Right Ventricle: Wrong Targets?

Pharmacotherapeutic algorithms developed to treat heart failure in adults with acquired heart disease have often provided the template for treatment of ventricular dysfunction or heart failure symptoms in patients with underlying congenital heart disease. Superficially at least, this is not completely irrational, because congenital patients often demonstrate similar levels of functional incapacity and a neurohomonal profile similar to that seen in acquired heart failure.1 Scratch beneath the surface, however, and the situation is much less clear. Systolic failure as the primary manifestation and driver of disease progression is rather rare, fundamentally the circulation in these patients is often quite different, and the rate of functional decline and adverse events tends to be substantially lower than that observed in the major studies of β-blockade and modifiers of the renin–angiotensin system in acquired heart failure. Consequently there is an ever-increasing catalog of, albeit small, randomized, controlled trials that have failed to show a significant clinical benefit of these therapies in congenital heart disease.2–6 The study by van der Bom et al,7 examining the role of valsartan in patients with a systemic right ventricle (RV) and published in this issue of Circulation , can now be added to that list.Article see p 322 In congenital heart disease with a biventricular physiology, there are 2 main situations where the circulation depends on the ability of the right ventricle (RV) to drive blood through the systemic vascular bed. These are transposition of the great arteries (TGA) after an atrial-level surgical correction and congenitally corrected transposition of the great arteries (ccTGA). In both settings, the RV is positioned beneath the aortic valve and is at high risk for long-term failure, the incidence of which increase with age. In each subgroup systemic RV dysfunction usually precedes the onset of clinical heart …