Left ventricular performance of pulmonary atresia with intact ventricular septum after right heart bypass surgery

Objective: The left ventricular performance in patients with pulmonary atresia with intact ventricular septum who were Fontan candidates before and after the bidirectional Glenn procedure and a staged total cavopulmonary connection was compared with that in patients with tricuspid atresia. Methods: Contractility (end-systolic elastance), afterload (effective arterial elastance), and ventricular efficiency (ventriculoarterial coupling, arterial elastance/endsystolic elastance ratio), and the ratio of stroke work and pressure-volume area were approximated on the basis of cardiac cathetefization data before the bidirectional Glenn procedure, before and after staged total cavopulmonary connection, and approximately 1 year after the completion of total cavopulmonary connection in 20 patients with pulmonary atresia with intact ventricular septum and 21 patients with tricuspid atresia. Results: The end-systolic elastance of the pulmonary atresia with intact ventricular septum group was significantly inferior to that of the tricuspid atresia group after bidirectional Glenn procedure and total cavopulmonary connection (I year after total cavopulmonary connection 1.85 +/- 0.51 mm Hg (.) m(2 .) mL(-1) vs 2.84 +/- 0.96 mm Hg (.) m(2) (.) mL(-1), P <.01). The arterial elastance was not different between groups throughout the assessment period and tended to increase in a stepwise fashion after bidirectional Glenn procedure and total cavopulmonary connection. The arterial elastance/end-systolic elastance ratio and ratio of stroke work and pressure-volume area of the pulmonary atresia with intact ventricular septum group tended to worsen, whereas those of the tricuspid atresia group tended to improve. The difference reached statistical significance I year after total cavopulmonary connection (1.15 +/- 0.35 vs 0.82 +/- 0.23 and 64.2% +/- 6.7% vs 71.3% +/- 5.7%, respectively, P <.05 and P <.05, respectively). Conclusion: The contractility and ventricular efficiency of patients with pulmonary atresia with intact ventricular septum are inferior to those of patients with tricuspid atresia after bidirectional Glenn procedure and total cavopulmonary connection. A high-pressure residual right ventricle may impair the left ventricular performance of patients with pulmonary atresia with intact ventricular septum after bidirectional Glenn procedure and total cavopulmonary connection.