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Cardiorespiratory fitness, physical activity and long term health in adult patients with congenital heart disease.

Chapter I</></> describes the relevance of the doctoral project, starting with a general introduction on congenital heart diseases and their prevalence. </>Cardiopulmonary exercise testing has an important role in the follow-up of patients with congenital heart disease. </>Therefore currently existing knowledge on exercise capacity, as well as physical activity and exercise training in children with congenital heart disease is summarized. Finally, this chapter describes what is known so far in an adult population with congenital heart disease. </>
The first part</> of the doctoral thesis is dedicated to the evaluation of cardiopulmonary exercise tolerance in adult patients with congenital heartdisease. </>
Chapter II</></> is a cross-sectional investigation of the maximal character of an exercise test in adults with congenital heart disease. Furthermore, different measures of exercise tolerance that donot require a maximal effort are investigated. Results show that although adult patients with congenital heart disease seem to be able to reachmaximal exercise levels, but some parameters show that not all patientsachieve the same maximal character on the exercise test compared to healthy adults. Moreover, the investigated maximal and submaximal parameters of exercise capacity are impaired in all patient groups compared to healthy controls and the impairment is more severe with higher complexity of the underlying heart defect. Different expressions of exercise tolerance clearly show similar differences in exercise capacity across groups of patients with different congenital heart diseases. Submaximal parameters may be useful in addition to peak oxygen uptake in order to accurately interpret the exercise tolerance in adult patients with congenital heart disease.</>
Results of the longitudinal investigation of cardiopulmonary exercise capacity in a group of patients who underwent Senning repair for transposition of the great arteries are discussed in chapter III</>. </></>This study shows that peak oxygen uptake and peak oxygen pulse, expressed as percentage of predicted, decrease faster with age thanin healthy controls. This decline is most obvious during childhood and adolescence and in female patients. The fact that the progressive decline is most obvious during childhood and adolescence suggests the impossibility to increase stroke volume to the same extent as healthy peers during growth. Furthermore, a faster decline in peak oxygen pulse is relatedto a decrease in right ventricular function. It seems that serial exercise testing can provide valuable information for individual patient assessment. </>
The second part</> of the doctoral thesis isdedicated to the investigation of physical activity in daily life in adult patients with aortic coarctation (chapter IV</>), tetralogy of Fallot (chapter V</>) and atrial switch operation for transposition of the great arteries (chapter VI</>). Overall, our results show that the investigated patients report lower levels of habitual physical activity and lower perceived physical functioning in comparison with healthy counterparts. The more physically active patients show a better exercise capacity and report a better physical health status than patients who are less active. Moreover, a body mass index larger than 25 kg/m² was present in onethird of the patients with aortic coarctation. A sedentary lifestyle and a unhealthy weight status probably substantially increase the cardiovascular risk profile of these patients. Further investigation of determinants of exercise tolerance in patients with atrial switch repair revealed that exercise capacity is higher in patients with Senning repair, witha well preserved right ventricular function and a more active lifestyle. Based on the results of these studies, it seems that physical activityguidance and exercise prescription deserve more attention in adult patients with congenital heart disease. </>
The third part</> of the doctoral thesis is dedicated to the prognostic value of the exercise tolerance in adult patients with congenital heart disease. </>
Chapter VII</></> is a multicentre investigation aiming at evaluating differences between hypertensive and non-hypertensive patients at baselineand at investigating the value of cardiopulmonary exercise testing to predict the development or progression of arterial hypertension in adult patients with coarctation of the aorta. Results reveal that hypertensivepatients with aortic coarctation are more often of male gender, are older and underwent surgical repair at an older age. In non-hypertensive patients with aortic coarctation, a reduced peak oxygen uptake, steeper ventilatory efficiency slope and higher blood pressure response to exercise are associated with an increased risk for the development of arterial hypertension. However, in hypertensive patients, only age remains a significant predictor for the progression of hypertension. </>
Hence, it seems warranted to actively and aggressively search for the first signs of hypertension in this patient cohort and to emphasize on primary prevention measures in order to delay/prevent the onset of hypertension as much as possible. </>
The prognostic value of measures of cardiopulmonary exercise testing along with variables known to be related with outcome, are investigated at medium term follow-up in adult patients with Tetralogy of Fallot and Fallot-type morphology. This study is the main subject of chapter VIII</> of this doctoral thesis. The results show that older age at surgical correction, reduced right ventricular function, rightventricular dilatation, decreased peak oxygen uptake and increased ventilatory efficiency slope are important independent predictors of hospital admission and death in these patients.</>
We furthermore contributed with our data in a multicentre study on the prognostic value of cardiopulmonary exercise testing in patients with tetralogy of Fallot (chapterIX</>). In a first part of this study, the prognostic value of exerciseand clinical variables for death and survived sustained ventricular tachycardia is investigated. Older age, a lower percentage of predicted peak oxygen uptake and a longer QRS-duration are independently associated with worse sustained ventricular tachycardia-free survival. Furthermore cut-off values related to a substantially worse prognosis are peak oxygenuptake ≤65% and QRS-duration≥170 ms and can serve as a guideline for clinical practice. </>
In the second part of this study, a cardiac-related event is defined as any admission directly caused by failure of the cardiac system needing inpatient care. Examples for cardiac event are cardiac surgery, interventional catheterization and symptomatic arrhythmias needing cardioversion, electrophysiological procedure or further treatment. </>The prognostic value of exercise tolerance is againshowed; both peak oxygen uptake and VE/VCO2-slope are related with event-free survival, along with age and QRS-duration. Again, cut-off scores are defined in order to guide clinical practice. </>Significant cardiac morbidity can be predicted by a peak oxygen uptake ≤65%, a VE/VCO2-slope ≥31, and a resting QRS duration ≥170ms, with the riskof events increasing in a stepwise manner for every added risk factor present. </>
Finally, in chapter X</> the main results ofthe doctoral research are discussed and considered in the context of the study as a whole. We can conclude that a</>dult patients with congenital heart disease show a reduced exercise capacity, which is more severe in patients with more complex underlying cardiac abnormalities. The reduced exercise tolerance is not only related with heart disease related impairments, but also with physical activity levels that are lower in comparison with the general population and lower then recommended. Moreover,a reduced exercise capacity is related with a higher risk for cardiovascular diseases, cardiovascular interventions requiring hospitalisation and death. </>
Based on our overall results we believe that it seems recommended to actively inform, encourage and guide adult patients with congenital heart disease to be more physically active withinthe limits of their cardiac condition. </>
Date:1 Jan 2008 →  5 Jul 2012
Keywords:heart disease, Adult patients, Trainability
Disciplines:Cardiac and vascular medicine, Respiratory medicine, Orthopaedics, Neurosciences, Biological and physiological psychology, Cognitive science and intelligent systems, Developmental psychology and ageing, Education curriculum, Education systems, General pedagogical and educational sciences, Specialist studies in education, Other pedagogical and educational sciences
Project type:PhD project