Long-term clinical outcome of atrial isomerism after univentricular repair

Abstract

Objectives: We retrospectively reviewed the long-term outcome of atrial isomerism patients after Fontan completion.

Methods: Since 1972, 58 patients underwent a palliative procedure prior to the Fontan-type operation. Twenty-eight out of 58 patients could not reach Fontan-type operation. Twenty-five patients underwent Fontan-type operation, and 12 of them expired less than five years after the Fontan completion. Eleven patients survived more than five years after the Fontan completion and were identified as long-term survivors. The mean follow-up period was 13+/-5 years.

Results: During follow-up period, four of the 11 patients expired. The actuarial survival rates at 10, 15, and 20 years after univentricular repair (UVR) were 100%, 71.4%, and 53.6%, respectively. The significant predictors of long-term survival by univariate analysis were the staged strategy (p=0.019), total cavo-pulmonary connection with extracardiac conduit (p=0.019), and the absence of postoperative common atrioventricular valve regurgitation (p=0.040). Six out of the seven present survivors showed New York Heart Association class I activity. All present survivors' mean percutaneous oxygen saturation, mean pulmonary arterial pressure, pulmonary capillary wedge pressure, single ventricular end diastolic volume index, and single ventricular ejection fraction were 88.8+/-6.8%, 11.0+/-2.6 mmHg, 5.8+/-2.0 mmHg, 104+/-37 mL/m2, and 52.0+/-6.5%, respectively.

Conclusions: There are still life-threatening problems 10 years after the UVR. However, the excellent performance status of the present long-term survivors suggests that these problems can all be overcome by the present strategies established for the Fontan-type operation.