Anomalous left coronary artery from the pulmonary artery (ALCAPA)
Easy fatigability. Echocardiography showed dilated coronary arteries suspicious for coronary fistula.
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The coronary arteries are tortuous and dilated. The right coronary artery arises from the anterior coronary sinus of the aortic root. The left main coronary artery arises from the main pulmonary artery. The left anterior descending and circumflex coronary arteries arise from the left main coronary artery. Multiple collaterals are seen. There is reflux of contrast from the left main coronary artery into the main pulmonary artery suggestive of reversal of flow. No significant stenosis or coronary fistula identified in this examination.
Overall findings are consistent with an anomalous left coronary artery from the pulmonary artery (ALCAPA).
Anomalous left coronary artery from the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare coronary artery anomaly that affects 1 in 300,000 live births 1. ALCAPA arises from either abnormal septation of the aorta and the pulmonary artery or from the persistence of aortic buds that form the coronary arteries 2. It can occur as an isolated anomaly or in conjunction with other lesions such as atrial septal defect, ventricular septal defect and coarctation of the aorta 3.
In fetal life, the pulmonary artery pressure equals systemic pressure, allowing for satisfactory myocardial perfusion from the pulmonary artery through the anomalous coronary artery. After birth, the pulmonary artery contains desaturated blood at a pressure that rapidly falls below systemic pressure. As pulmonary arterial pressure drops, the combination of low flow and desaturated blood causes myocardial ischemia, especially during exertion. Collateral vessels develop between the right and left coronary arteries and may provide adequate perfusion to the left myocardium. Further decrease in pulmonary arterial pressure results in a reversal of flow, as the left coronary artery drains from the right coronary artery, through the collaterals, into the pulmonary artery (left-to-right shunting). This is known as myocardial steal phenomenon, which may cause ischemia or infarction of the anterolateral left ventricular wall. Patients who survive to adulthood may develop a good collateral network with tortuous and dilated left and right coronary arteries 1-5.
- 1. Peña E, Nguyen ET, Merchant N et-al. ALCAPA syndrome: not just a pediatric disease. Radiographics. 2009;29 (2): 553-65. doi:10.1148/rg.292085059 - Pubmed citation
- 2. Lardhi AA. Anomalous origin of left coronary artery from pulmonary artery: A rare cause of myocardial infarction in children. J Family Community Med. 2010;17 (3): 113-6. doi:10.4103/1319-1683.74319 - Free text at pubmed - Pubmed citation
- 3. Brotherton H, Philip RK. Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort. Eur. J. Pediatr. 2007;167 (1): 43-6. doi:10.1007/s00431-007-0423-1 - Pubmed citation
- 4. Manoly I, Karangelis D, Viola N et-al. Repair of Bland-White-Garland syndrome via a modified technique. Tex Heart Inst J. 2014;41 (1): 48-50. doi:10.14503/THIJ-12-3112 - Free text at pubmed - Pubmed citation
- 5. Funabashi N, Takaoka H, Ishikawa K et-al. Bland-White-Garland syndrome diagnosed by computed tomography. Int. J. Cardiol. 2015;201: 465-8. doi:10.1016/j.ijcard.2015.08.004 - Pubmed citation