Left circumflex coronary artery from the pulmonary artery in scimitar syndrome
Ilaria Bo1, Thomas Semple2,3, Emma Cheasty2
1Department of Paediatric Cardiology, Royal Brompton Hospital, Sydney Street, London, SW3 6NP, UK.
Insights
Scimitar syndrome patients may have a higher incidence of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), specifically the left circumflex artery. Cardiovascular CT is recommended for early detection in these cases.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Scimitar syndrome is a rare cardiopulmonary abnormality.
- Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect.
Observation:
- This study investigated the incidence of left circumflex ALCAPA in scimitar syndrome patients.
- A review of 54 scimitar syndrome patients' medical records and imaging was conducted.
- Three patients (5.5%) were diagnosed with ALCAPA, specifically the left circumflex coronary artery (LCx).
Findings:
- The incidence of LCx-ALCAPA in scimitar syndrome patients was found to be 5.5%.
- In all identified cases, the anomalous coronary artery was the LCx, originating near the pulmonary arterial bifurcation.
- This suggests a potentially higher prevalence of LCx-ALCAPA in scimitar syndrome than previously recognized.
Implications:
- Patients with scimitar syndrome, particularly those with ischemic symptoms, should be screened for ALCAPA.
- Cardiovascular CT is proposed as the preferred initial diagnostic tool due to its noninvasive nature and ability to image both cardiac and pulmonary structures.
- Early diagnosis and intervention for ALCAPA in scimitar syndrome patients can improve outcomes.
Background:
Scimitar syndrome is a rare combination of cardiopulmonary abnormalities found in 1-3 per 1000 live births. Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is only found in 1 in 250-400 congenital heart disease patients.
Objective:
We aimed to investigate the incidence of left circumflex ALCAPA within our referral center's cohort of scimitar syndrome patients.
Materials And Methods:
A review of medical records, cardiac imaging and operative notes from all patients diagnosed with scimitar syndrome at our center between 1992 and 2016 was undertaken and all imaging reviewed.
Results:
Fifty-four patients with scimitar syndrome and imaging were identified. Of these, 3 patients (1 male and 2 female) with ALCAPA were identified, representing an incidence of 5.5% (95% confidence interval [CI] 0-11.67%). In all three cases, the anomalous coronary arising from the pulmonary artery was the left circumflex coronary artery (LCx) and the point of origin was close to the pulmonary arterial bifurcation.
Conclusion:
We hypothesize that the prevalence of LCx-ALCAPA, in the setting of scimitar syndrome, may be greater than previously thought. We suggest that any patient with scimitar syndrome, especially with evidence of ischaemia, should be investigated for ALCAPA. Given its noninvasive nature and simultaneous imaging of the lungs, we suggest that cardiovascular CT is the most appropriate first-line investigation for these patients.
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