foi realizada nas bases de dados MEDLINE, Cochrane, CINAHL, LILACS e Scielo, tendo-se limitado o período entre janeiro de 1997 e abril de 2007. Foram identificados 17 estudos, nos quais foi utilizada a análise temática de conteúdo como referencial metodológico. As categorias de análise foram os sete pontos previamente determinados. Os resultados mostraram que há categorias pouco exploradas, como, cuidados por ocasião de crise de cianose, promoção de atividade física e administração de fármacos. As outras concentram a maior parte dos estudos. São elas: conhecimento dos pais sobre a cardiopatia, promoção de alimentação (compreendendo aleitamento natural), promoção da saúde bucal e prevenção da endocardite infecciosa. Na análise geral percebe-se que o conhecimento dos pais, como um todo, é incompleto e fragmentado, tanto em países desenvolvidos, quanto nos em desenvolvimento. São descritos cuidados prestados por enfermeiros, dentistas e médicos, entre outros. Programas de capacitação de pais são poucos e apenas um é descrito como tendo êxito. Tais resultados apontam para a necessidade de mudanças, tanto em termos da relação direta com os pais, quanto em termos de re-organização dos serviços com vistas a abarcar de modo mais completo a necessidade de informação e suporte a estes / In the daily care regarding to children bearing congenital heart disease one can realize that parents have difficulties to deal, at home, with situations coming from the clinical situation itself, such as cyanosis, prevention of the infective endocarditis, administration of medicines, etc. In order to know better the main causes and possible solutions to such situations, one has sought to identify scientifical studies which approach the necessities of information and support to the parents of congenital heart disease from 0 up to 12 years old concerning seven points: congenital heart disease in the very sense of the problem; how to deal with cyanosis; promotion of physical activity; promotion of feeding; promotion of mouth and teeth hygiene; prevention of infective endocarditis and administration of medicines. The search was done in the bases of data MEDLINE, Cochrane, CINAHL, LILACS and Scielo, in the period between 1997 January to 2007 April. Seventeen studies were identified and the tematic analyses of content was used as methodological reference. The categories to analyse were the 7 points previously determinate. The results showed that there are categories with few reaserches carried out, as, for example, the care in situations of cyanosis crisis, promotion of physical activity and administration of medicines. The other ones concentrate the most of the studies. They are: parents\' knowledge about heart disease, promotion of feeding (including breast-feeding), promotion of mouth and teeth hygiene and prevention of infective endocarditis. The general analyses demonstrates that the parents\' knowledge, as a whole, is incomplete and fragmented both in developed world\'s countries and in the developing world ones. Cares rendered by nursery, odontological and medical staff are described along other sort of cares. Programs to capacitate parents are few and only one is describeb as succesful. Such results point to the need of changing.concerning the straight relation with parents and concerning the re-organization of the service as well, in order to embrace in a more complete way the necessity of information and support to parents

