In children, cardiac failure is most often due to CHDs. In the 1st week after birth, CHDs with ductus-dependent systemic circulation (such as severe aortic stenosis/aortic coarctation and hypoplastic left heart syndrome), in which the closure of the ductus arteriosus causes severe reduction of end-organ perfusion. After that period, CHDs provokes HF during follow-up for different mechanisms: pressure overload, volume overload, ischemia (coronary anomalies), pump failure (intrinsic miocardial dysfunction or effect of ventriculotomy), arrhythmias, pulmonary hypertension, and multiple mechanisms could intersect.

HF is the leading cause of death (25%) of adults with CHDs. In some CHDs the RV is systemic (exposed to systemic afterload) as example in congenitally corrected transposition of great arteries.

Take a look at the video below: an example of cardiac MRI of a patient with CCTGA without any symptoms of HF. Although patient is clinically asymptomatic, changes in the morphology of RV systemic can be noticed

CHD with RV systemic the therapeutical treatments options are different compared with LV systemic and in cases of single ventricle or Fontan circulation are multiple causes for ventricular dysfunction or failing circulation. By adulthood around 50% of patients with intact Fontan circulation will have HF.