ALCAPA in Children with Complex Congenital Heart Disease: A Multicenter Study

Area of Science:

• Pediatric Cardiology
• Congenital Heart Disease Surgery
• Medical Diagnostics

Background:

• Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect.
• Complex ALCAPA involves associated cardiac anomalies, potentially complicating diagnosis and treatment.
• Understanding outcomes in complex ALCAPA is crucial for surgical planning and patient management.

Purpose of the Study:

• To characterize the clinical presentation and surgical outcomes of children with complex ALCAPA.
• To compare the outcomes of complex ALCAPA cases with those of isolated ALCAPA.
• To identify factors contributing to morbidity and mortality in complex ALCAPA.

Main Methods:

• Retrospective analysis of pediatric ALCAPA cases undergoing cardiac surgery from 2009-2018 across 21 centers.
• Inclusion of 258 patients with ALCAPA, identifying 10 cases (3.9%) as complex.
• Statistical comparison of outcomes between complex and isolated ALCAPA groups using Fisher's exact test.

Main Results:

• Complex ALCAPA was associated with various cardiac lesions including HLHS, scimitar syndrome, VSD with aortic coarctation, and others.
• Preoperative diagnosis was rare in complex ALCAPA (1/10), with many diagnosed intraoperatively or postoperatively.
• Complex ALCAPA patients showed significantly higher rates of ECMO support (50% vs. 12%), CPR (30% vs. 6%), and operative mortality (50% vs. 3%) compared to isolated ALCAPA.

Conclusions:

• Complex ALCAPA is an uncommon but severe condition with a high rate of delayed diagnosis.
• Surgical repair of complex ALCAPA is associated with substantially increased postoperative morbidity and mortality.
• Improved preoperative diagnostic strategies for complex ALCAPA are warranted to improve patient outcomes.