Children with complex heart defects lead sedentary lives that limit involvement in peer activities, impact their growth and development, and jeopardize their long-term health. The goal of this research was to better understand the factors that influence daily moderate-to-vigorous physical activity (MVPA), which is associated with physical and psychological health. The physical activity levels of 64 children (25 female, 5 to 11 years of age) with a single pumping chamber in the heart were measured by accelerometry. Fitness and gross motor skill measures and medical history information were analyzed to identify factors associated with MVPA participation. Increased activity was related to the use of antithrombotic medication, spring season of the year, better motor skill and male sex. Group and individual discussions further explored psychosocial influences on the children’s level of MVPA. The children indicated physical activity was primarily motivated by having fun and being with their friends, while other children being more skilled discouraged participation. Parents of children with complex heart defects had dramatically different perceptions. They believe their child’s activity is primarily influenced by the heart condition and report often feeling uncertain about which activities are appropriate for their child. Finally, sources of parental uncertainty were examined by comparing the physical activity advice provided by the cardiologist to parent reports of the child’s activity restrictions, a content analysis of published activity guidelines and interviews with paediatric cardiologists. Parent uncertainty about activity was supported by the lack of agreement between parent and cardiologist reports of medically necessary activity restrictions. Parent reports of vague or variable activity advice were reflected in the published literature and cardiologist perspectives on activity counselling. These results suggest enabling children with complex heart defects to achieve an active lifestyle may rest on ensuring that the child and parents have appropriate physical activity beliefs and expectations.

Children with complex heart defects lead sedentary lives that limit involvement in peer activities, impact their growth and development, and jeopardize their long-term health. The goal of this research was to better understand the factors that influence daily moderate-to-vigorous physical activity (MVPA), which is associated with physical and psychological health. The physical activity levels of 64 children (25 female, 5 to 11 years of age) with a single pumping chamber in the heart were measured by accelerometry. Fitness and gross motor skill measures and medical history information were analyzed to identify factors associated with MVPA participation. Increased activity was related to the use of antithrombotic medication, spring season of the year, better motor skill and male sex. Group and individual discussions further explored psychosocial influences on the children’s level of MVPA. The children indicated physical activity was primarily motivated by having fun and being with their friends, while other children being more skilled discouraged participation. Parents of children with complex heart defects had dramatically different perceptions. They believe their child’s activity is primarily influenced by the heart condition and report often feeling uncertain about which activities are appropriate for their child. Finally, sources of parental uncertainty were examined by comparing the physical activity advice provided by the cardiologist to parent reports of the child’s activity restrictions, a content analysis of published activity guidelines and interviews with paediatric cardiologists. Parent uncertainty about activity was supported by the lack of agreement between parent and cardiologist reports of medically necessary activity restrictions. Parent reports of vague or variable activity advice were reflected in the published literature and cardiologist perspectives on activity counselling. These results suggest enabling children with complex heart defects to achieve an active lifestyle may rest on ensuring that the child and parents have appropriate physical activity beliefs and expectations.

The treatment of single ventricle anomalies is a formidable challenge for clinical teams caring for patients with congenital heart disease. Those patients fortunate to survive surgical palliation contend with lifelong physical limitations and late stage pathophysiology. A mechanical blood pump specifically designed to increase pressure in the great veins would augment flow through the lungs and provide hemodynamic stability until a donor heart is located. To support the development of such medical devices, this research characterized the fluid dynamics of mechanical assistance in the Fontan circulation by performing numerical analyses and particle image velocimetry (PIV) studies in a patient-specific in vitro model. This project investigated the performance of three pump prototype configurations. ANSYS-CFX was used to conduct the computational studies for a range of operating conditions and degrees of Fontan dysfunction. Pressure generation, blood trauma predictions, shear stresses, fluid streamlines, and velocity profiles were examined. Three-dimensional PIV studies were completed and compared to the numerical estimations. Computational findings and experimental data correlated to within literature expectations. Blood damage levels, shear stresses, and fluid residence times remained reasonable or below threshold limits. The blood pump configurations met expectations by achieving target design specifications for clinical application. The pumps enhanced the rate of hydraulic power gain in the cavopulmonary circuit, reduced inferior vena cava pressure, and minimally increased pulmonary arterial pressure. The blood pump with the twisted protective stent produced the most rapid increase in the rate of power gain and the highest pressure generation. The PIV measurements illustrated a strong dependency of the fluid dynamics on the patient-specific vessel geometry and the particular pump design. The pump having the twisted cage outperformed the other designs and had a dominating impact on the blood flow distribution in the cavopulmonary circuit. A strong rotational component in the flow was observed leaving the pumps. These results confirm that mechanical cavopulmonary assistance is a viable therapeutic option. Significant knowledge into a new class of blood pumps and how these pumps interact with a single ventricle physiology was gained, thus advancing the state-of-the-art in mechanical circulatory support and addressing a significant human health problem.

