A 2 day old neonate weighing 2.1 kg presented with respiratory distress and a systolic murmur. He was diagnosed with critical valvar pulmonary stenosis, intact ventricular septum with moderate RV dysfunction and mild LV dysfunction. There was right to left shunting across a stretched PFO and severe TR (gradient 74 mm Hg). The pulmonary valve annulus was 6 mm with a restrictive opening and an underestimated gradient of 45 mm Hg.

During cardiac catheterization, the pulmonary valve could not be crossed anterogradely from the RV. Aortic angiogram in lateral view showed a closed ductus. Prostaglandin E1 infusion was started (0.1 mcg/kg/min) and after 5 minutes, the ductus opened up. PDA was then crossed from the aortic end with a 4F JR catheter. The pulmonary valve was crossed retrogradely with a 0.014 X 190 PTCA wire which was stationed in the RV. The valve was dilated with a 7 X 2 mm mini Tyshak balloon with full disappearance of the waist. Post-balloon angiogram showed good opening of the pulmonary valve and RV-PA pressure gradient was reduced to 13 mm Hg. Prostaglandin infusion and ventilation were tapered off within 24 hours and the baby was discharged 3 days later.

Balloon pulmonary valvuloplasty is the recommended treatment for congenital pulmonary valve stenosis. In this case, as the procedure was performed on day 2 of life, only functional closure of the ductus had occurred. Thus, prostaglandin infusion effected reestablishment of ductal patency and allowed a retrograde approach through the stenosed pulmonary valve.