A dor ocasiona uma série de alterações sistêmicas que afetam o desenvolvimento do recém-nascido (RN), em particular, os submetidos à cirurgia cardíaca, além de acarretar alterações hemodinâmicas que podem comprometer sua sobrevivência. O objetivo do estudo foi caracterizar a população de neonatos submetidos a cirurgias cardíacas e descrever as práticas de controle da dor implementadas no primeiro pós-operatório. Estudo descritivo, transversal realizado na Sociedade Hospital Samaritano de São Paulo. Os dados foram obtidos de prontuários médicos de RN submetidos à cirurgia cardíaca, internados entre julho de 2001 e dezembro de 2005. Os registros sobre a avaliação da dor e intervenção medicamentosa para o alívio da dor no primeiro pós-operatório foram analisados. Dentre quarenta e dois RN submetidos a cirurgias cardíacas no período estudado, 30 atenderam aos critérios de inclusão e constituíram a casuística do estudo. A maioria dos RN nasceu a termo, pesava 2.500g ou mais na data do procedimento cirúrgico e foi submetida à cirurgia na primeira semana de vida. Foram identificados 24 diferentes diagnósticos de cardiopatias congênitas e 14 tipos de intervenções cirúrgicas, corretivas ou paliativas, no grupo estudado. Dentre 30 prontuários, foram identificados registros de avaliação de dor em 24 (80,0%). A avaliação por meio da escala NIPS foi anotada em 17 (56,7%) prontuários e em 15 (50,0%) verificou-se registro de comportamentos e alterações nos parâmetros fisiológicos indicativos de dor. Dos RN avaliados, 17 (56,7%) apresentaram registro de ocorrência de dor no primeiro pós-operatório. A maioria, 29 (96,7%), recebeu analgesia farmacológica: 24 (80,0%) RN receberam citrato de fentanil contínuo, 5 (16,7%) RN, dipirona intermitente, 5 (16,7%) RN, citrato de fentanil contínuo e dipirona intermitente, 1 (3,3%) RN recebeu citrato de fentanil contínuo e morfina intermitente. As doses administradas variaram amplamente. Foram também utilizados sedativos associados aos analgésicos: 12 (40,0%) RN receberam midazolan contínuo, 5 (16,7%) RN, midazolan intermitente, 1 (3,3%), besilato de cisatracúrio contínuo, 1 (3,3%) cloridrato de clorpromazina contínuo e 1 (3,3%) propofol intermitente Os resultados evidenciam não haver uniformidade e padronização na avaliação da dor pós-operatória e nos tipos e doses de fármacos utilizados para o controle da dor pós-operatória dos RN submetidos a cirurgias cardíacas no serviço estudado. / Pain causes several systemic alterations that affect the newborn’s development, specially the ones who underwent cardiac surgery. Besides, hemodynamic alterations related to pain can put their lives into risk. The aim of the study was to characterize pain control practices in newborns who underwent cardiac surgery. This is a cross-sectional and descriptive study performed in Sociedade Hospital Samaritano of São Paulo. The data were collected from the medical reports of the newborns who underwent cardiac surgery from July 2001 to December 2005. The records related to pain assessment and pharmacological treatment in the first post-operative day were analyzed. From 42 newborns who underwent cardiac surgery during the period studied, 30 of them were in accordance with the criteria of inclusion. Most of the neonates were term and their weights were 2.500 grams or more on the date of the surgery that was mostly performed in the first week of life. In the studied group, it was identified 24 different diagnoses of congenital heart deffects and 14 types of corrective or palliative surgical interventions. From the 30 medical reports, records of pain assessment were found in 24 of them (80.0%). The evaluations taken by using the NIPS were written in 17 medical reports (56.7%) and in 15 of them (50.0%) it was verified records of behavior and physiological parameters alterations that indicate pain. From the newborns evaluated, 17 (56.7%) presented records of pain in the first day post-surgery. 29 neonates (96.7%) received analgesics, as it follows: continuous fentanyl citrate, 24 newborns (80.0%); intermittent dipyrone, 5 (16.7%); continuous fentanyl citrate and intermittent dipyrone, 5 (16.7%) and fentanyl citrate and intermittent morphine, 1 (3.3%). The doses given to the neonates were very different. 12 (40.0%) newborns received the sedative midazolam by continuous infusion with analgesics. 5 (16.7%) neonates received intermittent midazolam, 1 (3.3%) received continuous cisatracurium besylate, 1 (3.3%) received continuous chlorpromazine hydrochloride and 1 (3.3%) received intermittent propofol. The results showed that there is no pattern and uniformity of pain assessment after surgery, neither of the types and doses of drugs used for pain control of the newborns who underwent cardiac surgery in the service that was studied.

