Abstract
Adults with a congenital heart disease (ACHD) have a lower functional capacity, reduced quality of life and worse prognosis compared to healthy individuals. ACHD and patients with heart failure (HF) induced by other aetiologies share many characteristics, incl. exercise intolerance, right ventricular (RV) dysfunction and increased inflammatory cytokine levels. Among the pathophysiological changes in HF, endothelial dysfunction is highlighted. However, the presence of endothelial dysfunction in ACHD is unknown as literature is limited and conflicting. I hypothesize that coronary microvascular dysfunction (CMD) is important in the pathophysiology of ACHD. I believe that multiple factors incl. genetics, underlying cardiac abnormality, history of cardiac surgery and RV overload, further aggravated by classical acquired risk factors (including overweight, hypertension and sedentary lifestyle), alter shear stress and promote systemic inflammation and endothelial oxidative stress in ACHD leading to a reduced nitric oxide bioavailability and endothelial dysfunction. As such I assume that CMD is associated with systemic endothelial dysfunction, reflecting CMD as part of a systemic microvascular disorder. I am convinced that detecting CMD is important to allow identification of ACHD with an unfavorable prognosis and that this CMD can easily be identified with adenosine-based Doppler echocardiography. Finally, the potential therapeutic effect of exercise training will be investigated.
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