The incidence of congenital heart disease (CHD) is estimated to vary between 4 to 50
per 1000 live births [1, 2] . This wide range of incidence is due to different phenotypes
of CHD, as well as the inclusion criteria used. All together, CHD can be seen as the most
common birth defect worldwide with approximately 1 million children born with a
CHD each year [3]. Epidemiological studies in the Netherlands report an incidence of 6
per 1000 children born with a CHD each year [4].
When looking at causes of infant death, congenital anomalies are the leading cause. One
third of these deaths are due to CHD [5]. However, the introduction of modern surgery
as well as improved treatment strategies for CHD resulted in a decline in mortality from
CHD [6]. In addition, death resulting form CHD shifted from newborns to young adults
[7]. Thus, this prolonged survival results in an increase in patients with CHD that need
treatment not only at birth, but also during adolescence and even during their adult
live. In a selected group of these patients, the structural abnormalities that define a
particular CHD phenotype results in prolonged RV pressure overload, such as Tetralogy
of Fallot, left hypoplastic heart syndrome or congenitally corrected transposition of
the great vessels. In addition, other diseases such as pulmonary hypertension or even
ischemic heart disease can result in increased RV loading conditions. The effects of
prolonged RV pressure overload on the heart’s function, as well as the effects on
molecular and cellular level is not yet fully understood. However, patient data suggest
that the RV is unable to cope with prolonged periods of (systemic) overload resulting
in RV failure [8-12]. Therefore, it is of utmost importance to understand the effects of
prolonged pressure overload on the RV in terms of ventricular function, as well as at
the molecular level.
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