Single ventricle heart defects are a rare but serious form of congenital heart disease, which affect approximately 2000 children born in the United States each year. Staged surgical palliation, culminating with the “Fontan Procedure,” is typically required to achieve adequate supply of blood to both the pulmonary and systemic circulations while avoiding chronic ventricular volume overload. This surgery reroutes the systemic veins to the pulmonary arteries, forming a total cavopulmonary connection (TCPC), to completely bypass the right side of the heart and restore a series configuration to the cardiovascular circuits. Despite improved survival through this operative course in first weeks and years of life, Fontan patients are subjected gradual attrition and decreased life expectancy through a multitude of chronic complications. It is suspected that the adverse hemodynamics of this surgically altered physiology, including those specific to the surgical TCPC, play a role in determining patient outcome. However, the small and heterogeneous patient population has hindered decisive progress and there is still not a good understanding of the optimal care strategies on a patient-by-patient basis. In recent decades, advances in medical imaging and image-based computational fluid dynamics (CFD) have redefined the realm of possibility for studying complex biomedical phenomena. Combined, these methods provide the means to create and evaluate patient-specific models of a wide range of cardiovascular structures, including the TCPC, with high fidelity. Results from these models can then be used for a wide array of different analyses, such as identifying regions of flow separation or stagnation, calculating hemodynamic power loss, or quantifying local flow distribution patterns. Through significant effort from numerous past investigators, a robust set of validated computational and image processing tools has been assembled, along with the largest library of cardiac magnetic resonance (CMR) data of TCPC anatomy and flow. These tools are leveraged in this thesis to characterize the functional implications of TCPC power loss at an unprecedented scale: we report the largest CFD analysis of patient-specific TCPC hemodynamics to date with particular focus on identifying functional correlates. Combining these data with imaging-based analysis of ventricle function, we directly compare the CFD-derived hemodynamics to the performance of the single ventricle for the first time. Motivated by the physiologic significance of these findings, the same patient-specific CFD framework is used for the translational application of prospective surgery planning for hemodynamic optimization, including the first implementation of a novel TCPC connection design hypothesized to uniquely streamline the energetic performance. We conclude with a first look at the longitudinal evolution of patient functional status to begin understanding how factors such as TCPC hemodynamics contribute to poor long-term performance in these patients.

Congenital heart disease is the most common birth defect globally, affecting both children and their families. Twenty –five percent of children experiencing a CHD birth defect are diagnosed with complex CHD (cCHD), signifying critical heart dysfunction requiring one or more open-heart surgeries during the first year of life. With medical advances, cCHD survival rates have almost tripled in the last three decades. This has resulted in an increase in the number of morbidities associated with cCHD, which is drastically impacting the need to support quality of life outcomes for a child with cCHD and their family. The two most prevalent unaddressed risks for quality of life outcomes in the cCHD population are child and caregiver mental health and child's neurodevelopmental disabilities. Congenital heart disease is the most common birth defect globally, affecting both children and their families. Twenty-five percent of children experiencing a CHD birth defect are diagnosed with complex CHD (cCHD), signifying critical heart dysfunction requiring one or more open-heart surgeries during the first year of life. With medical advances, cCHD survival rates have almost tripled in the last three decades. This has resulted in an increase in the number of morbidities associated with cCHD, which is drastically impacting the need to support quality of life outcomes for a child with cCHD and their family. The two most prevalent unaddressed risks for quality of life outcomes in the cCHD population are child and caregiver mental health and child's neurodevelopmental disabilities. The present study sought to address the relationship between caregiver knowledge of cCHD developmental challenges (i.e., outcomes related to neurodevelopmental disabilities, mental health, and provider quality-care approach) and children's and caregiver's quality of life outcomes, inclusive of academic functioning ability of children with cCHD, children with cCHD and their caregivers' mental health functioning, and the overall satisfaction with the nature of the healthcare provider of the child with cCHD. A total sample size of N = 46 participants were included in the current study. Results that caregivers' knowledge of cCHD risks to quality of life outcomes explained a much greater percentage of the variance in caregiver satisfaction with healthcare providers (R2 = 0.350, p < 0.001) compared to number of surgical interventions (R2 = 0.058, p = 0.047). Clinical implications and implementation for use of a holistic, integrated approach are strengthened by the study findings.

No description available.

