Anomalous left coronary artery from the pulmonary artery
Updates to Article Attributes
Anomalous left coronary artery off the pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome (BWG) is a rare congenital coronary artery anomaly and is considered one of the most serious of such anomalies4
There are two forms based on onset of disease each of which has different manifestations and outcomes 5.
- infant type
- adult type
Epidemiology
This abnormality only accounts for 0.25-0.5% of all congenital cardiac anomalies 3.
Associations
It is often an isolated anomaly but can be associated with other cardiac anomalies in approximately~ 5% of cases, including 3:
Clinical presentation
In the infantile type, ALCAPA presents typically when infants are 1-2 months old (see pathophysiologypathology below).
PathophysiologyPathology
ALCAPA results when the left main coronary artery arises from the pulmonary trunk instead of the ascending aorta. FunctionThe 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, leftleft-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.
Radiographic features
Cardiac CT
ECG-gated cardiac CT 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 ofon the extent of collateral formation, however most, most infants 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.
-<p><strong>Anomalous left coronary artery off the pulmonary artery (ALCAPA)</strong>, also known as <strong>Bland-White-Garland syndrome (BWG)</strong> is a rare <a href="/articles/congenital-coronary-artery-anomalies">congenital coronary artery anomaly</a> and is considered one of the most serious of such anomalies <sup>4</sup>.</p><p>There are two forms based on onset of disease each of which has different manifestations and outcomes <sup>5</sup>.</p><ul>- +<p><strong>Anomalous left coronary artery off the pulmonary artery (ALCAPA)</strong>, also known as <strong>Bland-White-Garland syndrome (BWG)</strong> is a rare <a href="/articles/congenital-coronary-artery-anomalies">congenital coronary artery anomaly</a> and is considered one of the most serious of such anomalies.</p><p>There are two forms based on onset of disease each of which has different manifestations and outcomes <sup>5</sup>.</p><ul>
-</ul><h4>Epidemiology</h4><p>This abnormality only accounts for 0.25-0.5% of all <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiac anomalies</a> <sup>3</sup>.</p><h5>Associations</h5><p>It is often an isolated anomaly but can be associated with other cardiac anomalies in approximately 5% of cases, including <sup>3</sup>:</p><ul>- +</ul><h4>Epidemiology</h4><p>This abnormality only accounts for 0.25-0.5% of all <a href="/articles/congenital-cardiovascular-anomalies">congenital cardiac anomalies</a> <sup>3</sup>.</p><h5>Associations</h5><p>It is often an isolated anomaly but can be associated with other cardiac anomalies in ~ 5% of cases <sup>3</sup>:</p><ul>
-</ul><h4>Clinical presentation</h4><p>In the infantile type, ALCAPA presents typically when infants are 1-2 months old (see pathophysiology below). </p><h4>Pathophysiology</h4><p>ALCAPA results when the <a href="/articles/left-main-coronary-artery-1">left main coronary artery</a> arises from the <a href="/articles/pulmonary-trunk">pulmonary trunk</a> instead of the <a href="/articles/ascending-aorta">ascending aorta</a>. Function of the left main coronary arterial territory then often requires extensive collateral formation from the right coronary artery.</p><p>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. </p><p>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. </p><h4>Radiographic features</h4><h5>Cardiac CT</h5><p>ECG-gated cardiac CT allows direct visualisation of anomalous left main coronary arterial origin from the posterior aspect of the pulmonary artery. The<a href="/articles/right-coronary-artery"> right coronary artery</a> 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. </p><h4>Treatment and prognosis</h4><p>Prognosis depends significantly of extent of collateral formation, however most infants die within the 1<sup>st</sup> year of birth <sup>4</sup>. Death is usually due to circulatory insufficiency from left ventricular dysfunction or mitral valve incompetence, myocardial infarction, or life-threatening cardiac dysrhythmias <sup>3</sup>. Early surgical repair is potentially curative. </p>- +</ul><h4>Clinical presentation</h4><p>In the infantile type, ALCAPA presents typically when infants are 1-2 months old (see pathology below). </p><h4>Pathology</h4><p>ALCAPA results when the <a href="/articles/left-main-coronary-artery-1">left main coronary artery</a> arises from the <a href="/articles/pulmonary-trunk">pulmonary trunk</a> instead of the <a href="/articles/ascending-aorta">ascending aorta</a>. The function of the left main coronary arterial territory then often requires extensive collateral formation from the <a title="Right coronary artery" href="/articles/right-coronary-artery">right coronary artery</a>.</p><p>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. </p><p>As pulmonary pressures drop, <a title="Cardiac shunts" href="/articles/cardiovascular-shunts">left-to-right shunting</a> from the higher pressure left coronary arterial system to the lower pressure pulmonary arterial system begins to occur, and patients become symptomatic. </p><h4>Radiographic features</h4><h5>Cardiac CT</h5><p>ECG-gated cardiac CT allows direct visualisation of anomalous left main coronary arterial origin from the posterior aspect of the pulmonary artery. The<a href="/articles/right-coronary-artery"> right coronary artery</a> 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. </p><h4>Treatment and prognosis</h4><p>Prognosis depends significantly on the extent of collateral formation, however, most infants die within the 1<sup>st</sup> year of birth <sup>4</sup>. Death is usually due to circulatory insufficiency from left ventricular dysfunction or mitral valve incompetence, myocardial infarction, or life-threatening cardiac dysrhythmias <sup>3</sup>. Early surgical repair is potentially curative. </p>
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