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Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery
Serdar Basgoze1, Bahar Temur1, Osman Guvenc2
1Department of Cardiovascular Surgery, Faculty of Medicine, Atakent Hospital, Acibadem Mehmet Ali Aydinlar University, İstanbul, Turkey.
Insights
Laterality anomalies, particularly right atrial isomerism (RAI), present significant challenges with poor survival rates. Early intervention like ductal stenting can be effective in neonates with specific conditions.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Laterality anomalies are frequently linked to severe cardiac defects.
- Understanding the demographics, procedures, and outcomes of these anomalies is crucial for patient management.
Purpose of the Study:
- To analyze demographic properties, procedural types, associated anomalies, and early to mid-term prognosis of four distinct laterality anomalies.
- To identify risk factors for mortality in patients with laterality anomalies.
Main Methods:
- A cohort of 64 patients with laterality anomalies was studied between July 2014 and July 2020.
- Patients were categorized into situs solitus dextrocardia (SSD), situs inversus (SI), right atrial isomerism (RAI), and left atrial isomerism (LAI).
- Follow-up averaged 19.06 months, with analysis of procedural outcomes and survival rates.
Main Results:
- Early postoperative mortality was 15.8% across 107 procedures, with 12 deaths in neonates.
- Right atrial isomerism (RAI) was the most prevalent anomaly (45.3%) and had the highest mortality (48.3%).
- Three-year survival rates varied significantly: 85% for LAI, 78.7% for SI, 55.8% for SSD, and 38% for RAI.
- Independent risk factors for early mortality included mechanical ventilation, kidney injury, RAI, and complex neonatal surgery.
Conclusions:
- Laterality anomalies often present with univentricular physiology, posing significant management challenges.
- Right atrial isomerism (RAI) is the most common anomaly and is associated with the poorest outcomes.
- Ductal stenting is a viable initial intervention for selected neonates, while complex neonatal surgeries carry a high mortality risk.
Objective:
Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed.
Methods:
A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1-72) months.
Results:
Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality.
Conclusion:
Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.
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