Tetralogy of fallot with diminutive pulmonary arteries: Preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries

AbstractObjectives. The study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries.Background. Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary stresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminative pulmonary arteries and pulmonary stenosis.Methods. Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995.Results. Initially, the Nakata index ranged from 20 to 98 mm2/m2(mean 67 ± 28 mm2/m2). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 ± 0.3), and the mean initial valve annulus Z score (−40 ± 1) increased to −3.3 ± 1.1 (p < 0.01). Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was −3.1 ± 0.7, and the aorta index increased to 143 ± 84 mm2/m2(p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 ± 2 years, right ventricular pressure was < 70% systemic in all patients and