P0397 - It’s Not Lupus! An Incidental Finding of Anomalous Right Coronary Artery Arising from the Pulmonary Artery in an Adolescent Rheumatology Patient

Coronary artery anomalies are a rare form of congenital heart disease. Most cases occur in association with other congenital heart disease, but isolated coronary anomalies occur in otherwise structurally normal hearts. Isolated anomalous right coronary artery arising from the pulmonary artery (ARCAPA) is a very rare phenomenon that is often diagnosed incidentally during evaluation for other diseases. It poses a challenging diagnostic puzzle due to its more insidious presentation with vague and nonspecific symptoms. This peculiar entity has not been extensively defined in the medical literature since it was first published appearance over a century ago, and it is generally discussed in case reports and case series. Many affected patients tend to present later in adolescence or as adults after the coronary system has had ample time to dilate and compensate for this left to right shunting lesion.
It has been established that there is an increased risk of sudden death from ARCAPA, due to effects from long-standing myocardial ischemia. Although ARCAPA does not have the more fear-inducing pathology and serious potential for early morbidity and mortality associated with the more popular anomalous left coronary from the pulmonary artery, when diagnosed, the recommendation remains prompt surgical correction to ensure proper coronary perfusion pressures for the myocardium. The evolution of diagnostic testing, particularly the widespread use of echocardiography, has shortened the time to diagnosis, and led to repair earlier in life. Corrective surgery is relatively straightforward, and pediatric outcomes are generally excellent. These patients, however, require follow-up, with no established care guidelines with regard to important indices such as duration for follow up, coronary artery surveillance testing, timing and degree of sports participation, and anticoagulation/antiplatelet therapy.
We present a case of a 17-year-old female who was referred to the pediatric cardiology clinic with a suspected diagnosis of systemic lupus erythematous. Her symptoms of sudden onset fatigue and low energy could have easily been attributed to this new diagnosis, however, a review of her medical history revealed multiple emergency room visits for chest pain and palpitations. Her physical exam was unremarkable, but her echocardiogram showed evidence of ARCAPA (figure 1). This was confirmed by cardiac computed tomography angiogram, which better delineated the diffusely dilated and tortuous coronary arteries (figure 2). While her magnetic resonance imaging revealed no evidence of prior ischemia or fibrosis, the dilated coronary arteries were consistent with coronary artery steal and collaterization. She had a successful reimplantation of the anomalous right coronary to the sinotubular junction of the aorta. The symptoms of sudden fatigue and exercise intolerance resolved, and subsequent exercise stress test showed no significant ectopy or ST segment changes. She has returned to her usual activities with no significant clinical sequalae.