Este estudo teve como objetivo realizar a validação clínica do diagnóstico de enfermagem da North American Nursing Diagnoses Association (NANDA, 2006) Desobstrução ineficaz de vias aéreas de crianças e adolescentes submetidos à correção cirúrgica de cardiopatia congênita, em um hospital de ensino público de nível terciário da cidade de Ribeirão Preto-SP. O projeto teve aprovação do Comitê de Ética e Pesquisa da referida instituição, foram incluídos os profissionais que aquiesceram em participar do estudo e os sujeitos cujos responsáveis autorizaram. Adotou-se o modelo de validação clínica proposto por Hoskins (1989) que inclui as seguintes etapas: análise de conceito, validação por especialistas e validação clínica. Após ampla revisão da literatura foram descritos os conceitos relacionados a cada uma das características definidoras, em seguida o diagnóstico foi analisado por 40 enfermeiros considerados peritos, segundo a pontuação de Fehring (1994). A adequação do título e da definição do diagnóstico em estudo foi considerada adequada por 70% dos peritos. Em relação às 13 características definidoras propostas pela NANDA (2006), 04 receberam, dos peritos, escores considerados maiores, 07 menores e 02 obtiveram escores abaixo de 0,50, ou seja, avaliadas como não indicativas para o diagnóstico desobstrução ineficaz das vias aéreas. Procedeu-se a validação clínica por duas enfermeiras peritas, que analisaram a presença do diagnóstico e as respectivas características definidoras em 50 crianças e adolescentes. Em relação a presença do diagnóstico na clientela estudada houve a concordância de 97,7% dos enfermeiros peritos. A única característica definidora que obteve o coeficiente de confiabilidade segundo os critérios de Fehring foi tosse ausente; pesar de apenas esta característica definidora estar presente, foi suficiente para confirmar a presença deste diagnóstico na população estudada. Por se tratar de uma população específica e em condições de pós-operatório imediato, ou seja, 60% dos sujeitos estavam sob efeitos de anestésicos no momento da validação clínica, torna-se necessário o desenvolvimento de estudos futuros que possam validar o diagnóstico Desobstrução ineficaz de vias aéreas em crianças e adolescentes em outras situações clínicas. Porém, a identificação deste diagnóstico em crianças e adolescentes submetidos à correção cirúrgica de cardiopatia congênita poderá contribuir para a elaboração de um plano de assistência de enfermagem de qualidade, uma vez que se não atendido prontamente pode levar os indivíduos a sérias complicações. / This study aimed to accomplish the clinical validation of the nursing diagnoses of the North American Nursing Diagnoses Association (NANDA, 2006) Ineffective airway clearance of children and teenagers who underwent surgical correction of congenital heart diseases at a public teaching hospital, working with complex diagnosis and treatments, in the city of Ribeirão Preto. The project has been approved by the Ethics and Research Committee of the referred institution. It has been included the professionals that wanted to participate in the study and subjects whose responsible have authorized. It has been adopted the clinical validation model proposed by Hoskins (1989) which includes the following stages: concept analysis, validation by specialist and clinical validation. After an ample literature review it has been described the concepts related to each one of the defining characteristics, afterwards the diagnoses was analyzed by 40 nurses who are considered to be experts, according to the Fehring scoring (1994). The adaptation of the title and the definition of the diagnoses under study were considered adequate by 70% of the experts. In relation to the 13 defining characteristics proposed by NANDA (2006), four received scores that were considered higher by experts, seven were considered lower and two scores were below 0,50 that is, they were assessed as not indicative for diagnosis of ineffective airway clearance. The clinical validation was carried out by two expert nurses, who analyzed the presence of the diagnoses and the respective defining characteristics in 50 children and teenagers. In relation to the presence of the diagnosis on the studied patients, there was the agreement of 97,7% of the expert nurses. The only defining characteristic which achieved the coefficient of reliability according to the Fehring criteria was absence of cough. Although it was the only defining characteristic present, it was sufficient to confirm the presence of this diagnosis on the studied population. Because of the characteristics of being a specific population, under condition of immediate post operative, that is 60% of the subjects were under anesthetic effect at the moment of clinical validation, it is necessary the development of future studies that can validate the diagnosis Ineffective airway clearance in children and teenagers in other clinical situations. However the identification of this diagnosis in children and teenagers who underwent surgical correction for congenital heart disease will be able to contribute to the elaboration of a quality assistance program in nursing, once that if the individual is not promptly assisted there can be serious complications to the patients.

