Pulmonary Atresia With Intact Ventricular Septum. Perforation and Pulmonary Vavuloplasty Using a Modified Mechanical Technique. Medium-Term Follow-Up

Introduction and objectives. In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. We report our experience with a modified mechanical technique that gave good results. Methods. Between November 2001 and October 2006, valve opening was carried out successfully in 11 consecutive neonates with a favorable anatomy (ie, Alwi groups A and B, and tricuspid valve Z-score ‐1.1 [1.3]). The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 [1.2] mm. Results. Valve opening was achieved in all patients, and right ventricular (RV) systolic pressure fell from 97 [17] mm Hg to 48 [13] mm Hg (P<.001). No pericardial effusion or cardiac tamponade was observed, though 1 neonate died 24 hours after the procedure due to pulmonary embolism. Six patients (54%) were discharged without any further intervention, while 4 (36%) required an additional increase in pulmonary blood flow. During the follow-up period of 25 [21] months, 2 patients died. Eight (72%) survived and were in New York Heart Association functional class 1. Two required additional surgery on the outflow tract, one of whom also needed a one-and-a-half ventricular repair. Data indicate that the valves remain open as RV structures grow, though without any change in the tricuspid valve Z-score. Conclusions. Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. Modification of the standard mechanical