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Published on: February 11, 2022
Early outcome of children with complex atrial isomerism undergoing uni-ventricular cardiac palliation
Giridhar V Santhanam1, Hani K Najm, Johannes P Duplessis
1Cardiac Science Department, King Abdul-Aziz Medical City, National Guard Health Affairs, Riyadh, Kingdom of Saudi Arabia.
Insights
Children with atrial isomerism and complex heart defects undergoing uni-ventricular repair had challenging but comparable outcomes to literature. Early results showed significant morbidity but also successful staged surgical palliation.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Atrial isomerism is a rare condition often associated with complex congenital heart disease (CHD).
- Management of these complex cardiac defects in children requires specialized surgical approaches, including uni-ventricular repair.
Purpose of the Study:
- To evaluate the intensive care unit (ICU) course and early outcomes of pediatric patients with atrial isomerism undergoing uni-ventricular heart repair.
- To assess the challenges and potential risks associated with surgical repair in this patient population.
Main Methods:
- Retrospective review of pediatric patients with atrial isomerism who underwent uni-ventricular repair between January 2000 and December 2004.
- Analysis of ICU course, complications, and early surgical outcomes.
Main Results:
- Ten patients underwent uni-ventricular repair, with an 80% survival rate for the first stage.
- Complications such as sepsis and prolonged mechanical ventilation occurred in 40% of cases.
- Over 50% of operated patients experienced prolonged ICU stays (>2 weeks).
Conclusions:
- Atrial isomerism frequently presents with complex cardiac defects requiring staged surgical repair.
- Surgical management and peri-operative care for these patients are challenging, carrying risks of morbidity and mortality.
- Outcomes for children with atrial isomerism undergoing uni-ventricular palliation in this setting were comparable to existing literature.
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.
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