Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism
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Published on: November 4, 2025
Sun Hyang Lee1, Bo Sang Kwon1, Gi Beom Kim1
1Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.
Insights
Left isomerism (LI) patients require lifelong monitoring for cardiac and non-cardiac issues. Key factors influencing survival include preoperative dysrhythmia and atrioventricular valve regurgitation.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Left isomerism (LI) is a complex congenital condition affecting cardiac and systemic anatomy.
- Understanding LI patient management is crucial for improving long-term outcomes.
Purpose of the Study:
- To analyze patient management outcomes for left isomerism (LI).
- To identify factors impacting survival and prognosis in LI patients.
Main Methods:
- Retrospective review of 76 patients diagnosed with LI between 1982 and 2014.
- Analysis of cardiac and extracardiac anomalies, surgical procedures, and survival data.
Main Results:
- 38.1% had univentricular heart, 56.5% had biventricular anomalies, 5.2% had normal hearts.
- Pulmonary arteriovenous fistulas (PAVFs) developed in 24% of patients after Kawashima procedures.
- 10, 20, and 30-year survival rates were 87%, 84%, and 76% respectively.
- Preoperative dysrhythmia and AV valve regurgitation were linked to late mortality.
- Late mortality was higher in single-ventricle patients after age 18.
Conclusions:
- LI patients necessitate vigilant, long-term follow-up.
- Monitoring should encompass late cardiovascular complications (dysrhythmia, valve issues, PAVFs) and systemic manifestations.
- Proactive management can mitigate risks and improve prognosis.
Background And Objectives:
We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis.
Subjects And Methods:
We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014.
Results:
Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age.
Conclusion:
Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.
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