Few therapeutic alternatives exist for patients with a failing single ventricle physiology. To address this need, this thesis project investigated two new therapeutic alternatives, which sought to positively augment the Fontan hemodynamics. The first modality introduced a non-invasive method of external pressure application to the lower extremities. A clinical study (n=2) was conducted, and results indicated an increase in flow as a consequence to an increase in transmural pressure in the lower extremities. The second modality investigated a minimally invasive blood pump. Numerical analyses of the pump were performed to examine hydraulic performance under physiologic conditions. The pump produced pressure rises of 1 to 25 mmHg over flows of 1 to 4 LPM, has a blood damage index less than 1% and was also found to successfully augment the hydraulic energy of the Fontan physiology. This work resulted in substantial progress to develop both modalities and address a significant human health problem.

Thèse (M.Sc.)--Université Laval, 2004. / Bibliogr. Publié aussi en version électronique.

Congenital heart disease (CHD) is the most common congenital anomaly, with single ventricle (SV) defects accounting for nearly 10% of all CHD. SV defects tend to be the most severe forms of CHD: all patients born with SV require multiple open heart surgeries, often beginning in the neonatal period, ultimately leading to the Fontan procedure. Due to improvements in surgical procedures and medical care, more patients are surviving into adolescence and adulthood. Brain imaging and pathology studies have shown that patients with SV have differences in brain structure and metabolism even before the first surgery, and as early as in utero. Furthermore, a significant number of patients have new or more severe lesions after the initial surgery, and many still have brain abnormalities into early childhood. However, there are no detailed brain structural data of SV patients in adolescence. Our group recruited a large cohort of post-Fontan SV patients aged 10-19 years. Separate analyses of neuropsychological and behavioral outcomes in these patients show deficits in multiple areas of cognition, increased rates of attention deficit-hyperactivity disorder (ADHD), and increased use of remedial and/or special education services compared to a control group. Post-Fontan adolescents have more gross brain abnormalities, including evidence of chronic ischemic stroke. Furthermore, there are widespread reductions in cortical and subcortical gray matter volume and cortical thickness, some of which are associated with medical and surgical variables. Diffusion tensor imaging (DTI) analyses show widespread areas of altered white matter microstructure in deep subcortical and cerebellar white matter. In this dissertation, I use graph theory methods to characterize structural connectivity based on gray matter (cortical thickness covariance) and white matter (DTI tractography), and examine associations between brain structure and neurodevelopment. I found that brain network connectivity differs in post-Fontan patients compared with controls, both at the global and regional level. Additionally, deficits in overall network structure were associated with impaired neurodevelopment in several domains, including general intelligence, executive function, and visuospatial skills. These data suggest that early neuroprotection should be a major focus in the care of SV patients, with the goal of improving long-term neurodevelopmental outcomes.

No description available.

