ABSTRACT
BACKGROUND: There are a number of surgical and interventional treatment options for infants with pulmonary atresia with intact ventricular septum (PAIVS). In our practice, we characterize coronary fistulae and interruptions with angiography in the newborn and have developed a strategy to safely decompress the right ventricle in association with ligation of fistulae if necessary. METHODS: All infants operated for PAIVS at age < 60 days from 1999 to 2018 were retrospectively studied. Pre- and postoperative variables were collected, angiograms were reviewed, and a territory score was created to grade the severity of coronary abnormalities. This study focused on the subgroup of patients who had early surgical decompression of the right ventricle. RESULTS: A total of 77 patients were included, with a mean follow-up of 8.6 years. Of these, 55 (71%) had coronary fistulae, including 28 (36%) with coronary artery interruption. Right ventricular decompression (RVD) was performed in 47 (60.5%) patients. There was no 30-day mortality in those who underwent RVD, whereas 6 (20%) without RVD died within 30 days (P = .003). Ten-year survival was 97.8% and 73.3% for RVD and non-RVD, respectively. In order to prevent coronary steal, 17 patients underwent coronary fistula ligation as their RV was decompressed with 100% early and late survival. CONCLUSION: Early and late survival in infants with PAIVS is better if the RV can be decompressed. Coronary fistula ligation with RVD has been introduced without an adverse outcome in selected patients with large fistulae.
