History
Infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) usually do well for a short period then gradually become fussy and irritable. Typically, they may display pallor, irritability, and diaphoresis after feeding, which are often attributed to colic.
Signs and symptoms of congestive heart failure, including tachypnea, tachycardia, diaphoresis, and poor feeding, eventually ensue, leading to poor weight gain. Usually no obvious evidence of a systemic illness is noted.
In rare instances, children outgrow these symptoms and gradually become asymptomatic, although periodic dyspnea, angina pectoris, syncope, or sudden death may still occur in adulthood.
Physical Examination
If congestive heart failure (CHF) is present, the infant appears distressed and exhibits tachypnea, tachycardia, diaphoresis, and irritability.
Auscultation may demonstrate a systolic murmur of mitral valve regurgitation and, possibly, a diastolic rumble of relative mitral stenosis best located at the apical left precordial region. A moderate third heart sound may be heard.
Rarely, a soft continuous murmur may be detected at the upper left sternal border that is reminiscent of a coronary artery fistula or a small patent ductus arteriosus.
The left ventricular precordial impulse may appear prominent and displaced both inferiorly and laterally.
The second heart sound may seem narrowly split with increased intensity of the pulmonic component, if left ventricular failure causes pulmonary artery hypertension secondary to elevated left atrial pressure.
In cases of severe CHF, hepatic enlargement may be observed, and the peripheral pulses may be diminished in intensity secondary to low cardiac output.
-
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.
