VALVULÆR PULMONAL STENOSE HOS BARN : LANGTIDSRESULTAT ETTER BEHANDLING MED BALLONGDILATASJON

Abstract

Summary

Background: v-PS is found in 8-10 % of all congenital heart disease. 80-90 % of all patients with pulmonary outflow obstuction have an isolated valvular pulmonary stenosis (v-PS). The aim of our study was to analyse the long term effect of balloon valvulotomy in patients with v-PS.

Material and methods: All the 46 patients included in our retrospective study were born between 1972 and 1992. Patients with diagnosis Noonan s syndrom, v-PS with dysplastic valv, v-PS with infundibular component, v-PS with ASD (atrium septum defect), v-PS with tricuspidal insuffisiency, v-PS withVSD (ventricle septum defect) were included in the study. Diagnosis were made by Doppler, they underwent balloon valvulotomy and were followed up with Doppler kontrols in an average of 10 years. The patient population was divided in two separate subgroups, with and without Noonan s syndrom.

Results and interpretation: Our study shows that the gradient across the pulmonary valve falls immediatly after the valvulotomy, but also that this gradient remains constant over time (10 years), with an unsignificant reduction of 0,7 m/s measured with Doppler in patients without Noonan s syndrom. For the group of Noonan patients who underwent this intervention 4 of 6 had positive immediate effect. The long time profile for this group was similar to the group without Noonan s syndrom. Only 8 patients had an increasing gradient across the pulmonary valve with an average of 0,5 m/s, 4 patients underwent this intervention twice because of restenosis, all the other had their gradient reduced without need of a second intervention. This means that balloon valvulotomy is an excellent way of treating v-PS in patients with or without Noonan syndrom and shows consequent immediate gradient reduction and that this effect is persistent over time (10 years in our study).