Treatment.
Ligation or percutaneous occlusion of the ARCAPA have been performed in the past with relief of the left-to-right shunt but with concerns about myocardial ischemia . The most common surgical strategy nowadays is coronary reimplantation. With good results and low risk profile in experienced centers, it guarantees antegrade and oxygenated blood flow to the myocardium, establishing dual coronary artery system and preventing the steal phenomenon .
Indications for surgery of ARCAPA are based on general consensus, since for the rarity of the disease randomized trials and observational studies are lacking. In adults, according to the 2020 ESC guidelines, surgical intervention due to ARCAPA is needed when symptoms or ventricular dysfunction or other signs of myocardial ischemia are present as a consequence of the malformation . Since ARCAPA can lead to increased risk of myocardial infarction and sudden cardiac death even in asymptomatic patients, surgical correction is recommended in all cases also in infancy and adulthood. Symptomatic patients should receive surgical correction (RCA reimplantation onto the aorta) as soon as possible to relief myocardial damage, unless, especially older patients, deemed inoperable and good candidates for ligation or occlusion of ARCAPA . Asymptomatic patients should receive elective surgical reimplantation : this approach is practically applied in 90% of asymptomatic patients and in our center accordingly. In neonates, usually asymptomatic, elective repair should be planned when they reach a good weight in the first months of life. Our patient is planned to receive surgery in few months (around 6 kg).