From Fetal Aortic Valvuloplasty to Ross Operation: An Exciting Journey

Fetuses with critical left ventricular outflow tract obstruction (LVOTO) without ventricular septal defect are at risk of developing signs of an evolving hypoplastic left heart syndrome. Besides reduced flow across the left ventricular outflow tract and impaired left ventricular function, abnormal size of the left ventricle (either hypoplastic or severely dilated), abnormal left-to-right shunt across the atrial septum, and retrograde flow in the aortic arch can be observed. Other left heart structures may also be affected, including the mitral valve, subaortic region, and aortic arch. Fetal aortic valvuloplasty (FAV) can relieve left ventricular outflow obstruction, improve left ventricular haemodynamics, and promote left ventricular growth.1,2 In selected patients, single ventricle palliation can be avoided and a biventricular circulation achieved. Nevertheless, most patients after FAV will need postnatal surgery to enable functional improvement. In cases of severe LVOTO and duct-dependent systemic circulation postnatally, an early Ross–Konno procedure is a radical and sustainable solution with growth potential to decompress the stressed left ventricle.3–5