Orientador: Claudinei José Gomes Campos / Dissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências Médicas / Made available in DSpace on 2018-08-18T19:37:16Z (GMT). No. of bitstreams: 1 Bueno_GiselliCristinaVillela_M.pdf: 1248064 bytes, checksum: 41025ac97f6190db0bc41ed7c8b46c9d (MD5) Previous issue date: 2011 / Resumo: Com o aumento da longevidade das crianças com cardiopatias congênitas, esta patologia torna-se crônica, e como tal, necessita de um cuidador, que na grande maioria das vezes, é a própria mãe para garantir cuidados específicos em relação à alimentação, atividade física e administração de medicamentos em domicílio. As crenças relacionadas à causa da doença e à manutenção do tratamento constituem parte da herança cultural da família; elas influenciam o modo pelo qual os cuidadores lidam com o problema de saúde e a maneira pela qual respondem aos profissionais de saúde. Alguns modelos teóricos apresentados na literatura dos últimos anos tentam explicar a adoção de determinados comportamentos, dentre eles, destaca-se o Modelo de Crenças em Saúde (MCS). Este trabalho estudou os significados e as crenças atribuídas pelos cuidadores ao tratamento de crianças com cardiopatias congênitas, utilizando o referencial teórico do MCS, desenvolvido por Rosenstock em 1966. Trata-se de um estudo de caso, realizado na unidade de internação de um Hospital Universitário do interior de São Paulo. Participou do estudo oito cuidadoras que acompanhavam crianças portadoras de qualquer tipo de cardiopatia congênita, com até 12 anos de idade incompletos. Foram realizadas entrevistas semidirigidas, gravadas e transcritas para análise dos dados. A escolha das cuidadoras seguiu a técnica de amostragem proposital e a delimitação da amostra se deu através da técnica de saturação teórica. A partir do estudo de cada categoria criada, utilizando a técnica de análise de conteúdo, surgiram outros temas pertinentes que foram divididos em subcategorias não apriorísticas. Os resultados mostraram que os cuidadores só conseguem perceber a doença quando ela se manifesta de alguma maneira; crianças que não apresentam sintomas e que conseguem realizar as atividades do cotidiano pertinentes a sua idade não são consideradas doentes. Os sentimentos de medo, desamparo e impotência emergiram nas falas, porém o medo da morte esteve mais presente nas entrevistas. O facilitador que se destacou durante o tratamento foi a importância que os cuidadores dão ao entendimento e cumprimento das orientações médicas e ao comparecimento às consultas. A fé permeou todas as entrevistas, ela dá força e sustentação para o cuidador acompanhar a criança, superando todas as barreiras para cumprir o tratamento adequadamente. Dentre as barreiras citadas, mas que não influenciaram no seguimento do tratamento destaca-se a ausência da participação do pai no tratamento do filho e as dificuldades de entendimento dos cuidados necessários à criança em domicílio. A falta de citação do enfermeiro nas falas das cuidadoras é um resultado preocupante e emerge discussões necessárias sobre o papel que o enfermeiro está exercendo nas instituições de saúde / Abstract: With increased longevity of children with congenital heart disease, this disease becomes chronic, and as such requires a caregiver, which in most cases, it is the mother to ensure special care in relation to food, activity physical and drug administration at home.The beliefs related to the cause of illness and maintenance treatment are part of the cultural heritage of the family, they influence the way caregivers deal with the health problem and the way they respond to health professionals. Some theoretical models presented in the literature of recent years have tried to explain the adoption of certain behaviors, among which stands out the Health Belief Model (HBM). This study examined the meanings and beliefs attributed by caregivers to treat children with congenital heart disease, using the theoretical framework of the HBM, developed by Rosenstock in 1966. This is a case study, conducted at the inpatient unit of a university hospital in Sao Paulo State. Participated in the study eight caregivers who accompanied children with any type of congenital heart disease, with up to 12 years old incomplete. Semi structured interviews were conducted, recorded and transcribed for data analysis. The choice of caregivers followed the purposive sampling technique and the delimitation of the sample was made through the technique of theoretical saturation. From the study of each category were using the technique of content analysis, there were other relevant issues that were not a priori divided into subcategories. Results showed that caregivers can only perceive the disease as it manifests itself in some way, children who have no symptoms and are able to perform everyday activities relevant to their age are not considered diseased. The feelings of fear, helplessness and powerlessness emerged in the interviews, but the fear of death was present in the interviews. The facilitator pointed out that during treatment was the importance that carers make to the understanding and compliance with medical advice and attend to queries. The faith permeated all the interviews, it gives strength and support for the caregiver accompany the child, overcoming all barriers to follow the treatment properly. Among the barriers identified, but no effect following the treatment there is the lack of father participation in child treatment and difficulties in understanding the necessary care to children in the household. The lack of citation in the speech of the nurse caring is a worrying outcome emerges and necessary discussions on the role that nurses are exercising at health institutions / Mestrado / Enfermagem e Trabalho / Mestre em Enfermagem