Im Rahmen einer vergleichenden retrospektiven Analyse wurden die präoperativen und intraoperativen Einflussfaktoren auf den frühen und mittelfristigen postoperativen Verlauf nach Fontan-Operation untersucht und die Ergebnisse der totalen cavopulmonale Anastomose mit einem intraatrialen lateralen Tunnel (LTFO, n = 25) und mit einem extrakardialen Kunststoff-Konduit (ECFO, n = 25) gegenüber gestellt. Postoperativ wurde nach ECFO ein komplikationsärmerer früher Verlauf mit kürzerer maschineller Beatmung und signifikant geringerer Inzidenz für frühe und mittelfristige Arrhythmien als nach LTFO beobachtet. Im frühen postoperativen Verlauf waren die schnellste Extubation und Entlassung bei den ECFO-Patienten möglich, bei denen keine Kardioplegie verwendet wurde. Unabhängige Risikofaktoren nach multivariater Analyse waren die Dauer der Kardioplegie in Bezug zu den unmittelbaren postoperativen Komplikationen, eine LTFO - sowohl für Tachy- als auch für Bradyarrhythmien - und die Inzision in die cavoatriale Verbindung bei LTFO in Bezug zu Bradyarrhythmien mit der Notwendigkeit einer Schrittmacherimplantation. Durch Vermeidung von Kardioplegie und chirurgischer Schaffung eines lateralen Tunnels sowie Verkürzung des kardiopulmonalen Bypasses bei ECFO werden vor allem die postoperativen Arrhythmien reduziert und es ist wahrscheinlich möglich, den postoperativen Verlauf bei Patienten mit einem durch eine langwierige Zyanose und durch Volumenbelastung vorbeschädigten singulären Ventrikel zu verbessern. Eine prospektive langfristige Vergleichsstudie mit einer größeren Patientenzahl ist notwendig, um die Frage zu beantworten, ob eine signifikante Minderung der spätpostoperativen Komplikationen und eine Verbesserung der Lebenserwartung der Patienten mit univentrikulärer Physiologie durch eine korrekt geplante und optimal durchgeführte extrakardiale Modifikationen der Fontan-Operation erreicht werden kann. / The preoperative and intraoperative risk factors for early and mid-term postoperative course after Fontan operation were analyzed and results after total cavopulmonary connection with lateral intraatrial tunnel (LTFO, n = 25) and with extracardiac conduit (ECFO, n = 25) were compared retrospectively. The early postoperative course after ECFO was better with shorter duration of mechanical ventilation. Lower incidence of arrhythmias was observed after ECFO compared to LTFO both early postoperatively and during mid-term follow-up. Patients after ECFO, in whom cardioplegia was not used intraoperatively, had the shortest duration of mechanical ventilation and of the hospital stay. Multivariate analysis revealed duration of cardioplegia as an independent risk factor for early postoperative complications, LTFO as a risk factor for tachyarrhythmias and incision into the cavoatrial junction during LTFO as a risk factor for the bradyarrhythmias requiring pacemaker implantation. With the avoidance of cardioplegia and intracardiac surgery and shorter cardiopulmonary bypass times during ECFO, postoperative arrhythmias could be reduced. Further, the early postoperative course in patients with univentricular physiology who have had cyanosis and ventricular volume overload for a long time seems to be better after extracardiac modification of Fontan operation. To investigate whether late complications are reduced by optimal extracardiac Fontan operation and whether life expectancy in patients with single ventricle is improved, long-term prospective comparative follow-up studies are needed.

