EP09.15: Diagnosis, management and outcomes of right aortic arch: from fetal life to long‐term follow‐up at a tertiary centre

We collected clinical and instrumental data from 502 patients with RAA referred at our tertiary center. 157 patients (31.3%) had fetal diagnosis (medium follow-up 7.6 years). 330 (65%) had CHD and 89 (17%) genetic anomalies, with DiGeorge syndrome the most frequent (9.5%). Genetic disorders were significantly more frequent in those with CHD (23% versus 7.5%, p < 0.001). 58 (11%) had extracardiac anomalies. Among the RAA not forming CVR, left anterior ductus arteriosus with mirror-image (MI) branching pattern of epiaortic vessels (RAA/LADA/MI) was present in 29, 100% associated with CHD (conotruncal in 72%). Right ductus arteriosus (RAA/RDA/MI) was found in 8, 4 with complex CHD. CVR was present in 246 (49%): the most frequent were RAA, left posterior ductus arteriosus with aberrant left subclavian artery (RAA/LPDA/ALSA – 163 cases, 66%) and double aortic arch (DAA – 66 cases, 27%), Left posterior ductus arteriosus (LPDA) with MI branching (RAA/LPDA/MI – 12 cases, 5%) and aberrant left innominate artery (RAA/LPDA/ALIA – 5 cases, 2%) were rare. CVR were most frequently associated with minor CHD (26% versus 17% major CHD). At CT mean tracheal compression was 46% (57% for DAA, 43% for RAA/LPDA/MI. 109 (45%) were operated (median age 31.2 months). Three among 16 pts were re-operated for persistent symptomatic compression, the majority being DAA. RAA is commonly associated with CVR, asymptomatic in 2/3 of cases but requiring surgery in 50%. Symptoms resolves almost completely and reoperations are reserved to the DAA. RAA is present along with major CHD in half cases and these patients are at higher risk of genetic syndromes, however not frequent.