Early outcome of children with complex atrial isomerism undergoing uni-ventricular cardiac palliation.

Objective: To review the postoperative intensive care unit (ICU) course and early outcome of children with complex atrial isomeric hearts undergoing cardiac surgery for uni-ventricular heart repair.

Methods: A retrospective review and analysis of ICU course of pediatric patients with atrial isomerism admitted from January 2000 to December 2004 in King Abdul-Aziz Medical City, Riyadh, Kingdom of Saudi Arabia, who underwent uni-ventricular repair.

Results: During the study period, 18 (n=18) patients were identified to have complex congenital heart disease (CHD) associated with atrial isomerism. They were in the form of right atrial isomerism (n=12), and left atrial isomerism (n=6). Eight patients did not meet the inclusion criteria of the study and were excluded. Ten of the 18 patients fulfilled the study criteria and underwent first stage uni-ventricular heart repair with 8 survivals (80%). Three of the 8 operated survival cases underwent second stage repair with 2 survivals (66%) and one patient completed Fontan surgery with a good outcome. An uneventful ICU course was noted in 3 of 10 operated patients (30%). Complications were noted in (40%) of cases including sepsis (n=4), tracheostomy (n=1), prolonged mechanical ventilation >7days (n=2). Over 50% of the operated patients required prolonged ICU stay (>2 weeks).

Conclusion: Atrial isomerism is frequently associated with complex cardiac defects that often present in the neonatal stage and requires multiple staged cardiac surgeries. The surgical repair and peri-operative management of this group of patients can be difficult, challenging, and with potential risk of significant morbidity or mortality. In our setting, we found the outcome of children with atrial isomerism undergoing uni-ventricular palliation comparable to the literature.