Anomalous left coronary artery from the pulmonary artery
Citation, DOI & article data
Anomalous left coronary artery from 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 severe of such anomalies.
There are two forms, based on the onset of the disease, each of which has different manifestations and outcomes 5:
infants experience myocardial infarction and congestive heart failure
90% die within the 1st year of life
manifests in adults
results in chronic myocardial ischemia and dysrhythmias
a cause of sudden cardiac death
This abnormality only accounts for 0.25-0.5% of all congenital cardiac anomalies 3.
It is most often an isolated anomaly, associated with other cardiac anomalies in only ~5% of cases 3,7:
In the infantile type, ALCAPA presents typically when infants are 1-2 months old. Presenting complaints are typically nonspecific and include diaphoresis, irritability, wheezing and respiratory distress. Precordial auscultation may reveal a murmur.
deep (>5 mm), narrow Q waves
typically in the lateral leads I, aVL, V5-6
most suggestive feature on the ECG, but may be absent in up to 45% of patients 10
high left ventricular voltage 8
meeting voltage criteria of left ventricular hypertrophy
characteristic of adolescents and older children
may be accompanied by left axis deviation
poor R wave progression 9
defined by an R wave height <3 mm in lead V3
Either of two etiologies can be responsible for ALCAPA:
the bulbus cordis undergoes abnormal septation into the aorta and pulmonary trunk
persistence of the pulmonary buds with concomitant involution of the aortic buds that are precursors of the coronary arteries
During the first month of life, physiologic pulmonary hypertension tends to preserve antegrade blood flow within the left coronary artery, and infants usually remain asymptomatic. Shortly thereafter, pulmonary artery pressure, resistance, and oxygen content decrease. Thus, the left ventricle receives blood with low oxygen content at low pressure, causing myocardial ischemia. Further increases in myocardial oxygen consumption leads to infarction of the anterolateral left ventricular free wall, often with resultant mitral valve insufficiency in approximately 80-85% of cases (infantile type).
In 10-15% of patients however, these events stimulate the development of collateral circulation via the right coronary artery to the anomalous left coronary circulation, thus these patients can reach adulthood. The disease most commonly manifests in adulthood as late-onset rhythm disorders due to the altered cardiac electric currents. Diminished pulmonary vascular resistance results in flow reversal in the left coronary artery into the pulmonary trunk (i.e. coronary steal phenomenon). Congestive heart failure (CHF) is the end result of left ventricular dysfunction in combination with significant mitral insufficiency.
ECG-gated cardiac CT allows direct visualization 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 on the extent of collateral formation, however, most infants die within the first 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. The Takeuchi procedure involves the creation of an aortopulmonary window and an intrapulmonary tunnel that baffles the aorta to the ostium of the anomalous left coronary artery.
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