Center Experience and Step-by-Step Approach for Fetal Pulmonary Valvuloplasty

In our center (the Children’s Heart Center Linz), the fetal cardiac intervention program was started in the year 2000. In fact, the very first procedure, which we performed, was in a fetus with pulmonary atresia with intact ventricular septum (PAIVS) at 28 weeks [1]. Since then a total of 91 interventions have been here conducted, the majority in fetuses with critical aortic stenosis, few in fetuses with hypoplastic left heart syndrome and restrictive foramen ovale, and altogether 20 procedures in 14 fetuses with PAIVS or critical pulmonary stenosis with intact ventricular septum. We reported our first case together with a case that was done in the UK in a publication in the year 2002. This fetus was thought to develop restriction at the atrial level with increased central venous pressures and imminent hydrops as indicated by severely abnormal venous Doppler waveforms – a complication that may result in fetal hydrops and intrauterine death. The fetus had a hypoplastic RV with growth arrest and supra-systemic right ventricular pressures, a significant tricuspid regurgitation (TR), and a favorable anatomy with a membranous atresia of the pulmonary valve and well-developed confluent pulmonary arteries. For this first procedure, we used a 16-gauge needle and managed to perforate the membrane between the RV and main pulmonary artery. Clear antegrade as well as retrograde flow across the pulmonary valve annulus was observed immediately after the procedure by color-flow and CW Doppler. This fetus was followed longitudinally in our center, and over the following weeks, significant changes in hemodynamics were documented: growth of RV long axis, growth of tricuspid valve annulus, change from a short monophasic to a longer biphasic RV inflow, as well as complete disappearance of the TR jet. Six weeks after the procedure, TR reappeared again with high velocities due to an increasing restenosis of the perforated pulmonary valve. This baby was delivered at 38 + 2 weeks; postnatally it received a pulmonary valvuloplasty, and because of a still borderline RV output, a 3.5 mm modified right BT shunt was placed. This shunt was successfully removed at the age of 8 months. Now 15 years later, this girl still has had no other interventions; she has mild to moderate pulmonary stenosis and mild pulmonary regurgitation with some dilation of the main pulmonary artery. She is on no medication, is very sportive, and has no exercise intolerance at all.