Anomalous left coronary artery off the pulmonary artery
An anomalous left coronary artery off the pulmonary artery (ALCAPA) also known as the Bland-White-Garland syndrome (BWG) is a rare congenital coronary artery anomaly and is considered one of the most serious of such anomalies 4.
This abnormality only accounts for 0.25 - 0.5 % of all congenital cardiac anomalies 3.
It is often an isolated anomaly but can be associated with other cardiac anomalies in ~ 5% of cases. These include 3
Typically ALCAPA presents when infants are 1 - 2 months old (see pathophysiology below).
ALCAPA results when the left main coronary artery arises from the pulmonary trunk instead of the aorta. Function of the left main coronary arterial territory then often requires extensive collateral formation from the right coronary artery.
In the first month of life, physiologic pulmonary hypertension tends to preserve antegrade blood flow within the left coronary artery, and infants usually remain asymptomatic.
As pulmonary pressures drop, left-to-right shunting from the higher pressure left coronary arterial system to the lower pressure pulmonary arterial system begins to occur, and patients become symptomatic.
CTA allows direct visualisation of anomalous left main coronary arterial origin from the posterior aspect of the pulmonary artery. The right coronary artery may be unusually dilated and tortuous with evidence of collateral formation. Intercoronary collateral arteries along the external surface of the heart or within the interventricular septum may also be seen.
Treatment and prognosis
Prognosis depends significantly of extent of collateral formation, however most infant die within the 1st year of birth 4. Death is usually due to circulatory insufficiency from left ventricular dysfunction or mitral valve incompetence, myocardial infarction, or life-threatening cardiac dysrhythmias 3.
Early surgical repair is potentially curative.
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- 1. Fierens C, Budts W, Denef B et-al. A 72 year old woman with ALCAPA. Heart. 2000;83 (1): E2. Heart (link) - Free text at pubmed - Pubmed citation
- 2. Awasthy N, Marwah A, Sharma R et-al. Anomalous origin of the left coronary artery from the pulmonary artery with patent ductus arteriosus: a must to recognize entity. Eur J Echocardiogr. 2010;11 (8): E31. doi:10.1093/ejechocard/jeq039 - Pubmed citation
- 3. Khanna A, Torigian DA, Ferrari VA et-al. Anomalous origin of the left coronary artery from the pulmonary artery in adulthood on CT and MRI. AJR Am J Roentgenol. 2005;185 (2): 326-9. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Kim SY, Seo JB, Do KH et-al. Coronary artery anomalies: classification and ECG-gated multi-detector row CT findings with angiographic correlation. Radiographics. 26 (2): 317-33. doi:10.1148/rg.262055068 - Pubmed citation
Synonyms & Alternative Spellings
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|Bland-White-Garland syndrome (BWG)||✗|