The field of structural design optimization is one with great breadth and depth in many engineering applications. From the perspective of a designer, three distinct numerical methodologies may be employed. These include size, shape, and topology optimization, in which the ordering typically (but not always) corresponds to the order of increasing complexity and computational expense. This, of course, depends on the particular problem of interest and the selected numerical methods. The primary focus of this research employs density-based topology optimization with the goal of improving structural resistance to failure. Beginning with brittle fracture, two topology optimization based formulations are proposed in which low weight designs are achieved with substantially increased fracture resistance. In contrast to the majority of the current relevant literature which favors stress constraints with linear elastic physics, we explicitly simulate brittle fracture using the phase field method during the topology optimization procedure. In the second formulation, a direct comparison is made against results obtained using conventional stress-constrained topology optimization and the improved performance is numerically demonstrated. Multiple enhancements are proposed including a numerical efficiency gain based on the Schur-complement during the analytical sensitivity analysis and a new function which provides additional path information to the optimizer, making the gradient-based optimization problem more tractable in the presence of brittle fracture physics. Subsequently, design for ductile failure and buckling resistance is addressed and a numerically efficient topology optimization formulation is proposed which may provide significant design improvements when ductile materials are used and extreme loading situations are anticipated. The proposed scheme is examined regarding its impact on both the peak load carrying capacity of the structure and the amount of external work required to achieve this peak load, past which the structure may no longer be able to support any increase in the external force. The optimized structures are also subjected to a post-optimization verification step in which a large deformation phase field fracture model is used to numerically compare the performance of each design. Significant gains in structural strength and toughness are demonstrated using the proposed framework. Additionally, the failure behavior of 3D-printed polymer composites is investigated, both numerically and experimentally. A large deformation phase field fracture model is derived under the assumption of plane-stress for numerical efficiency. Experimental results are compared to numerical simulations for a composite system consisting of three stiff circular inclusions embedded into a soft matrix. In particular, we examine how geometric parameters, such as the distances between inclusions and the length of initial notches affect the failure pattern in the soft composites. It is shown that the mechanical performance of the system (e.g. strength and toughness) can be tuned through selection of the inclusion positions which offers useful insight for material design. Finally, a size optimization technique for a cardiovascular stent is proposed with application to a balloon expandable prosthetic heart valve intended for the pediatric population born with Congenital Heart Disease (CHD). Multiple open heart surgical procedures are typically required in order to replace the original diseased valve and subsequent prosthetic valves with those of larger diameter as the patient grows. Most expandable prosthetic heart valves currently in development to resolve this issue do not incorporate a corresponding expandable conduit that is typically required in a neonate without a sufficiently long Right Ventricular Outflow Tract (RVOT). Within the context of a particular design, a numerical methodology is proposed for designing a metallic stent incorporated into the conduit between layers of polymeric glue. A multiobjective optimization problem is solved, not only to resist the retractive forces of the glue layers, but also to ensure the durability of the stent both during expansion and while subject to the anticipated high cycle fatigue loading. It is demonstrated that the surrogate-based optimization strategy is effective for understanding the trade-offs between each performance metric and ultimately efficiently arriving at a single optimized design candidate. Finally, it is shown that the desired expandability of the device from 12mm to 16mm inner diameter is achievable, effectively eliminating at least one open heart surgical procedure for certain children born with CHD.

Thesis (M. Med.(Paediatrics and Child Health)) -- University of Limpopo, 2019 / Background: Congenital heart disease (CHD) is a significant contributor to Under 5 Mortality rate(U5MR) in Limpopo. Atrioventricular septal defect (AVSD) is the best ascertained lesion in Limpopo and is strongly associated with Down syndrome. Few children from Limpopo with CHD including AVSD access cardiac diagnostic and surgical services. Objectives: The study aimed to enumerate, describe syndromes associated with AVSD and outcomes of children with AVSD at Pietersburg hospital. Methods: This is a Retrospective study of all children (n=80) diagnosed with AVSD from 1 January 2010 to 31 December 2014 at Pietersburg hospital. Data were drawn from echocardiogram reports and patient records. District Health Information Software (DHIS) data was used to obtain the number of live births per district during the 5 years study period. Results: Eight hundred and sixty six (n=866) patients had a first diagnosis of CHD confirmed on echocardiography and 80 (9.2%) of these had AVSD (an estimated 31.5% of expected cases of AVSD). Eighty four per cent (84%) of AVSD patients were associated with Down syndrome. 42/67 (63%) AVSD patients were referred for surgical assessment and of those 15/42 (36%) had surgery. The median interval between diagnosis and surgery was 13 months. Seventy five percent (n=50/67) of patients defaulted follow up. Conclusion: The study confirmed that most children with AVSD had associated Down syndrome and that the majority of children with AVSD from Limpopo do no access surgery. There is under referral of children with Down syndrome for screening of CHD.

