The body composition of children and adolescents with congenital heart disease and residual pulmonary regurgitation (PR) was compared to that of healthy age- and sex-matched controls. Testing included height, weight, skinfolds, skeletal widths, circumferences, bio-electrical impedance (for estimation of total body water from resistance index), hydrostatic weighing, bone mineral content from single photon absorptiometry, and an assessment of maturational status. Activity levels were assessed by questionnaires and an accelerometer. The two groups were found to be different in height, skeletal widths, bone mineral content, bone mineral index and total body water determined by bio-electrical impedance. After adjusting the data for height differences, the groups were different for skeletal widths and bone mineral index. The PR and control subjects had similar skinfolds and circumferences, as well as percent fat determined by body density, body water and bone mineral content.

published_or_final_version / Sports Science / Master / Master of Science in Sports Science

Mothers who have an infant with severe congenital heart disease (CHD) face an uncertain and emotionally challenging postpartum period as their baby is hospitalised and undergoes life-saving cardiac surgical treatment. There are many potential risk factors to mother-infant bonding, that is, the emotional tie a mother develops with her baby, in the context of infant illness. Having an infant with a diagnosis of severe CHD could be seen as a threat to the mother's experience of bonding. However, there is limited understanding about the maternal perception of bonding with an infant with severe CHD. This study aimed to explore mothers' bonding with their infant with severe CHD throughout antenatal, perinatal and postnatal periods and how they coped with any challenges to this bond. Interviews were conducted with eight mothers of infants aged between eight and fifteen months with severe CHD, who were recruited from a children's hospital and who were diagnosed either antenatally or postnatally. Interpretative Phenomenological Analysis was used to identify themes across the mothers' accounts. Four superordinant themes were identified: ‘An Emotional Start to Motherhood and the Mother-Infant Bond', ‘Losing Control in the Context of CHD', ‘Keeping Connected to the Baby' and ‘Moving on Together'. The findings identify mother-infant bonding as a process that can withstand challenges such as maternal-infant separation, potential loss of the infant and maternal feelings of disconnection from the baby. Practical strategies were used by mothers to maintain their bond with their infant following diagnosis and during hospitalisation. These included being close to their infant and taking over caregiving duties from the nurses. Mothers described strength and resilience from the experience and a process of increasingly feeling closer to their infant. Several potential research implications and clinical recommendations for healthcare professionals are suggested.

Congenital heart disease (CHD) includes a variety of disorders that are characterized by structural defects to the heart or the coronary blood vessels that occur in fetal development. CHD occurs in 8 of every 1,000 live births. CHD often requires surgical repair and increases caregiving burden for families. The purpose of this study was to better understand the relations between illness-related parenting stress, coping resources, and psychological functioning in primary caregivers of young children with CHD. 69 parents provided demographic information and completed measures of parenting stress, self-efficacy, mindfulness, social support, and adjustment. Results revealed that psychological functioning in this sample is comparable to other chronic illness populations. In regression analyses, illness-related parenting stress was positively related and mindfulness was negatively related to psychological distress.

En litteraturbaserad studie har gjorts med analys av nio kvalitativa studier från 2005-2016. Idag överlever fler patienter med medfött hjärtfel tack vare tidig diagnostisering och förbättrade behandlingsmöjligheter. För unga vuxna mellan 13-39 år finns skilda meningar om hur de upplever sitt medfödda hjärtfel. Ett medfött hjärtfel uppstår redan i fosterstadiet, då hjärtat utvecklas. Ungefär en procent av världens barn föds varje dag med ett medfött hjärtfel. En tredjedel av hjärtfelen är varken i behov av operation eller behandling. Hjärtfelet kan förändras över tid vilket gör att uppföljning under livets gång är viktig. Tidigare har det inte varit möjligt för unga vuxna med medfött hjärtfel att växa upp, vilket gör att vuxensjukvården fått en ny patientgrupp. Det gör att det saknas kunskap om vad som kommer vänta de unga vuxna i framtiden. Dessutom har allmänsjuksköterskan inte en traditionell utbildning inom området, trots det kan allmänsjuksköterskan möta den här patientgruppen i sjukvårdens alla vårdformer. För att kunna bemöta unga vuxna på bästa sätt krävs en ökad kunskap om medfödda hjärtfel. Sjuksköterskan bör sätta sig in i patientens livsvärld och på så sätt göra vården personcentrerad. Antalet vuxna med medfött hjärtfel har ökat, vilket gör att fundera kring yrkesval, förmåga att bilda familj och ärftlighet