Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair"

Ann Thorac Surg. 1995 Dec;60(6):1762-4. doi: 10.1016/0003-4975(95)00764-4.

Abstract

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 post-operatively.

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.

MeSH terms

  • Cardiac Surgical Procedures / methods
  • Child
  • Child, Preschool
  • Follow-Up Studies
  • Heart Septum / pathology
  • Heart Ventricles / pathology
  • Heart Ventricles / surgery*
  • Humans
  • Infant
  • Infant, Newborn
  • Palliative Care
  • Pulmonary Atresia / pathology
  • Pulmonary Atresia / surgery*
  • Tricuspid Valve / pathology