In utero intervention for severe congenital heart disease

Abstract

The aim of foetal cardiac therapy is to treat an abnormality at the developmental stage so that the process of cardiac growth, which is complex and relies on the volume and direction of circulating blood as well as genetic determinants, can continue. In reality, most cardiac interventions are palliative; hence, major abnormalities are still present at birth. Nevertheless, tangible benefits following successful foetal intervention include improved haemodynamics and reduction in secondary damage leading to better postnatal outcomes. In cases of semilunar valve stenosis, or atresia, foetal valvuloplasty aims to achieve a biventricular, rather than univentricular, circulation. Opening and stenting a restrictive atrial foramen may preserve the pulmonary function in cases of hypoplastic left heart syndrome, thereby increasing the chances of successful postnatal surgery. More recent endeavours include percutaneous implantation of a miniaturised pacemaker to treat complete heart block and the promotion of left-sided heart growth by chronic maternal hyperoxygenation. The true clinical benefit of these interventions over natural history remains uncertain because of the paucity of appropriate randomised controlled trials (RCTs). Foetal cardiac therapy must now move from a pioneering approach to one that is supported by evidence, as has been done successfully for other foetal therapies.

Keywords: Congenital heart disease; Foetal cardiac therapy; Foetal pacing; Foetal valvuloplasty; Maternal hyperoxygenation; Restrictive atrial foramen stenting.