Introduction

Heart failure (HF) is currently recognised as one of the most clinically important events resulting from the prolonged survival to adulthood of patients with congenital heart disease (ACHD). Hospitalisations involving HF have increased 91% over the last two decades,1 with an impact on rehospitalisations and deaths during follow-up. According to data extracted from the Dutch CONCOR Registry,2 patients with CHD first admitted for HF have a fivefold higher mortality risk than those with no admissions; more recently, a 25% risk of death within 1 year following first HF admission has been reported by Lal et al.3 Consequently, a steady increase in the proportion of HF-related deaths with ageing has been described and HF is currently cited as the leading cause of cardiac death in patients with ACHD.4

Despite the contribution of HF to lifelong mortality and morbidity, management strategies remain challenging. Correction of residual lesions may be of benefit for treating ventricular dysfunction. However, the patients with ACHD have been traditionally excluded from HF trials and recruiting a sufficient number of patients for specifically ACHD-dedicated clinical studies with hard endpoints may be very difficult considering the heterogeneity of this patient group. Thus, these studies are frequently unpowered to detect effects of the drugs and HF medical therapy frequently rests on the extrapolation of acquired HF guidelines.5 6 Otherwise, the interplay among the unique CHD haemodynamics, biventricular or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic affectation makes …