Anomalous origin of left coronary artery from pulmonary artery (ALCAPA): A case report
Devraj Kandel1, Irfa Mustafa1, Kritisha Rajlawot1
1Department of Radiodiagnosis and Imaging, Shahid Gangalal National Heart Centre, Bansbari, Bagmati +977, Kathmandu, Nepal.
Insights
Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a rare congenital heart defect. Early diagnosis via CT angiography is crucial for surgical intervention and preventing myocardial ischemia.
Area of Science:
- Cardiology
- Congenital Heart Defects
- Pediatric Cardiology
Background:
- Anomalous origin of left coronary artery from pulmonary artery (ALCAPA), or Bland-White-Garland Syndrome, is a rare congenital heart defect (0.25%-0.5%).
- It can lead to coronary steal phenomenon, left ventricular dysfunction, myocardial ischemia, and infarction in children.
- While often isolated, ALCAPA can co-occur with other cardiac anomalies in 5% of cases.
Observation:
- This case report details a 7-year-old female diagnosed with ALCAPA.
- The patient presented with collateral circulation between the right coronary artery (RCA) and left coronary artery (LCA).
- Additional findings included juxtaposition of the left atrial appendage, an anomaly typically associated with other major congenital heart diseases.
Findings:
- The presented case highlights a rare association between ALCAPA and juxtaposition of the left atrial appendage.
- Collateral formation between RCA and LCA was observed, indicating compensatory mechanisms.
- Multislice CT angiography facilitated early and accurate diagnosis.
Implications:
- Early diagnosis of ALCAPA is critical for timely surgical correction, preventing severe cardiac complications.
- Surgical intervention remains the definitive treatment for ALCAPA.
- This case expands the understanding of rare cardiac anomaly associations and diagnostic imaging utility.
Abstract:
Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) also known as Bland-White-Garland Syndrome is a rare anomaly of coronary arteries comprising of 0.25%-0.5% of all congenital heart defects with a prevalence of 1 in every 300,000 live births. Its clinical significance lies in the possibility of resultant coronary steal phenomenon with a left-to-right shunt causing aberrant left ventricular perfusion which may ultimately lead to myocardial ischemia and infarction in children having the abnormality. ALCAPA may manifest as an isolated defect but in 5% of cases it may be associated with other cardiac anomalies such as atrial septal defect, ventricular septal defect, and aortic coarctation. We present a case of 7 years female with ALCAPA with collaterals between RCA and LCA and additional findings of juxtaposition of left atrial appendage. Juxtaposition of atrial appendage is associated with some major congenital heart diseases, transposition of great vessels being the common one. In our case, however, juxtaposition of left atrial appendage is associated with ALCAPA. Surgery is the definite treatment modality for ALCAPA available till date. Early diagnosis of ALCAPA with the help of multislice CT angiography is always good for the patient to prevent the possible grave consequences.
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