Abstract
Background: Outcomes of surgical repair of pulmonary atresia with intact ventricular septum(PAIVS)have improved with the adoption of appropriate approaches based on right ventricula(r RV)and coronary artery anatomy.Patients with a RV of adequate size and an RV independent coronary circulation have good surgical results after biventricular repai(r BVR).However,the long-term outcomes after BVR with regard to RV and left ventricula(r LV)function have not been clarified.
Objectives: To assess biventricular systolic function of PAIVS patients after BVR using cardiac MR(I cMR).
Methods: Four patients with PAIVS(P group)who were followed-up for more than 10 years after BVR in our institution were reviewed,and biventricular systolic function assessed by cMR was compared to that of the normal group(N group)who had no cardiac disease.
Results: Tricuspid valve diameter and right ventricular end-diastolic volume(RVEDV)at cardiac catheterization at birth and post-BVR increased from 73% ± 3.9%(mean ± SD)of the normal value to 88% ± 9.9% and from 45% ± 27% to 102% ± 49%,respectively.There were no significant differences in the RVEDV and LVEDV indices measured by cMR between the P and N groups,but the biventricular values of contractility were lower in the P group than in the N group(RV,P:N = 7.8 ± 3.7:16 ± 8.0,P = 0.06;LV,P:N = 36 ± 3.0:82 ± 49,P < 0.05).
Conclusions: PAIVS patients must be closely followed after BVR,because biventricular systolic function might be impaired even though biventricular volume may be comparable to normal.