Background: The right ventricular outflow tract (RVOT) is known to be the origin of potentially dangerous ventricular tachycardias (VT). Arrhythmias can occur in what are otherwise assume to be structurally normal hearts as well as in arrhythmia syndromes like Brugada syndrome, catecholaminergic polymorphic VT and arrhythmogenic right ventricular dysplasia. In adults with surgically corrected tetralogy of Fallot (ToF), VT often originates in the RVOT. Methods: To investigate potential arrhythmia substrates in the structurally normal heart, the RVOT of adult rats was investigated and compared to the right ventricle (RV). In the human, the right atrium (RA), RV and the RVOT were examined in patients free of electrical and structural disease at the time of clinically indicated surgery on the left heart. The same tissue samples were collected from adults with surgically corrected ToF at the time of pulmonary valve replacement. Real-time reverse transcription quantitative polymerase chain reaction (RT-qPCR) was used to explore changes on the mRNA level. Antibody based techniques (immunohistochemistry and Western blot) and mass spectrometry were used to identify changes on the protein level. In the rat RVOT, fine microelectrodes were employed to investigate electrophysiological characteristics. Results: A reduction in the Na+ channel Nav1.5 was found in the structurally normal RVOT in the rat and human. In the rat, RVOT-nodal like myocytes were identified. Unlike ventricular myocytes, the myocytes did not express the gap junction channel Cx43 and the K+ channel Kir2.1, but they did express the pacemaker Ca2+ channel Cav3.2 and the transcription factor Tbx3. Ectopic pacemaker and nodal-like action potentials with phase 4 depolarization were recorded from the region of the pulmonary valve. In the RA of ToF patients, SERCA2A was found to be downregulated (mRNA and protein) as seen in heart failure and atrial fibrillation. Further widespread remodelling was found in the RA, the RV and RVOT identifying multiple changes as seen in heart failure with downregulation of K+ channels and β-receptors and an increase in inflammatory markers and extracellular matrix molecules. Multiple molecules involved in the mitochondrial respiration chain were found to be downregulated in the RVOT of ToF patients. Conclusion: This is the first study that demonstrates reduction in Nav1.5 in the RVOT of structurally normal human hearts. The changes found in the rat RVOT could explain why arrhythmias originate in the RVOT. The widespread remodelling in ToF, especially in the ECM, could form a substrate for reentry arrhythmias. Mitochondrial dysfunction could point towards potential treatment targets for heart failure in this patient group.

京都大学 / 0048 / 新制・論文博士 / 博士(医学) / 乙第13331号 / 論医博第2199号 / 新制||医||1044(附属図書館) / (主査)教授 湊谷 謙司, 教授 横出 正之, 教授 戸口田 淳也 / 学位規則第4条第2項該当 / Doctor of Medical Science / Kyoto University / DFAM

No description available.

On a daily basis critically ill paediatric patients are admitted in the Paediatric Critical Care Unit (PCCU). Some of these paediatric patients require cardiothoracic surgery and is mechanically ventilated post-operatively. Chapter one of this study gives an orientation to this research and explains that in order to prevent ventilator associated complications and high hospitalisation costs, the mechanically ventilated paediatric patient following cardiothoracic surgery should be extubated as soon as he/she is ready. Chapter two is dedicated to the available literature on this topic and indicates that literature on extubation criteria for the mechanically ventilated paediatric patient is minimal. The methodology of this study is discussed in detail in Chapter three. Chapter four gives a detailed explanation of the research findings and the researcher included the developed clinical pathway for the extubation of the paediatric patient following cardiothoracic surgery in a private hospital in Gauteng. The relevant clinical pathway functions as a guideline and evidence-based tool in the PCCU. Lastly Chapter five gives a summary of this study and a few recommendations are made. The researcher has included a personal reflection in this Chapter. / Dissertation (MCur)--University of Pretoria, 2014. / tm2015 / Nursing Science / MCur / Unrestricted

The ability to generate cardiomyocytes from human induced pluripotent stem cells (hiPSCs) provides unprecedented opportunities in the study and treatment of cardiac diseases. The objective of this dissertation is the development of novel methods of utilizing hiPSC-derived cardiomyocytes (hiPSC-CMs). First, we leveraged the potential of hiPSC-CMs to model congenital heart disease caused by mutations in the transcription factor ZIC3. We developed a method to directly explore the effect of ZIC3 inhibition using hiPSCs at a molecular, cellular, and functional level through utilization of CRISPR interference. Our results identified the role of ZIC3 in regulating Nodal signaling, Wnt signaling, and cell structure and motility processes during cardiac development, suggesting that ZIC3 mutation leads to congenital heart disease in humans by the abnormal regulation of multiple steps during left-right axis establishment. Next, we leveraged the potential of hiPSC-CMs to treat myocardial infarction. We demonstrated that the extended delivery of extracellular vesicles secreted by hiPSC-CMs could attenuate injury and promote recovery of the heart after infarction by regulating apoptosis and inflammatory pathways. These results suggest that hiPSC-CM secreted extracellular vesicles represent a novel cell free tool in the treatment of myocardial infarction and the understanding of heart recovery.