av sjukdomen uppstår. Studiens resultat grundar sig på artiklar från olika delar av världen, vilket gör att upplevelserna kan skilja sig åt beroende på land och sjukvårdssystem. Studiens resultat visar att unga vuxna upplever sitt hjärtfel på olika sätt. En del känner att de har kontroll över sin sjukdom och därmed upplever de sig friska. Hjärtfelet är en del av identiteten och de känner sig inte begränsade i sin vardag, medan andra unga vuxna upplever att det är hjärtfelet som styr och kontrollerar deras liv och de ser sig som inneboende i sin sjukdom. Det kan leda till upplevelser om att vilja ge upp och det kan ha en negativ påverkan på hälsotillståndet. Unga vuxna lever med oro och osäkerhet om sin framtid eftersom den upplevs som oviss. En vanlig upplevelse unga vuxna har är att de känner sig annorlunda. Det kan bero på fysiska begränsningar och operationsärret men den största anledningen är hur de upplever sig bli bemötta från sin omgivning. Studien är tänkt för att sjuksköterskan ska få en ökad förståelse för patientens livsvärld. / Background: Every day approximately one percent of the world's children are born with a congenital heart disease. Life span has increased for patients with congenital heart disease due to early diagnosis and development in recent decades in cardiac surgery. Living with a congenital heart disease got young adults to think about the disease itself, the ability to start a family, sexual activity, pregnancy, childbirth, choice of profession, physical activity and heredity. Today there are more adults than children with a congenital heart disease and the study highlights the experiences of the disease of young adults aged 13-39 years. Aim: The aim of the study was to describe young adults' experiences of living with a congenital heart disease. Method: A literature-based study has been made of qualitative studies retrieved from databases Cinahl and PubMed. The study is based on nine articles from different countries around the world. The articles have been analyzed by content analysis for qualitative studies. Results: The study's analysis resulted in three main themes; "feel different", "to be controlled by the disease," and "to take control of the disease." Conclusion: Living with a congenital heart disease was experienced in different ways, it was splitted opinions if the young adults felt they had control over their heart disease or not. The most common experience was to feel different. The young adults felt anxiety and uncertainty for the future because it could be experienced as uncertain.

M.Sc. (Med. (Paediatric Cardiology)), Faculty of Health Sciences, University of the Witwatersrand, 2008 / Left ventricular (LV) noncompaction is characterized by the presence of an extensive trabecular myocardial layer within the luminal aspect of the compact myocardium of the ventricular wall. The trabeculae are both excessive in number and more prominent than normal. Noncompaction may occur in isolation usually with clinical features of dilated cardiomyopathy, or it may be associated with congenital or acquired heart diseases. Echocardiography is the reference standard for diagnosis, where a ratio of thickness of trabecular-to-compact myocardium (compaction ratio) of >2 is a major diagnostic criterion. Noncompaction is usually considered to result from persistence of the highly trabeculated myocardium found in early cardiogenesis of the human embryo. If persistence of excess trabeculae is the only determinant of the compaction ratio it would be expected that it would remain a consistent measurement in postnatal life. However, temporal changes in the degree of noncompaction in individual case reports have raised the question as to whether the compaction ratio might be sensitive to haemodynamic or other factors. In the present dissertation, I assessed echocardiographically whether the compaction ratio is associated with increases in indices of LV volume preload in 100 children or adolescents with ventricular septal defects (VSD), and 36 with chronic rheumatic heart disease (RHD). Compared to 79 normal controls (compaction ratio=1.4±0.07), patients with VSDs (compaction ratio=2.0±0.2, p<0.0001) and RHD (compaction ratio = 2.0±0.3, p< 0.0001) had a marked increase in the compaction ratio. A compaction ratio>2 was found in 42% of patients with VSDs and 47% with RHD. In VSDs, independent of age and gender, the compaction ratio was positively associated with LV mass index (LVMI) (partial r=0.44, p<0.0001), VSD size (partial r=0.4, p<0.0001), LV end diastolic diameter indexed (LVEDD) (partial r=0.24, p= 0.01), and the presence of additional shunts (partial r=0.21, p=0.02). In RHD, independent of age and gender, the compaction ratio was positively associated with LVEDD (partial r=0.62, p=0.0001), and LVMI (partial r=0.48, p=0.005), and negatively with LV ejection fraction (partial r=0.31, p=0.03). The strong association of indices of LV volume load and the compaction ratio would suggest that haemodynamic influences are contributing to the compaction ratio both in congenital and acquired cardiac disease in childhood. Thus an increased compaction ratio may be the consequence of an increased volume preload, and therefore may not necessarily occur only as a result of persistence of embryonic patterns.

