Adult congenital heart disease When left seems right and right is wrong

Congenital heart disease (CHD) is the most common birth defect and affects approximately 9 per 1,000 newborns. Major advances in the management of children with CHD has yielded a dramatic change in age distribution, with more adults living with CHD nowadays than children. With increased longevity, caregivers need to be prepared to diagnose and treat late complications of this growing and aging patient population, who —in contrast to acquired heart disease— more commonly have right-sided (RV) complications. In this thesis, we addressed the role of the RV in adult CHD in the following clinical settings: in the subpulmonic position in pulmonary arterial hypertension (PAH-CHD), in the subaortic position in transposition of the great arteries (TGA), and when subjected to hemodynamic stress during exercise training.
Effective collaborations at both national and international levels have enabled us to challenge the existing paradigm that RV failure is inevitable in CHD. In TGA patients, we observed slow disease progression to end-stage heart failure or death. Several drugs (i.e. selexipag and valsartan) showed less beneficial effects in patients with PAH-CHD or TGA respectively than expected from landmark trials in PAH and LV failure. Exercise training however seemed to be safe, even in those with severe CHD. Lastly, we developed several simplified prediction tools for adverse outcome to help identify those at greatest risk.
Better understanding of the risk factors and treatment options for the RV in patients with CHD is highly relevant and must continue to be the focus of intensive research in future.