Anomalous left coronary artery connected to the pulmonary artery associated with other cardiac defects: a difficult
Daniela Laux1, Claire Bertail, Fanny Bajolle
1Centre de Référence Malformations Cardiaques Congénitales Complexes-M3C-CCML, Department of Congenital Cardiac Surgery, Centre Chirurgical Marie Lannelongue, 133 Avenue de la Résistance, Le Plessis Robinson, France, d.laux@ccml.fr.
Insights
Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is often misdiagnosed with other heart defects, leading to poor surgical outcomes. Early diagnosis and treatment are crucial for improving survival rates in these complex pediatric cases.
Area of Science:
- Cardiology
- Pediatric Cardiac Surgery
- Congenital Heart Disease
Background:
- Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) is a rare congenital heart defect.
- ALCAPA can be associated with other complex cardiac anomalies, complicating diagnosis and management.
- Preoperative diagnosis of combined ALCAPA and other defects remains challenging.
Purpose of the Study:
- To evaluate the diagnostic challenges, surgical outcomes, and survival rates of patients with ALCAPA associated with other congenital heart defects.
- To identify factors contributing to misdiagnosis and their impact on patient outcomes.
- To analyze the spectrum of associated cardiac defects in ALCAPA patients.
Main Methods:
- A retrospective, bicentric study assessed 12 patients with ALCAPA and associated cardiac defects from 1987 to 2012.
- Data collected included associated defects, timing of diagnosis, surgical intervention, and patient outcomes.
- Analysis focused on the relationship between diagnostic accuracy and postoperative survival.
Main Results:
- Coarctation of the aorta (n=5) and tetralogy of Fallot (n=3) were the most common associated defects.
- Only 4 out of 12 patients received a complete preoperative diagnosis of both ALCAPA and the associated defect.
- Postoperative mortality was high at 58%, with half of deaths occurring within 30 days, often linked to delayed diagnosis.
Conclusions:
- ALCAPA associated with other cardiac defects is frequently misdiagnosed preoperatively, often due to masked myocardial ischemia.
- Delayed diagnosis of the coronary anomaly significantly compromises survival.
- Improved diagnostic strategies and timely surgical intervention are essential for better outcomes in these complex pediatric cardiac cases.
Abstract:
Anomalous left coronary artery connected to the pulmonary artery (ALCAPA) can be associated rarely with other congenital heart defects. The preoperative joint diagnosis is challenging. From 1987 to 2012, a retrospective bicentric assessment of 12 patients with ALCAPA related to other cardiac defects focused on the associated heart defect, the moment of complete diagnosis related to surgery, and outcome. Coarctation was the most frequently associated heart defect (n = 5) followed by tetralogy of Fallot with or without pulmonary atresia (n = 3). The study group comprised one case of hypoplastic left heart syndrome, one right aortic arch, one congenital mitral malformation, and one infant with divided left atrium and anomalous pulmonary venous return. Only four patients had a complete diagnosis of both the cardiac defect and the coronary abnormality before surgery. In two cases, the coronary anomaly was discovered during surgery performed for another cardiac defect and treated at the same time. The diagnosis of the six remaining patients was determined after cardiac repair. Of the 12 patients, 7 (58 %) died after surgery. Half of these patients died within the first 30 days after repair. At this writing, the remaining patients are in good health after a median follow-up period of 5.4 years (range, 2.1-8.5 years). This study confirmed that ALCAPA associated with other cardiac defects often is misdiagnosed before surgery, mostly due to specific hemodynamics masking myocardial ischemia preoperatively. Survival was compromised due to the unrecognized diagnosis of an associated coronary abnormality but also because of midterm complications related to the other cardiac defects.
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