Anomalous Origin of a Right Coronary Artery from Pulmonary Artery

Abstract

Congenital defects of the coronary arteries are noted in 0.2–1.4% of the general population. The first case of an anomalous origin of right coronary artery from pulmonary artery (ARCAPA) was described by Brooks in 1885. ARCAPA has an overall incidence of 0.002% in the general population. Most of the cases are asymptomatic; however, it can lead to serious complications such as heart failure, ischemia, and sudden death. A 57-year-old man presented to the cardiologist's office with complaints of shortness of breath and fatigue. The patient also had a previous history of coronary stents and heart failure. Initially, he was evaluated with a stress test which was reported as abnormal. The patient then underwent an invasive coronary angiography that revealed anomalous origin of the right coronary artery (RCA) and multivessel disease. Cardiothoracic surgery evaluated the patient and coronary artery bypass graft was performed. During the surgery, the anomalous origin of RCA from the pulmonary artery was identified and was successfully corrected by reimplanting the RCA into the ascending aorta. The anomalous origin of RCA is a rare yet life-threatening condition. The RCA due to its location of origin from the pulmonary artery tends to be a low-pressure vessel with a very thin and fragile wall. It also serves as a retrograde venous conduit from the left coronary circulation into the pulmonary artery. This connection results in a left-to-right shunt that explains the increase in oxygen saturation in the pulmonary artery and the high cardiac output which is normally seen in these cases. The clinical presentation can vary from coronary ischemia to heart failure or sudden death. Therefore, surgical correction is recommended even in asymptomatic patients. We present a case of an anomalous origin of RCA from the pulmonary artery which, unlike the origin of left coronary from pulmonary artery, is very rare. Patients with this condition should have early correction even if they are asymptomatic in order to prevent long-term complications.

Long-term outcomes after repair for anomalous right coronary artery from the pulmonary artery

“…However, given the benign course of the reimplanted ARCAPA in the simple cases, we expect that their outcomes will likely be defined mostly by the underlying CHD. 22 One patient with ToF had evidence of complete heart block post-surgical repair of ToF. Albeit coronary perfusion abnormalities in the distribution of the RCA may contribute to the heart block; in our patient, it is likely related to the surgical repair of ToF.…”

Cardiac imaging findings in anomalous origin of the coronary arteries from the pulmonary artery; narrative review of the literature

“…After screening based on title and abstract and removing duplicates, 34 papers were selected. Finally, 25 papers including 27 cases were eligible for our study after full-text reading [ 3 – 28 ]. The main characteristics of each case report are listed in Table 1 .…”

“…Although most cases of ARCAPA have isolated lesions, approximately 40% of patients have concomitant congenital cardiac defects. Aortopulmonary window is the most common associated congenital lesion with ARCAPA accounting for one-fourth of cases [ 1 , 3 , 6 , 14 , 34 ]. Other associated lesions include VSD, atrial septal defect (ASD), tetralogy of Fallot, patent ductus arteriosus, aberrant right subclavian artery, and bicuspid aortic valve [ 32 , 34 ].…”

“…Patients who were not surgically treated had shorter life spans, and besides surgical correction have low postoperative mortality of 2–3%. Surgical treatment of ARCAPA has 2 purposes, elimination of left to right shunt or “coronary steal” and establishment of dual coronary circulation from the aorta [ 3 , 6 ]. Formerly, ligation of the anomalous coronary artery was often used, and oxygenation of RCA territory was dependent on the collateral vessels, but this method was associated with high early and late mortality [ 33 ].…”

“…Echocardiography, multislice computed tomography angiography (CTA), and magnetic resonance angiography (MRA) are generally used to diagnose ARCAPA. Patients with ARCAPA are usually asymptomatic and may be incidentally detected when undergoing paraclinical examinations for other problems, a common example is a coronary angiography following chest pain [ 3 , 4 ].…”