Introdução: Pacientes com cardiopatia congênita submetidos ao procedimento de Fontan (PF) vêm atingindo a idade adulta com significativas consequências sistêmicas, particularmente hepáticas. Tais injúrias hepáticas podem resultar em fibrose, cirrose e nódulos hepáticos (NH), que podem ser benignos ou malignos. Porém, não há, até o momento, consenso na literatura quanto ao rastreamento desses nódulos, sobretudo acerca do início e da melhor modalidade para tal fim. Objetivo: Esse estudo objetivou (a) avaliar a frequência de NH em pacientes submetidos ao PF na ultrassonografia (USG), tomografia computadorizada (TC) e ressonância magnética (RM), assim como a concordância entre tais métodos; (b) investigar se há correlação entre a presença de NH e algumas variáveis clínicas e laboratoriais; (c) analisar se há diferença nos valores de rigidez hepática utilizando a elastografia por USG por meio da técnica acoustic radiation force impulse (ARFI) entre os pacientes com e sem NH. Métodos: Foram recrutados, prospectivamente, 49 pacientes submetidos ao PF entre agosto de 2014 e junho de 2016. Esses pacientes foram submetidos a rastreamento clínico e laboratorial de hepatopatia, e elastografia hepática por USG com ARFI, TC e RM. A concordância entre os testes foi acessada utilizando o kappa de Cohen. A correlação entre NH com as outras variáveis foi realizada com teste t de Student ou teste de Mann-Whitney para variáveis contínuas sem distribuição normal e teste qui-quadrado ou teste de Fisher para variáveis categóricas. Resultados: NH foram detectados em 3/49 (6%), 14/44 (31,8%) e 19/48 (39,6%) pacientes na USG, TC e RM, respectivamente. Houve uma concordância quase perfeita entre TC e RM na detecção de NH (kappa: 0,849, p < 0,001), porém a USG não demonstrou concordância com TC e RM (kappa: 0,006, p=0,095 e kappa: 0,083, p = 0,032, respectivamente). Nenhuma variável clínica ou laboratorial apresentou correlação significativa com a presença de NH, inclusive o tempo após o PF. Os valores de rigidez hepática no ARFI foram significativamente mais elevados nos pacientes com NH (2,64 ± 0,81 m/s vs. 1,94 ± 0,49 m/s; p=0,002) e foram um preditor significativo para NH (AUC: 0,767, p=0,002). Conclusão: Na nossa população, mais de um terço dos pacientes após o PF apresentou NH na TC ou RM, mas a USG não detectou a grande maioria dos nódulos. Nenhum dado clínico ou laboratorial apresentou correlação significativa com a presença de NH. A rigidez hepática no ARFI foi significativamente mais elevada nos pacientes com NH. Tais achados sugerem que a USG não é um método diagnóstico efetivo no rastreamento de NH, porém a elastografia por ARFI pode ser útil, orientando quais pacientes devem submetidos à TC ou RM / Background: Patients with congenital heart disease after Fontan procedure (FP) are reaching the adulthood with significant systemic consequences, particularly to the liver. Those hepatic injuries can cause liver fibrosis, cirrhosis and hepatic nodules (HN), which can be benign or malignant. Currently, there is no consensus in the literature regarding screening of HN in patients after FP concerning when to start and which is the best imaging modality indicated for it. Purpose: This study aimed (a) to evaluate the frequency of HN in patients after FP on ultrasound (US), computed tomography (CT) and magnetic resonance imaging (MRI), as well as their inter-test agreement; (b) to investigate if there is any correlation between presence of HN and clinical or laboratorial variables; (c) to analyze if liver stiffness (LS) values using acoustic radiation force impulse (ARFI) on US elastography differ between patients with and without HN. Methods: We prospectively recruited 49 patients after FP from August 2014 to June 2016. These patients underwent clinical and laboratorial screening of hepatic disorders, ARFI elastography of the liver, abdominal US, CT and MRI. Intertest agreement was assessed by using weighted Cohen\'s kappa as statistics. The dependence of HN with the variables was performed using Student\'s t test or ANOVA for independent continuous variables without normal distribution; and chi-square test or Fisher\'s test for categorical variables. Results: HN were detected in 3/49 (6%), 14/44 (31.8%), and 19/48 (39.6%) patients on US, CT and MRI, respectively. There was an almost perfect agreement between CT and MRI in detecting HN (kappa: 0.849, p < 0.001); however, US had a non-significant correlation with CT and MRI (kappa: 0.006, p=0.095 and kappa: 0.083, p = 0.032, respectively). No clinical or laboratorial data had any significant correlation with the presence of HN, including time since FP. LS on ARFI was significantly higher in patients with HN (2.64 ± 0.81 m/s vs. 1.94 ± 0.49 m/s; p=0.002) and was a significant predictor of HN (AUC 0.767, p=0.002). Conclusion: In our study, more than one-third of patients after FP had HN on CT or MRI, but US did not detect the vast majority of them. No clinical or laboratorial data had any significant correlation with the presence of HN. LS on ARFI was significantly higher in patients with HN. These findings may suggest that US is not an effective imaging modality for screening of HN; however, ARFI elastography may help guiding which patients should be further imaged with CT or MRI

This thesis project examined the interactive fluid dynamics between a blood pump and the univentricular Fontan circulation. 2-D particle image velocimetry (PIV) measurements were conducted on an idealized total cavopulmonary connection (TCPC) with an axial pump prototype in the inferior vena cava (IVC). Fluid velocity profiles were examined under various physiologic conditions for Fontan patients. The velocity profiles for all cases demonstrated the shunting of flow from the IVC toward the right pulmonary artery. A rotational component in the pump outflow was observed forcing flow to the periphery as compared to the flow profile without a pump present in the IVC. The inclusion of the pump provides a pressure rise of 3 to 9 mmHg. These results demonstrate the ability of the intravascular blood pump to support a Fontan circulation and support the continued optimization and development of the pump.