Introduction
Anomalous right coronary artery from pulmonary artery (ARCAPA) is an extremely rare congenital coronary lesion in which the right coronary artery is anomalously connected to the pulmonary artery instead of the aorta. This leads to abnormal coronary artery flow and needs surgical correction for the anomaly. Due to the rarity of the lesion, ARCAPA and its outcomes are only described in isolated case reports and case series. Here, we report the in-hospital and long-term outcomes of patients who underwent surgical repair for ARCAPA within the Pediatric Cardiac Care Consortium (PCCC), a US-based registry of interventions for congenital heart diseases.

Methods
We queried the PCCC for patients who underwent surgical repair of ARCAPA at <21 years of age. Outcomes were obtained from the PCCC records and after linkage with the US National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN).

Results
25 cases of ARCAPA that were treated with surgery were found in the PCCC with a median age at diagnosis of 4.60 years (IQR 1.67-9.69). There were 23 patients who had “simple” ARCAPA diagnoses (ARCAPA diagnosed with fistulas, atrial or ventricular septal defects, patent ductus arterioses, and patent foramen ovales) and 2 “complex” ARCAPA diagnoses (ARCAPA diagnosed with hypoplastic left heart, transposition of great vessels, and tetralogy of Fallot). Patients with simple ARCAPA had reimplantation surgeries and the complex patients had surgeries to repair their respective congenital heart diseases. Patients with complex ARCAPA were diagnosed at an earlier age than simple ARCAPA (5.00 yrs vs. 0.11 yrs). One patient from the simple cohort died from heroin overdose ten years after reimplantation. 15 of the 25 cases were submitted to the NDI with median follow-up time of 18.68 (IQR 17.43-24.57). Follow-up characteristics of echocardiography and angiography was available for 9 total patients showing good functioning ventricles with innocuous dilations of coronary arteries.

Conclusions
ARCAPA is a rare finding. Many patients are asymptomatic at time of presentation. Work up is mostly done for patients to investigate a murmur or other congenital cardiac lesion. The PCCC is a reliable collection of information that presents data on patients diagnosed with ARCAPAs’ courses and follow-up data, while also tracing surgical intervention for ARCAPA, which is usually done soon after diagnosis. From the small numbered pediatric cohort, reimplantation proves to have a reliable long-term survival rate, and via the literature search, follow-up seems to have no further complications post-surgery. The PCCC did not have remodeling characteristics of the coronary artery nor ventricular perfusion at follow-up and should be evaluated in a prospected multicenter study.