Aim: To describe innovative uses of catheter based treatment in a variety of native and post surgical stenoses in children and young adults with congenital heart disease. Background: Cardiac catheterization in man was first described 1929 and since then there has been a drive to develop endovascular techniques to investigate and treat both congenital and acquired heart disease. Many of the advances are being made in congenital heart disease. Methods: A number of congenital cardiac stenotic lesions were studied including baffle obstruction after atrial switch for transposition of the great arteries, aortic stenosis in infants, coarctation of the aorta, peripheral pulmonary artery stenosis and superior vena caval obstruction. The use of angioplasty balloons, cutting balloons, stents and alternative catheter approaches were investigated for these lesions. Results: Following atrial redirection surgery for transposition of the great arteries balloon angioplasty improved baffle haemodynamics. The technique of anterograde balloon dilation of the aortic valve was developed and had superior outcomes in terms of aortic insufficiency compared to a retrograde approach in neonates with severe aortic valve stenosis. In an animal model of peripheral pulmonary arterial stenosis, the application of cutting balloon angioplasty produced effective relief in a controlled fashion. Balloon mounted stents were used in patients with native and post surgical coarctation of the aorta with significant relief of stenosis and relief of hypertension. Finally, a group of patients with superior vena obstruction syndrome after surgical repair of partial anomalous pulmonary venous drainage had successful treatment using balloon mounted stents. Conclusions: Catheter based treatment of congenital and post surgical vascular stenoses of the heart and great arteries using angioplasty balloons, cutting balloons and balloon mounted stents is safe and appears to be effective in the short and medium term. It may represent a useful alternative to surgery and will reduce the number of surgical procedures required over a lifetime. Future directions will include bio-absorbable stents and hybrid techniques involving surgery.

The development and function of the heart is governed by a conserved set of transcription factors (TFs) that regulate gene expression in a cell-type, time point and stimulus driven manner. Of these core cardiac TFs, the most ubiquitously expressed is the zinc finger protein GATA4. In cardiomyocytes, GATA4 is central to proliferation, differentiation, hypertrophy and induction of pro-survival pathways. In cardiac endothelial cells, it is required for valve and septal development, although the exact mechanisms remain unclear. To regulate such a wide array of functions in a spatially and temporally controlled manner, GATA4 interacts with specific protein partners, the majority of whom have been identified in cardiomyocytes. However, a complete understanding of the protein interactome of GATA4, particularly in cardiac endothelial cells, has not yet been achieved. Using a mass spectrometry-based approach, we have identified a series of novel GATA4 interacting partners in cardiac endothelial cells. 3xFlag GATA4 was stably overexpressed via retroviral transduction in the TC13 cardiac endothelial precursor cell line, immunoprecipitated from nuclear protein extracts and sent for HPLC-ESI-MS/MS. Several novel GATA4 interacting partners were identified including the chaperone protein Heat Shock Protein 70 (HSP70), the inducible orphan nuclear receptor Nerve Growth Factor 1β (NGFIβ, NUR77) and the Drosophila-Binding/Human Splicing protein family members Non-POU Domain Containing Octamer Binding Protein (NONO) and Paraspeckle 1 (PSPC1). Chapter 1 discusses the interaction between GATA4 and HSP70 and its role in cardiomyocyte survival upon exposure to chemotherapeutic agent Doxorubicin (DOX). HSP70 binds directly to GATA4, preventing DOX-mediated cleavage and degradation by Caspase-1, cardiomyocyte cell death and heart failure. Chapter 2 focuses on the cooperative interaction between GATA4 and NUR77 in cardiac microvascular endothelial cells and its central role in myocardial angiogenesis in response to pressure overload. The GATA4-NUR77 complex transactivates the promoter of Angiopoietin-Like 7 (ANGPTL7), a secreted pro-angiogenic chemotactic factor, triggering endothelial cell proliferation and tube formation in cultured cardiac endothelial cells and increasing myocardial capillary density in vivo. Chapter 3 discusses the interaction between GATA4 and the DBHS proteins NONO and PSPC1 in the regulation of cardiac development. These proteins play opposing roles when bound to GATA4 as PSPC1 enhances GATA4 activation of critical cardiac promoter targets and NONO acts as a rheostat to repress GATA4 activity. In vivo, loss of NONO results in left ventricular non-compaction consistent with humans with loss-of-function mutations. However, simultaneous Gata4 haploinsufficiency partially rescues this phenotype. Together, this data identifies multiple novel cell type and time point specific GATA4 protein partners and sheds light on GATA4 regulatory mechanisms in cardiac development and disease.