The Hybrid Norwood (HN) is a relatively new first stage procedure for neonates with Hypoplastic Left Heart Syndrome (HLHS), in which a sustainable univentricular circulation is established in a less invasive manner than with the standard procedure. A computational multiscale model of such HLHS circulation following the HN procedure was used to obtain detailed hemodynamics. Implementation of a reverse-BT shunt (RBTS), a synthetic bypass from the main pulmonary to the innominate artery placed to counteract aortic arch stenosis, and its effects on local and global hemodynamics were studied. A synthetic and a 3D reconstructed, patient derived anatomy after the HN procedure were utilized, with varying degrees of distal arch obstruction, or stenosis, (nominal and 90% reduction in lumen) and varying RBTS diameters (3.0, 3.5, 4.0 mm). A closed lumped parameter model (LPM) for the peripheral or distal circulation coupled to a 3D Computational Fluid Dynamics (CFD) model that allows detailed description of the local hemodynamics was created for each anatomy. The implementation of the RBTS in any of the chosen diameters under severe stenosis resulted in a restoration of arterial perfusion to near-nominal levels. Shunt flow velocity, vorticity, and overall wall shear stress levels are inverse functions of shunt diameter, while shunt perfusion and systemic oxygen delivery correlates positively with diameter. No correlation of shunt diameter with helicity was recorded. In the setting of the hybrid Norwood circulation, our results suggest: (1) the 4.0mm RBTS may be more thrombogenic when implemented in the absence of severe arch stenosis and (2) the 3.0mm and 3.5mm RBTS may be a more suitable alternative, with preference to the latter since it provides similar hemodynamics at lower levels of wall shear stress.

Denna uppsats behandlar interaktioner i ett diskussionsforum på Familjeliv.se, där deltagarna är föräldrar eller blivande föräldrar till barn eller foster med ett medfött hjärtfel. Syftet med studien är att undersöka hur detta forum kan fungera som en kunskapsresurs och en plats där deltagarna förhandlar sig fram till gemensamma förståelser om till exempel hjärtfelsdiagnosen och livet med ett medfött hjärtfel. Genom analyser av dels de inledande presentationerna från deltagare som positionerar sig som nya, dels av de diskussioner som följer på dessa presentationer fokuserar studien legitimitet i två bemärkelser: legitimiteten i medlemskapet och legitimeringar i interaktionerna som ingår i kunskapsförhandlingen och bygger upp gemensamma förståelser. Resultaten från studien visar inledningsvis att det finns tydligt återkommande drag i presentationerna, något som tyder på att det finns sociala normer och överenskommelser för vilka forumet är till för, hur deltagandet är uppbyggt och vad som utgör ett legitimt medlemskap. Därefter visar analyserna av de efterföljande diskussionerna bland annat hur deltagarna tydligt orienterar sig kring vad som är legitimt att säga och på vilka grunder, där exempelvis det egna barnets diagnos och erfarenheterna från denna verkar vara avgörande. Detta skisserar ut en bild av forumets funktion och visar på en medvetenhet hos deltagarna vad gäller vad de kan bidra med i förhållande till vården. Analyserna visar även hur interaktionerna är uppenbart flerröstade och rymmer såväl professionell expertis, i form av rekontextualiserade samtal med vårdpersonal, som rekontextualiseringar av deltagarnas egna erfarenheter av hjärtfelen. Genomgående innebär forumet också ett jämförelsematerial där deltagarnas rekontextualiseringar tillsammans bygger en bild av hur saker och ting förhåller sig, som sedan kan appliceras på enskilda deltagares situation. Studien indikerar därmed att forumet kan fungera som en intern kunskapsbank. / This thesis investigates the interaction on an online discussion forum, where the participants are parents or expectant parents of children with congenital heart defects. The aim of the study is to shed light on how this forum can function as a source of knowledge and a place where joint understandings about for example the diagnosis and the life with this medical condition are negotiated in interaction. Analyzing both the initial presentations written by newcomers in the forum, and the discussions following these presentations, the study draws on the concept of legitimacy in two ways: the legitimacy in relation to the forum membership and the legitimations that are used to construct a shared understanding between the participants. The results from the study indicate that there are recurring patterns in the presentations written by new members when introducing themselves in the forum threads. This in turn suggests that there are social norms regarding which participants the forum is directed towards, how the participation is organized and what constitutes a legitimate membership in the community. In addition, a key result from the analysis of the interactions following these presentations is the participants’ navigation of what information is legitimate in which situation, where the own child’s diagnosis and type of heart defect is of great importance. This shows awareness among the participants regarding the nature of the forum’s contribution in relation to healthcare professionals. Furthermore, the analysis also shows how the interactions are strikingly multi-voiced, in that the participants through the discursive process of recontextualization make use of both previous conversations with doctors and other medical professionals, as well as their own experience of the children’s heart defects. The forum then serves as a comparison where these recontextualizations are used to negotiate a joint understanding, which subsequently can be applied to the participants’ individual storylines. The study therefore indicates that the forum can function as a resource of knowledge.

