| Background: In patients with congenital heart disease (CHD), often mechanical or biological prosthetic valves are implanted. These patients differ from adults who have prosthetic valves implanted for acquired valve disease (e.g. differences in age, lifestyle, variety of valve location). The prevalence of pulmonary and tricuspid prosthetic valves is high. The prevalence of prosthesis-patient-mismatch (PPM) is probably high because often valves were implanted during childhood. In patients with acquired valve disease PPM is associated with decreased survival and increased incidence of heart failure. Data about the prevalence of PPM in adults with CHD are lacking. The probable high prevalence of PPM and the complicated history of many CHD patients may predestinate them for complications such as heart failure and arrhythmias. The influence of prosthetic valve characteristics (type, location, size/PPM) in adults with CHD on functional outcome and quality of life has however not been investigated. Moreover, data about the long-term complications of prosthetic valves in adults with CHD are scarce. For example, in the pulmonary position usually biological valves are implanted because of a presumed high incidence of thrombo-embolism associated with mechanical valves, but scientific evidence confirming this presumption is lacking. Hypothesis: 1. Prosthetic valve characteristics (type; location; size resulting in the presence/absence of PPM) are related to functional outcome and quality of life. 2. The incidence/spectrum of prosthetic valve-related complications in adults with CHD differs from populations with acquired valve disease. Objectives: To investigate the relation between characteristics of valve prosthesis on functional outcome and quality of life in adult patients with CHD. To investigate the prevalence and predictors of PPM in a population with CHD. To investigate the incidence of valve prosthesis-related complications in a population with CHD (re-operation, valve thrombosis, bleeding complications, hemolysis, paravalvular regurgitation, endocarditis, arrhythmias, pregnancy-related complications, heart failure). To investigate the relation between prosthesis related complications and valve type. Methods: In the CONCOR database currently 726 patients with prosthetic valves are included. These patients will be asked to participate in a multicentre cross-sectional study. Primary outcomes are VO2max and quality of life. Secondary outcomes are the prevalence of PPM and the incidence of valve related complications. Patient files of CHD patients with prosthetic valves identified from the CONCOR database will be studied for past medical history including surgical procedures and for the occurrence of valve-related complications. The cross-sectional evaluation comprises history, physical examination, VO2max, quality of life questionnaires, echocardiogram including prosthetic valve area, MRI (ventricular volumes and mass), laboratory evaluation (including NT-pro-BNP). Expected results: We expect that prosthetic valve characteristics are related to functional outcome and quality of life. We expect the spectrum of prosthetic valve-related complications and the prevalence of PPM in our CHD population to differ from complications and prevalence of PPM as reported in the literature in populations with acquired valve disease. We expect that the outcome of mechanical pulmonary prosthetic valves compares favourably to biological prosthetic valves. Objectives Primary objective: -To investigate the relation between characteristics of valve prosthesis on functional outcome and quality of life in adult patients with congenital heart disease. Secondary objectives: -To investigate the prevalence of prosthesis-patient-mismatch in an adult population with congenital heart disease. - To determine predictors of prosthesis-patient-mismatch in an adult population with congenital heart disease. -To investigate the incidence of prosthesis related complications in an adult population with congenital heart disease (re-operation, valve thrombosis, bleeding complications, hemolysis, paravalvular regurgitation, endocarditis, arrhythmias, pregnancy-related complications, heart failure). - To investigate the relation between prosthesis related complications and valve type. Regarding to this issue, we will focus on the difference between mechanical and biological prosthetic valves in the pulmonary and tricuspid position. Relevance for cardiovascular diseases Type, location and size (resulting in the absence or presence of prosthesis-patient mismatch) of prosthetic valves are viewed as important factors determining post-operative cardiac function and quality of life. The incidence of specific complications is related to prosthetic valve characteristics. However, the impact of prosthetic valve characteristics on functional outcome, quality of life, and incidence of complications has not been studied in an adult population with CHD. We expect that prosthetic valve characteristics are related to functional outcome and quality of life in adults with CHD. We expect the spectrum of prosthetic valve-related complications and the prevalence of prosthesis-patient mismatch in our CHD population to differ from complications and prevalence of prosthesis-patient mismatch as reported in the literature in populations with acquired valve disease. Our results may influence the choice of valve prosthesis, the indication for more extensive surgery (e.g. annulus enlargement) and the indication for re-operation in patients with prosthesis-patient-mismatch. Keywords complications - exercise - prosthesis - quality of life - valves |
