In patients with a surgically corrected transposition of the great arteries or a congenitally corrected transposition of the
great arteries, the right ventricle supports the systemic circulation. Although the right ventricle adjusts remarkably well
to systemic pressures, long term complication seem inevitable. Most adult patients with a systemic right ventricle have decreased
exercise intolerance and right ventricular dysfunction, and arrhythmias and clinical heart failure are common. Heart failure
is the main cause of premature death in this population. This thesis focusses on the treatment and prevention of heart failure
and exercise intolerance in patients with a systemic right ventricle. In a randomized clinical trail the efficacy of angiotensin
II receptor blocker to prevent further deterioration of systemic right ventricular function. While valsartan did not approve
right ventricular function, it attenuated further ventricular remodeling. Moreover, in a subgroup of patients with symptomatic
heart failure, valsartan preserved right ventricular function, compared to placebo. In another randomized clinical trial we
showed a beneficial effect of sports on the exercise capacity in patients with a systemic right ventricle. A separate, but
related section of the thesis explores the epidemiology, in particular the prevalence and survival of adults with congenital
heart disease in general.
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