A descriptive design was used in this study to describe the feeding behaviors (as defined by changes in body position) in infants with congestive heart failure secondary to congenital heart disease. In addition, short term physiologic changes associated with the behaviors were measured through the use of pulse oximetry and cardiorespiratory monitors. Nine infants were observed in this study as they were being fed by their nurses. The method chosen was naturalistic observation. Descriptive statistics were used to analyze the demographic data and the measured physiologic variables. A point by serial correlation was conducted to describe any relationships between the position changes and the measured physiologic variables. Statistically significant relationships were found between certain positions and oxygen saturation, heart rate and respiratory rate. The results indicate that infants do experience position changes that are accompanied by changes in physiologic variables during feeding. These position changes can be categorized as infant-mediated or nurse-mediated.

Background and aims: Many adults with congenitally malformed hearts are at risk for complications such as decreased function and capacity of the heart due to the heart defect and previously surgery. This advocates self-management behaviours related to medical treatments, physical activity, preventions of endocarditis, some restrictions regarding suitable employment and spare time activities, birth control and pregnancy, but also lifestyle concerns such as refraining from smoking and healthy eating. Sufficient knowledge and support are requirements for successful self-management. The overall aim of this thesis was to describe educational needs, develop a tool for assessing knowledge and to evaluate the effects of a follow-up model providing education and psychosocial support to adults with congenitally malformed hearts. Subjects and methods: Adults (≥18 years of age) with the ten most common heart defects namely ventricular septal defect, atrial septal defect, coarctation of the aortae, aortic valve stenosis (defined as uncomplicated heart defects) and tetralogy of Fallot, complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Ebstein anomaly and Eisenmenger syndrome (defined as complicated heart defects) were included in the studies. To apprehend the educational needs (I), sixteen adults with heart malformations, ranging from 19-55 years of age, were interviewed and data were analysed qualitatively using phenomenographic method. As a tool to evaluate knowledge, an instrument named Knowledge scale for adults with Congenital Malformed Hearts (KnoCoMH) was developed and psychometrically evaluated (II) in 19 + 114 adults with the ten most common heart defects average age 34 ± 13.5. A model for follow-up was described and initially evaluated (III) by 55 adults with the most common heart defects and finally tested in a randomised controlled trial (IV) with a total of 114 adults with congenitally malformed hearts (56 participants in intervention group and 58 in control group with average age 34 ± 13.5). The intervention group recived a model for follow-up with individualise and computer-based eduction and psychosocial support by a multidisciplinary team. Results: Two-way communication when given information was found to be crucial in order to enhance knowledge (I). Knowledge was seen as a tool for managing important areas in life. The KnoCoMH (II) was found to be a valid and reliable scale and can now be used to estimate knowledge in adults with congenitally malformed hearts. The model for follow-up (III) was effective in improving and maintaining knowledge (IV) about self-management in adults with heart malformation.