Congenital increase in cardiac outflow tract resistance would lead to chronic ventricular pressure overloading, and eventually ventricular hypertrophy and dysfunction. Conventional echocardiographic assessment of global ventricular systolic and diastolic function is far from ideal because of the lack of sensitivity. There is a pressing need in developing a clinical tool to unveil the degree of myocardial dysfunction as well as to monitor the treatment effect in these patients. M-mode and tissue Doppler recordings of the left and right atrioventricular rings' motion in a longitudinal cardiac axis allowed us to assess segmental ventricular function in a more sensitive and specific way. This thesis aimed to assess ventricular long axis function in patients with congenital outflow tract obstructions. It is based on the anatomical observation that a major part of long axis function is subtended by subendocardial fibers, and the hypothesis that the function of these fibers might be more sensitive to effect of pressure overload than circumferential ones. / Normal values for left and right ventricular (RV) long axis function (in left, septal and tricuspid sites of atrioventricular rings) were first established by studying normal healthy volunteers without cardiovascular diseases with M-mode and pulse wave (PW) tissue Doppler Imaging (TDI). These values included: / 1) M-mode derived systolic amplitude of motion (displacement) / 2) Any long axis incoordination (long axis shortening after end of ejection which was reported as a sign of coronary ischemia after excluding interventricular conduction abnormalities) / 3) TDI-derived peak systolic and diastolic velocities. / The following groups of patients with congenital outflow tract obstructions were therefore studied: / 1) Congenital aortic valve stenosis and subaortic stenosis (21 patients, study 1). / 2) Congenital coarctation of aorta (23 patients, study 2). / 3) Congenital pulmonary valve stenosis (43 patients, study 3). / Study 1 investigated the presence of "isolated" diastolic disease, defined as reduced long axis early diastolic velocity with normal systolic velocity in 21 young patients with congenital aortic valve stenosis (AVS). Most patients with normal left ventricular (LV) ejection fractions in fact had depressed long axis systolic velocities. This work demonstrated the selective sensitivity of long axis function in unveiling myocardial dysfunction in AVS patients. Moreover, good correlation was found between long axis systolic and diastolic velocities which suggested isolated diastolic disease is unlikely. / Patients with coarctation of aorta (CoA) are often less symptomatic (fewer reported chest pain or heart failure) that AVS patients despite having similar degree of outflow resistance. Study 2 addressed such phenomenon by studying the long axis function in 23 CoA patients and the results were compared to 23 AVS patients and normal controls. This work again confirmed the presence of LV long axis impairment in patients with chronic increase in LV afterload. Moreover, a worse deterioration of LV long axis function and a higher prevalence of long axis incoordination independent of LV outflow resistance is seen in patients with proximally increased LV afterload (AVS) compared with distal disease (CoA) that might account for their difference in clinical behavior. / Data are scant to address the impact of chronic increase in RV outflow resistance on RV diastolic performance. Study 3 evaluated the relationship of restrictive RV physiology (defined as the presence of antegrade pulmonary arterial flow in late diastole measured by conventional PW Doppler echocardiography) to RV long axis function and patients' symptoms in 43 patients with pulmonary valve stenosis (PVS). Restrictive RV physiology was found in 42% studied patients who were more symptomatic and had