Pediatric Case Challenge: Alarming Symptoms in a Toddler Who Had Myocarditis and Recently Immigrated

Shah Azmoon, MD; Matthew Budoff, MD; David Atkinson, MD


January 17, 2023

More than 35 years after ALCAPA was described by Bland, White, and Garland, Sabiston and colleagues[8] first showed that retrograde flow into the PA via an anomalous coronary artery could be effectively treated by ligation of the anomalous coronary artery at the junction of the PA and direct anastomosis of the anomalous coronary artery from the PA to the aorta. Surgical correction is necessary to avoid further strain on the heart and progressive cardiomyopathy.

As described by Takeuchi and colleagues[9] in 1979, the creation of an intrapulmonary aortocoronary tunnel may be required in young patients with anomalous coronary arteries that are in positions not suitable for direct transfer. This procedure, however, may result in tunnel stenosis, aortic regurgitation, or PA stenosis.[10] When significant cardiac dysfunction is present, an evaluation for cardiac transplantation may be necessary. The prognosis after surgical correction is usually good, with improvement of global left ventricular dysfunction or mitral regurgitation; however, patients are still susceptible to atheromatous and nonatheromatous coronary stenoses.

Because of the delay in diagnosis and the history of significant cardiomyopathy, the patient in this case was referred to a nearby transplantation hospital and underwent a cardiac transplantation. At 2 years posttransplant, significant improvement in the patient's symptoms was noted; however, the patient and her family moved out of state shortly thereafter and were lost to follow-up. With this case of ALCAPA, the authors hope to draw attention to the need for suspicion and early recognition of coronary artery anomalies when presented with clinical symptoms of heart failure or ischemia in infants.


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