Diagnostic Considerations
Important considerations
The most serious, yet avoidable, complication is the mistaking of symptoms in the patient older than several months who presents with anomalous left coronary artery from the pulmonary artery (ALCAPA) as idiopathic dilated cardiomyopathy [9, 10] or end stage myocarditis. [9] The initial presentation may be entirely similar but the treatments are dramatically different. The clinical and initial laboratory findings may be remarkably similar. If there is significant pulmonary artery hypertension, the diagnostic electrocardiographic (ECG) findings may not be apparent. Careful definition of the origin of the coronary arteries by echo-Doppler ultrasonographic studies in every patient presenting with the above clinical scenario should be undertaken. Selective cine-aortography to evaluate the origin of coronary arteries is no longer routinely necessary because of the usefulness of echocardiographic studies.
A high index of suspicion is necessary to make the diagnosis of ALCAPA, as surgical treatment is extremely successful and results in an excellent long-term prognosis.
Special concerns
With successful surgical revascularization and resolution of myocardial dysfunction in female patients, pregnancy and normal vaginal delivery are not contraindicated.
In patients with persistent myocardial dysfunction, mitral valve insufficiency, or coronary ischemia, the ability to carry a pregnancy to term varies. These patients should be considered high risk and require close observation throughout the pregnancy by both a cardiologist and a perinatologist because the increase in blood volume may be deleterious to the patient's hemodynamic status. A normal vaginal delivery may also be contraindicated in this situation.
In a relatively recent study, the pregnancy and delivery outcomes of six patients who had coronary artery reimplantation for ALCAPA were examined. [11] Six women delivered 10 babies, of which 8 babies were delivered vaginally and 2 were by cesarean section because of maternal congestive heart failure (CHF). All did well with the exception of one cesarean section patient, with both maternal and infant complications. The authors concluded that pregnancy is safe in asymptomatic repaired ALCAPA. Pregnant patients with symptomatic CHF secondary to myocardial damage should be carefully monitored and managed. [11]
Differential Diagnoses
-
Preoperative electrocardiogram in a 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery demonstrating pathologic Q waves in leads I and aVL and diffuse ST-T wave changes consistent with an anterolateral infarction.
-
Electrocardiogram in 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery 17 months following successful surgical revascularization, demonstrating complete resolution of the anterolateral infarction pattern and ST-T wave changes.
-
Two-dimensional echocardiographic image (parasternal short axis view) in a patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The left coronary artery (white arrow) appears to course towards the main pulmonary artery (MPA) just above the pulmonary valve and not to the aortic root (Ao). RV = Right ventricle.
-
Two-dimensional echocardiographic image with color flow mapping (parasternal short axis view) in the same patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The addition of color flow mapping to the 2-dimensional image demonstrates abnormal flow reversal within the left coronary artery (white arrows) towards the main pulmonary artery (MPA) just above the pulmonary valve. RV = Right ventricle. Ao = Aortic root.
-
Doppler interrogation of the abnormal color flow jet is depicted, demonstrating abnormal flow within the main pulmonary artery towards the transducer in diastole, which represents runoff from the anomalous left coronary artery (large white arrowhead). Small white arrow: Normal antegrade main pulmonary artery flow in systole. MPA = Main pulmonary artery.
-
Aortogram in a patient with suspected anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Frontal (left panel) and lateral (right panel) images demonstrating an enlarged right coronary artery (small white arrow), which fills a small left coronary system (solid arrow head) via collaterals with eventual faint opacification of the main pulmonary artery (not demonstrated in this frame).
-
Main pulmonary artery angiogram demonstrating the technique of stop flow angiography. There is retrograde opacification of the entire left coronary artery system, which originates from the distal main pulmonary artery (MPA), including the anterior descending (solid white arrowhead) and circumflex (small white arrow) branches. Left panel: Frontal image. Right panel: Lateral image.
