Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Diagnoses and Surgical Results in 12 Pediatric Patients

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital condition. It responds well to early diagnosis and treatment, but otherwise the prognosis is poor. We present our case series of 12 patients (mean age, 2 ± 2.58 yr; age range, 2 mo-8 yr), emphasizing the diagnostic process and discussing our surgical results. The diagnosis of ALCAPA should be suspected in infants who have dilated cardiomyopathy with electrocardiographic changes that suggest ischemia, and in older children who have isolated mitral regurgitation. When clinical suspicion is high, the results of 2-dimensional echocardiography combined with color-flow Doppler studies in expert hands can establish the diagnosis, thus avoiding angiography in critically ill infants. The treatment of choice in our patients was transfer and reimplantation of the left coronary artery onto the ascending aorta. There were 2 deaths: both were infants in extremis who underwent emergency surgery. An older child with severe ventricular dysfunction was given mechanical ventricular assistance and then heart transplantation. As of this report, all 10 survivors remained well and asymptomatic.

Keywords: Abnormalities, multiple/diagnosis; cardiac surgical procedures/methods; cardiomyopathy, dilated/etiology/physiopathology; collateral circulation/physiology; coronary vessel anomalies/diagnosis/pathology/radiography/surgery; echocardiography, Doppler, color/methods; heart arrest/etiology; pulmonary artery/abnormalities/pathology; time factors; treatment outcome.

Fig. 1.

Electrocardiograms show signs of acute anterolateral myocardial infarction: deep Q waves, marked ST-segment elevation, and T-wave inversion in leads A) I and aVL (Patient 6) and B) V₄ through V₆ (Patient 8).

Fig. 2.

Two-dimensional echocardiograms. A) Patient 6. The parasternal short-axis view gives the erroneous impression that the left coronary artery emerges from the aortic root (arrow). In color-flow Doppler mode, diastolic flow from right to left (in blue) is seen inside the left coronary artery from the pulmonary artery. B) Patient 10. The parasternal short-axis view shows that the left coronary artery has no connection to the aorta despite their proximity and has retrograde diastolic flow (in blue) from right to left (asterisk). The dilated right coronary artery emerges from the aortic root (arrow). C) Patient 4. The parasternal short-axis view shows retrograde diastolic flow in the main pulmonary artery from the left coronary artery (in red) from left to right (arrow). The origin of the left coronary artery from the main pulmonary artery (asterisk) should not be mistaken for a persistent ductus arteriosus.

Fig. 3.

Patient 7. Two-dimensional echocardiograms (short-axis views in color-flow Doppler mode, with posterior angle to the apex) in A) mid septal and B) apical locations show diastolic or continuous flow (arrows) within the ventricular septum. These signs of intercoronary collateral vessels should not be mistaken for multiple ventricular septal defects.

Fig. 4.

A) Patient 10. Right-sided coronary angiogram shows a well-developed network of intercoronary collateral vessels (asterisk) that enable flow from the right coronary artery to the left coronary artery (arrow). B) Patient 12. Pulmonary arteriogram (posteroanterior projection) shows the left coronary artery and its division into the left circumflex and left anterior descending coronary arteries (arrow). C) Patient 11. Aortogram shows a dilated right coronary artery (arrow).