Pulmonary atresia with intact ventricular septum: Long-term results of “One and a Half Ventricular Repair”

Background. Between 1982 and 1984, we successfully performed “one and a half ventricular repair” using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. Methods. In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from −5.2 to −6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years postoperatively. Results. All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. Conclusions. Although the lower limits of the tricuspid valve diameter for “one and a half ventricular repair” using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than −5.0. The results up to 10 years postoperatively are acceptable.