| Summary: | Adult congenital heart disease (ACHD) patients have markedly depressed exercise capacity. This thesis examined (i) the prevalence of chronotropic incompetence, its relationship to symptoms and exercise capacity and its prognostic value in ACHD patients; (ii) investigated exercise capacity in patients with Eisenmenger syndrome and assessed survival prospects in this cohort as well as risk factors for mortality. In addition, clinical effects of Bosentan (a pulmonary vasodilator) were examined during longer-term follow-up. (iii) Mathematical modelling studies were employed to assess the impact of intracardiac shunting on oxygen delivery and tissue oxygenation potentially affecting exercise capacity, (iv) As pulmonary and systemic endothelial dysfunction are integral features of pulmonary arterial hypertension (and are related to exercise intolerance in this setting), this thesis examined the number and function of endothelial progenitor cells (EPC) in patients with idiopathic pulmonary hypertension and Eisenmenger syndrome.
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