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).