Abstract
Coronary artery anomalies are rare forms of congenital heart disease. Often seen in association with other congenital heart disease, isolated coronary anomalies can be present in otherwise structurally normal hearts. Anomalous right coronary artery arising from the pulmonary artery (ARCAPA) is a very rare phenomenon often diagnosed incidentally during evaluation for various symptoms. This peculiar entity has not been extensively defined in the literature since its first published appearance over a century ago. Many affected patients present later in adolescence or as adults after the coronary system has had ample time to dilate and compensate for the “stealing” left to right shunt.
There is an established increased risk of sudden death from ARCAPA related to long-standing myocardial ischemia. ARCAPA requires prompt surgical correction to ensure proper coronary perfusion pressures to the myocardium. The evolution of diagnostic testing, particularly the widespread use of echocardiography, has shortened the time to diagnosis, and pediatric surgical outcomes are generally excellent. Follow-up is not standardized but favors the long-term. Routine echocardiography, exercise stress testing, and 3-dimensional imaging all factor into decision making for assessing the degree of sports or exercise participation, duration of anticoagulation or antiplatelet therapy, and future surgical revisions.
Highlights
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A rare form of coronary artery anomalies is the anomalous right coronary artery from the pulmonary artery.
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Discovery of anomalous right coronary artery from the pulmonary artery remains an indication for surgical correction.
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A high index of suspicion is needed to diagnose coronary anomalies.
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Multiple imaging modalities are used to confirm the diagnoses.
1
Introduction
Anomalous right coronary originating from the pulmonary artery is an extremely rare congenital coronary abnormality. When first described by Brooks in 1885 [ ] , the malpositioned coronary was thought to be mainly an incidental finding at autopsy. Case reports have described the association of anomalous right coronary artery originating from the pulmonary artery with myocardial ischemia, syncope, dilated cardiomyopathy, and the risk for sudden death. With an estimated incidence of only 0.002 % in the general population [ ] , there is a limited definition of natural history, and authors have been unable to elucidate a clear presentation or pathognomonic feature. In one of the very few pediatric case series describing cases at a single institution over a 25 year period, 6 of the 7 cases were found in asymptomatic children [ ]. A more recent review by Modi et al. examined 99 case reports from 1950 to 2010, of which 56 cases were documented in patients 18 years of age or younger. Improvements in diagnostic imaging will increase identification of cases, potentially leading to interventions being performed earlier. Historically, medical management played a role in the care of these patients. However, with refinements in surgical technique and mainly positive outcomes, operative re-implantation of the right coronary into the right aortic sinus has become the standard therapy.
A detailed informed consent was obtained from the patient prior to completion and submission of this case report .
2
Case
A 17-year-old female was undergoing evaluation for suspected systemic lupus erythematosus. She had positive anti-nuclear antibodies and a maternal history of lupus. A referral to the pediatric cardiology clinic was prompted by her complaints of frequent episodes of sudden onset fatigue and tiredness and a history of multiple emergency room visits for chest pain and palpitations. There were no abnormal findings on her physical exam, but her electrocardiogram showed sinus rhythm with T-wave inversion in inferior and precordial leads ( Fig. 1 ). Echocardiography and cardiac computed tomography angiogram revealed origin of the right coronary artery from the main pulmonary artery ( Figs. 2 and 4 ), with diffuse dilation of the right coronary artery, left main coronary artery and left anterior descending artery ( Figs. 3 and 5 ). There were prominent collateral vessels in the right ventricle free wall, apex, and septum. There was an initial suggestion to perform exercise stress testing to assess inducible ischemia. However, given the patient’s current symptoms, it was felt that there would likely be sub-optimal performance, which could lead to a false sense of reassurance. A nuclear stress test was not considered at the time of presentation. On cardiac magnetic resonance imaging, there was no myocardial delayed enhancement to suggest prior myocardial infarction, myocardial inflammation/fibrosis, or infiltrative myocardial disease.