more severe RV long axis dysfunction. This work implied regular follow-up of adult PVS patients using simple qualitative RV Doppler echocardiographic measurements should guide toward early identification of myocardial dysfunction and the need for removal of outflow tract obstruction before irreversible damage occurs. / Given the selective sensitivity of long axis function in assessing myocardial damage In patients with pressure overloaded ventricle, I further studied the effect of interventions on change of long axis behavior in patients with CoA: / 1) Before and 14 month after successful endovascular stenting (21 patients, study 4). / 2) Long term follow up after surgical correction, by either angioplasty or endovascular stenting (80 patients, study 5). / Study 4 prospectively evaluated the effect of endovascular stenting on intermediate-term biventricular function in 21 adults with CoA and the results were compared with surgically repaired patients and normal controls. LV long axis disturbances were improved with sparing of RV long axis function after intervention. Subclinical myocardial dysfunction was observed in both stented and repaired patients compared with normal controls. This work supported aortic stenting in patients with anatomically suitable lesions. It also prompted further studies on the clinical significance of persistent myocardial dysfunction in "corrected" CoA patients. / In study 5, I went on to examine the prevalence of LV long axis diastole dysfunction (defined as septal PW TDI early diastolic velocity 8cm/s) in a relatively large cohort of CoA patients (n=80) and its relationships to patient demo graphics and aortic elastic properties. Forty-seven patients (59%) were found to have LV long axis dysfunction. As a group, they were older when received treatment and had higher ascending aortic stiffness indices despite similar systemic blood pressure, prevalence of anti-hypertensive use and associated bicuspid aortic valve as compared to other CoA patients. This work addressed the age at intervention and increased aortic stiffness are important determinants of persistent myocardial dysfunction after intervention. Intervention at an early age before structural damage to proximal aorta and appropriate medications to reduce central aortic stiffness might preserve LV long axis function in "corrected" CoA patients and further improve their long term prognosis. / CONCLUSIONS: / In conclusion, conventional global echocardiographic assessment is inadequate for assessing patients with congenital outflow tract obstructions. Segmental ventricular long axis function is frequently abnormal at rest in these patients. The site of these disturbances corresponds to the ventricle that is subjected to pressure overloading. Assessment of long axis function is simple and sensitive. It has considerable potential in investigating the natural course of myocardial damage, to clarify the basis of abnormal ventricular function, particularly during diastole and to evaluate treatment effect on myocardial recovery in patients with congenital outflow tract obstructions. Further studies should focus on the role of long axis function in determining prognosis for these patients. / Lam, Yat Yin. / "November 2011." / Thesis (M.D.)--Chinese University of Hong Kong, 2012. / Includes bibliographical references (leaves 134-160). / ABSTRACT --- p.1 / Chapter PART1 --- HISTORIC REVIEW --- p.5 / Chapter Chapter1 --- VENTRICULAR OUTFLOW TRACT OBSTRUCTION SYNDROME --- p. 6 / Chapter 1.1 --- BACKGROUND --- p.7 / Chapter 1.2 --- AORTIC VALVULAR STENOSIS --- p.8 / Chapter 1.2.1 --- Anatomy pathophysiology and clinical features --- p.8 / Chapter 1.2.2 --- Investigations --- p.9 / Chapter 1.2.3 --- Treatment --- p.9 / Chapter 1.3 --- COARCTATION OF AORTA --- p.9 / Chapter 1.3.1 --- Anatomy pathophysiology and clinical features --- p.9 / Chapter 1.3.2 --- Investigations --- p.11 / Chapter 1.3.3 --- Treatment --- p.11 / Chapter 1.4 --- PULMONARY VALVULAR STENOSIS --- p.12 / Chapter 1.4.1 --- Anatomy pathophysiology and clinical features --- p.12 / Chapter 1.4.2 --- Investigations --- p.13 / Chapter 1.4.3 --- Treatment --- p.13 / Chapter Chapter2 --- VENTRICULAR RESPONSE TO HIGH AFTERLOAD --- p.14 / Chapter 2.1 --- VENTRICULAR ADAPTATION IN AORTIC VALVULAR STENOSIS --- p.15 / Chapter 2.2 --- VASCULAR STIFFENING AORTIC COARCTATION --- p.17 / Chapter 2.3 --- VENTRICULAR ADAPTATION IN PULMONARY VALVULAR STENOSIS --- p.18 / Chapter 2.4 --- LIMITATIONS OF CONVENTIONAL ECHOCARDIOGRAPHY --- p.19 / Chapter Chapter3 --- MYOCARDIAL FIBER ARCHITECTURE AND LONG AXIS FUNCTION --- p.21 / Chapter 3.1 --- MYOCARDIAL FIBER STRUCTURE AND LONG AXIS FUNCTION --- p.22 / Chapter 3.2 --- NOMRAL LONG AXIS EXCURSION AND TIMING --- p.25 / Chapter 3.3 --- QUANTIFICATION OF LONG AXIS MOTIONS --- p.26 / Chapter 3.3.1 --- M-mode --- p.26 / Chapter 3.3.2 --- Clinical applications of atrio-ventricular plane displacement --- p.27 / Chapter 3.3.3 --- Tissue Doppler Imaging --- p.28 / Chapter 3.3.4 --- Clinical application of tissue Doppler imaging --- p.30 / Chapter 3.3.5 --- Limitations of M-mode and TDI in the assessment of long axis function --- p.31 / Chapter Chapter4 --- HYPOTHESIS AND SPECIFIC OBJECTIVES OF THE THESIS --- p.32 / Chapter 4.1 --- INTRODUCTION --- p.33 / Chapter 4.2 --- HYPOTHESIS --- p.34 / Chapter Chapter5 --- METHODOLOGY --- p.35 / Chapter 5.1 --- STUDY PATIENTS --- p.36 / Chapter 5.1.1 --- Patients with ventricular outflow tract obstruction --- p.36 / Chapter 5.1.2 --- Normal controls --- p.36 / Chapter 5.2 --- CLINICAL ASSESSMENT --- p.37 / Chapter 5.3 --- ECHO CARDIOGRAPHIC ASSESSMENT --- p.37 / Chapter 5.3.1 --- Imaging acquisition protocol --- p.37 / Chapter 5.3.2 --- Assessment of severity of outflow tract obstruction --- p.38 / Chapter 5.3.3 --- Assessment of global ventricular structure and function --- p.40 / Chapter 5.3.4 --- Assessment of long axis function --- p.42 / Chapter 5.3.5 --- Evaluation of restrictive RV physiology in PVS patients --- p.42 / Chapter 5.3.6 --- Determination of aortic elastic properties in CoA patients --- p.43 / Chapter 5.3.7 --- Reproducibility --- p.45 / Chapter 5.4 --- CARDIAC CATHETERIZATON AND ENDOVASCULAR STENTING FOR COARCTATION OF AORTA --- p.45 / Chapter 5.5 --- STATISTICS --- p.46 / Chapter PART 2 --- CLINICAL STUDIES --- p.48 / Chapter Chapter6 --- "ISOLATED" DIASTOLIC DYSFUNCTION IN LEFT VENTRICULAR OUTFLOW TRACT OBSTRUCTION --- p.49 / Chapter 6.1 --- INTRODUCTION --- p.50 / Chapter 6.2 --- METHODS --- p.50 / Chapter 6.2.1 --- Study population --- p.50 / Chapter 6.2.2 --- Echocardiographic examination --- p.51 / Chapter 6.2.3 --- Reproducibility --- p.51 / Chapter 6.2.4 --- Statistics --- p.51 / Chapter 6.3 --- RESULTS --- p.52 / Chapter 6.3.1 --- Baseline characteristics --- p.52 / Chapter 6.3.2 --- Echocardiographic measurements --- p.52 / Chapter 6.3.3 --- Correlation analysis between long axis variables --- p.55 / Chapter 6.3.4 --- Reproduci bility --- p.58 / Chapter 6.4 --- DISCUSSION --- p.58 / Chapter 6.4.1 --- Selective sensitivity oflong axis function --- p.58 / Chapter 6.4.2 --- Close coupling of long axis systolic and diastolic function --- p.59 / Chapter 6.4.3 --- Limitations --- p.60 / Chapter 6.5 --- CONCLUSIONS --- p.61 / Chapter Chapter7 --- EFFECT OF CHRONIC AFTERLOAD INCREASE ON LEFT VENTRICULAR MYOCARDIAL FUNCTION IN PATIENTS WITH CONGENITAL LEFT-SIDED OBSTRUCTIVE LESIONS --- p.62 / Chapter 7.1 --- INTRODUCTION --- p.63 / Chapter 7.2 --- METHODS --- p.63 / Chapter 7.2.1 --- Study population --- p.63 / Chapter 7.2.2 --- Echocardiographic examination --- p.64 / Chapter 7.2.3 --- Reproducibility --- p.65 / Chapter 7.2.4 --- Statistics --- p.65 / Chapter 7.3 --- RESULTS --- p.66 / Chapter 7.3.1 --- Baseline characteristics --- p.66 / Chapter 7.3.2 --- Patients versus controls --- p.66 / Chapter 7.3.3 --- Aortic valvular stenosis versus coarctation patients --- p.70 / Chapter 7.3.4 --- Reproducibility --- p.70 / Chapter 7.4 --- DISCUSSION --- p.72 / Chapter 7.4.1 --- Long axis function in patients with increased LV afterload --- p.72 / Chapter 7.4.2 --- Difference between aortic valvular stenosis and coarctation patients --- p.72 / Chapter 7.4.3 --- Limitations --- p.74 / Chapter 7.5 --- CONCLUSIONS --- p.74 / Chapter Chapter8 --- RESTRICTIVE RIGHT VENTRICULAR PHYSIOLOGY: ITS PRESENCE AND SYMPTOMATIC CONTRIBUTION IN PATIENTS WITH PULMONARY STENOSIS --- p.75 / Chapter 8.1 --- INTRODUCTION --- p.76 / Chapter 8.2 --- METHODS --- p.77 / Chapter 8.2.1 --- Study population --- p.77 / Chapter 8.2.2 --- Echocardiographic examination --- p.77 / Chapter 8.2.3 --- Reproducibility --- p.78 / Chapter 8.2.4 --- Statistics --- p.78 / Chapter 8.3 --- RESULTS --- p.79 / Chapter 8.3.1 --- Baseline characteristics --- p.79 / Chapter 8.3.2 --- Patients versus controls --- p.81 / Chapter 8.3.3 --- Comparison between patient groups --- p.81 / Chapter 8.3.4 --- Predictors for symptoms --- p.84 / Chapter 8.3.5 --- Reproducibility --- p.85 / Chapter 8.4 --- DISCUSSION --- p.85 / Chapter 8.4.1 --- Main findings --- p.85 / Chapter 8.4.2 --- Characterization of diastolic performance in pulmonary valvuar stenosis patients --- p.85 / Chapter 8.4.3 --- Mechanism for restrictive RV physiology in PVS patients --- p.87 / Chapter 8.4.4 --- Study inlplications --- p.89 / Chapter 8.4.5 --- Limitations --- p.90 / Chapter 8.5 --- CONCLUSIONS --- p.90 / Chapter Chapter9 --- EFFECT OF ENDOVASCULAR STENTING OF AORTIC COARCTATION ON BIVENTRlCULAR FUNCTION IN ADULTS --- p.91 / Chapter 9.1 --- INTRODUCTION --- p.92 / Chapter 9.2 --- METHODS --- p.92 / Chapter 9.2.1 --- Study population --- p.92 / Chapter 9.2.2 --- Implantation procedure --- p.93 / Chapter 9.2.3 --- Echocardiographic examination --- p.94 / Chapter 9.2.4 --- Reproducibility --- p.94 / Chapter 9.2.5 --- Statistics --- p.95 / Chapter 9.3 --- RESULTS --- p.95 / Chapter 9.3.1 --- Baseline characteristics --- p.95 / Chapter 9.3.2 --- Blood pressure data --- p.96 / Chapter 9.3.3 --- Echocardiographic measurements --- p.97 / Chapter 9.3.3.1 --- Pre-stenting versus post-stenting (group 1) --- p.97 / Chapter 9.3.3.2 --- Stenting (group 1) versus post-surgical repair (group 2) --- p.101 / Chapter 9.3.3.3 --- Coarctation patients (group 1 post-stenting and group 2) versus control (group 3) --- p.101 / Chapter 9.3.4 --- Reproducibility --- p.101 / Chapter 9.4 --- DISCUSSION --- p.103 / Chapter 9.4.1 --- Main findings --- p.103 / Chapter 9.4.2 --- Potential impact of stenting on cardiovascular outcome --- p.103 / Chapter 9.4.2.1 --- BP control --- p.103 / Chapter 9.4.2.2 --- LV mass and long axis function --- p.104 / Chapter 9.4.3 --- RV function after stenting --- p.105 / Chapter 9.4.4 --- Subclinical LV myocardial function in coarctation patients --- p.105 / Chapter 9.4.5 --- Limitations --- p.106 / Chapter 9.5 --- CONCLUSIONS --- p.107 / Chapter Chapter10 --- LEFT VENTRICULAR LONG AXIS DYSFUNCTION IN ADULTS WITH "CORRECTED" AORTIC COARCTATION IS RELATED TO AN OLDER AGE AT INTERVENTION AND INCREASED AORTIC STIFFNESS --- p.108 / Chapter 10.1 --- INTRODUCTION --- p.109 / Chapter 10.2 --- METHODS --- p.110 / Chapter 10.2.1 --- Study population --- p.110 / Chapter 10.2.2 --- Echocardiographic examination --- p.111 / Chapter 10.2.3 --- Reproducibility --- p.111 / Chapter 10.2.4 --- Statistics --- p.112 / Chapter 10.3 --- RESULTS --- p.112 / Chapter 10.3.1 --- Patients versus controls --- p.112 / Chapter 10.3.2 --- Patients with long axis dysfunction (LAD) versus without LAD --- p.113 / Chapter 10.3.3 --- Predictors of LAD --- p.117 / Chapter 10.3.4 --- Reproducibility --- p.120 / Chapter 10.4 --- DISCUSSION --- p.120 / Chapter 10.4.1 --- Main findings --- p.120 / Chapter 10.4.2 --- LV dysfunction and adverse remodeling in "corrected" aortic coarctation --- p.120 / Chapter 10.4.3 --- Mechanism behind LV dysfunction in "corrected" aortic coarctation --- p.121 / Chapter 10.4.4 --- Study implications --- p.124 / Chapter 10.4.5 --- Limitations --- p.124 / Chapter 10.5 --- CONCLUSIONS --- p.125 / Chapter Chapter 11 --- DISCUSSION --- p.126 / Chapter 11.1 --- DISCUSSION --- p.127 / Chapter 11.2 --- LIMITATIONS --- p.129 / Chapter 11.3 --- THE FUTURE --- p.129 / Chapter Chapter12 --- CONCLUSIONS --- p.131 / APPENDIX --- p.133 / BIBLIOGRAPHY --- p.134

Resumo: O objetivo geral deste estudo foi avaliar a presença, a sintomatologia predominante e as fases do estresse, identificar estratégias de enfrentamento de problemas utilizadas por mães de crianças cardiopatas submetidas a cirurgia cardíaca, e os objetivos específicos associar as variáveis tais como estratégia de enfrentamento e cardiopatia, fase de estresse e cardiopatia, fase de estresse e faixa etária da mãe, estratégia de enfrentamento e idade da mãe, fase de estresse e estratégia de enfrentamento, classe sócio-econômica e estratégia de enfrentamento. A amostra da pesquisa constituiu-se de 60 mães de crianças cardiopatas. Foram incluídas no estudo somente as mães biológicas, as quais vivenciavam a experiência da primeira cirurgia cardíaca do filho logo após a revelação do diagnóstico. Foi um estudo epidemiológico de prevalência do tipo transversal. Os resultados indicaram que a maioria das mães apresentou estresse na fase de resistênci , fase caracterizada por demasiado estresse e vulnerabilidade a doenças, com predominância de sintomas psicológicos, a estratégia de enfrentamento prioritária foi a busca de práticas religiosas. Para a análise estatística foi utilizado o teste Qui-quadrado, que mostrou não haver associação entre as variáveis tipo de cardiopatia com as estratégias de enfrentamento P= 0,840, cardiopatia com a fase de estresse P= 0,675, fase de estresse com estratégia de enfrentamento P= 0,375, fase de estresse com faixa etária, estratégia de enfrentamento com faixa etária e classe social com estratégia de enfrentamento P= 0,444, pois os valores de P foram > 0,05. Com base nos resultados obtidos concluiu-se que é necessário uma intervenção profissional com o objetivo de acolher, orientar e disponibilizar recursos de auxílio as mães desde o diagnóstico à alta hospitalar da criança para favorecer a um enfrentamento e adaptação... (Resumo completo, clicar acesso eletrônico abaixo) / Abstract: Study general objectives: to assess stress and coping in mothers of children with congenital heart disease who had undergone surgery; and specifics objectives to associate coping style, stress, mothers age, heart disease and social class. Methods: Sixty mothers whose children had undergone surgery for congenital heart disease filled the Lipp Stress Symptoms Inventory for Adults, and the Ways of Coping Scale. Results: most mothers were in the resistance stage of stress. Psychological symptoms of stress were predominant and the most used coping strategy was religion. Chi-square test showed no association (p≤0.05) between heart disease and coping style (p = 0,840), heart disease and stress stage (p = 0,675), stress stage and coping (p = 0,375), stress and age, coping and age and social class and age and social class and coping (P = 0,444). The presence of and age stress in the resistance stage in most participants indicates the need for professional intervention from diagnosis until the discharge after surgery, to favor positive coping strategies and a better quality of life for mother and child. / Orientador: Antonio Sergio Martins / Coorientador: Ulisses Alexandre Croti / Banca: Marcos Augusto de Moraes Silva / Banca: Reinaldo Ayer de Oliveira / Mestre