Pediatric heart failure therapy: why β1-receptor blocker, tissue ACE-I and mineralocorticoid-receptor-blocker?

Pediatric heart failure (HF) treatment lagged behind the knowledge of pharmacological research and evidence-based clinical experience in adults. Considering the lack of prospective, double blind randomized studies in children, the review is focused on the preferred indication of specific beta 1-adrenoreceptor blockers (ARB), mineralocorticoid antagonists and tissue angiotensin-converting enzyme inhibitors (ACE-I). Our recommendations are based on the specificity in children, the effectiveness and the side-effect profile of HF-drugs, the receptor-physiological knowledge and the negative results of the few pediatric HF studies with an "evidence study label". In the interest of our pediatric patients, effective HF treatment has not longer to be postponed by balancing between evidence-based versus pathophysiology-based approach. At our institution, bisoprolol, lisinopril and spironolactone (BLS) are used treating HF in patients with left-right shunt lesions, reduced ejection fraction as well as during the inter-stage after HLHS-Hybrid approach. Chronic use of diuretics and fluid restriction is avoided, if always possible; intravascular volume deficiency stimulates further the neurohumoral axis. Pediatric HF needs to be treated with a strategy respecting the variable pathophysiology and the differences of receptor physiology between children and adult patients. The personalized treatment can be easily proofed by the surrogate parameters as heart rate, breath pattern, weight gain and image-derived parameters as well as biomarkers. Effective HF-therapy is also the basis for novel regenerative strategies in particular for young children with "end-stage" HF avoiding cardiac transplant or death.