Second trimester fetuses with critical aortic stenosis (AS), dilated left ventricles (LV) with endocardial fibroelastosis and reverse aortic arch flow will have a postnatal univentricular circulation.
Since December 2001 we have attempted 15 fetal aortic valvuloplasties in 14 fetuses (23+4 to 32+5 weeks) with these criteria. In 3 fetuses impaired right ventricular filling had resulted in advanced end-stage heart failure with hydrops. Percutaneous ultrasound guided valvuloplasty was technical successful in 9/15 procedures (60%) or 9/14 patients (64%). Overall mortality was 3/15 (20%), in the last 13 procedures 7,7%. Of the 5 patients with no technical success 2 died in-utero and 3 ended up as hypoplastic left heart syndrome. Of the 9 fetuses with technical success 7 (78%) patients now have a biventricular circulation after birth, 1 had a sudden IUD, 1 received a hybrid stage I. All of the 7 biventricular patients had to undergo another aortic valve dilation in the first week of life. In 4 of them this was the only procedure so far with a follow-up of 1,2, 24 and 29 months respectively. One child needed a coarctation repair, 3 underwent a Ross-Konno operation between 1 and 3 months of age, 1 of them had the mitral valve replaced at the age of 6 months. All 3 fetuses with hydrops had a successful procedure and hydrops resolved within 3-5 weeks.
In selected fetuses with critical AS in-utero valvuloplasty has the potential to reverse end-stage heart failure and to save the LV for a postnatal biventricular circulation.