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Objective <p>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.</p> Methods <p>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.</p> Results <p>Among 82 patients, anomalous left coronary artery from the pulmonary artery (ALCAPA) comprised 39.0% (<i>n</i> = 32), anomalous right coronary artery from the left sinus (ARCA-LS) 42.7% (<i>n</i> = 35), and anomalous left coronary artery from the right sinus (ALCA-RS) 14.6% (<i>n</i> = 12), and other variants 3.7% (<i>n</i> = 3). Significant differences were observed in age at diagnosis (<i>P</i> &lt; 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 &lt; 30% and 33.3% requiring ECMO support. Surgical intervention rates varied significantly: ALCAPA 81.3%, ALCA-RS 66.7%, and ARCA-LS 34.3% (<i>P</i> &lt; 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.</p> Conclusion <p>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.</p>

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

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年3月2日

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  • Weiqing Liu,
  • Jiasi Zhou,
  • Yuping Xu,
  • Tuanjie Wang,
  • Xiaoliang Qian,
  • Fanwei Meng,
  • Jianchao Li,
  • Shujun Li

摘要

Objective

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.

Methods

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.

Results

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.

Conclusion

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.