Coronary artery anomalies in children: subtype classification, age of onset, clinical outcomes, and prognostic implications

To investigate the clinical characteristics, treatment strategies, and prognostic outcomes of different coronary artery anomaly (CAA) subtypes in pediatric patients, with emphasis on risk stratification and the role of extracorporeal membrane oxygenation (ECMO) in acute management. A retrospective cohort study was conducted on 82 pediatric patients diagnosed with CAA at Central China Fuwai Cardiovascular Hospital between September 2020 and September 2024. Data on demographics, anatomical classification, clinical presentation, cardiac function, treatment modalities, and follow-up outcomes were systematically analyzed using ANOVA for continuous variables and chi-square tests for categorical variables. Among 82 patients, anomalous left coronary artery from the pulmonary artery (ALCAPA) comprised 39.0% (n = 32), anomalous right coronary artery from the left sinus (ARCA-LS) 42.7% (n = 35), and anomalous left coronary artery from the right sinus (ALCA-RS) 14.6% (n = 12), and other variants 3.7% (n = 3). Significant differences were observed in age at diagnosis (P < 0.001), with 78.1% of ALCAPA patients diagnosed in infancy (median age 0.8 years) versus adolescent presentation for ALCA-RS (median age 13.5 years). ALCA-RS demonstrated the highest risk profile with 41.7% presenting with EF < 30% and 33.3% requiring ECMO support. Surgical intervention rates varied significantly: ALCAPA 81.3%, ALCA-RS 66.7%, and ARCA-LS 34.3% (P < 0.01). Overall mortality was 3.7% (3/82), including sudden cardiac death in 2.4% (2/82) of patients, concentrated in high-risk subtypes. CAA subtypes demonstrate distinct clinical trajectories requiring individualized management strategies. Early surgical intervention for high-risk subtypes, particularly ALCAPA and ALCA-RS with intramural course, significantly improves outcomes. ECMO serves as critical rescue therapy for acute cardiac arrest, though long-term prognosis remains guarded. Comprehensive risk stratification based on anatomical features and cardiac function is essential for optimal patient management.

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Journal
BMC Pediatrics
Published
2026-03-02
DOI
https://doi.org/10.1186/s12887-026-06574-3
Primary Topic
Coronary Artery Anomalies
Type
article
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article

Coronary artery anomalies in children: subtype classification, age of onset, clinical outcomes, and prognostic implications

J N Li, X. Qian, Teng Wang, Yuping Xu et al.
BMC Pediatrics
Coronary Artery Anomalies
article

Coronary artery anomalies in children: subtype classification, age of onset, clinical outcomes, and prognostic implications

J N Li, X. Qian, Teng Wang, Yuping Xu, Fanwei Meng, Shujun Li, Jiasi Zhou, Weiqing Liu
article en

Abstract

To investigate the clinical characteristics, treatment strategies, and prognostic outcomes of different coronary artery anomaly (CAA) subtypes in pediatric patients, with emphasis on risk stratification and the role of extracorporeal membrane oxygenation (ECMO) in acute management. A retrospective cohort study was conducted on 82 pediatric patients diagnosed with CAA at Central China Fuwai Cardiovascular Hospital between September 2020 and September 2024. Data on demographics, anatomical classification, clinical presentation, cardiac function, treatment modalities, and follow-up outcomes were systematically analyzed using ANOVA for continuous variables and chi-square tests for categorical variables. Among 82 patients, anomalous left coronary artery from the pulmonary artery (ALCAPA) comprised 39.0% (n = 32), anomalous right coronary artery from the left sinus (ARCA-LS) 42.7% (n = 35), and anomalous left coronary artery from the right sinus (ALCA-RS) 14.6% (n = 12), and other variants 3.7% (n = 3). Significant differences were observed in age at diagnosis (P < 0.001), with 78.1% of ALCAPA patients diagnosed in infancy (median age 0.8 years) versus adolescent presentation for ALCA-RS (median age 13.5 years). ALCA-RS demonstrated the highest risk profile with 41.7% presenting with EF < 30% and 33.3% requiring ECMO support. Surgical intervention rates varied significantly: ALCAPA 81.3%, ALCA-RS 66.7%, and ARCA-LS 34.3% (P < 0.01). Overall mortality was 3.7% (3/82), including sudden cardiac death in 2.4% (2/82) of patients, concentrated in high-risk subtypes. CAA subtypes demonstrate distinct clinical trajectories requiring individualized management strategies. Early surgical intervention for high-risk subtypes, particularly ALCAPA and ALCA-RS with intramural course, significantly improves outcomes. ECMO serves as critical rescue therapy for acute cardiac arrest, though long-term prognosis remains guarded. Comprehensive risk stratification based on anatomical features and cardiac function is essential for optimal patient management.

BMC Pediatrics
First Affiliated Hospital of Xinxiang Medical University (CN), Zhengzhou Central Hospital (CN), Henan Medical University (CN)
Good health and well-being
Openalex Percentile: Top 37%
Coronary Artery Anomalies
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