Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in a Morbidly Obese Patient Presenting with Chest Pain

Abstract

Patient: Female, 58Final Diagnosis: Anomalous origin of RCA from PA with reversible myocardial ischemia of the inferior wallSymptoms: Chest pain • shortness of breathMedication: —Clinical Procedure: —Specialty: CardiologyObjective:Congenital defects/diseasesBackground:Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital anomaly with an incidence of 0.002%.Case Report:A 58-year-old African American female with a history of diabetes mellitus, hyperlipidemia, and hypertension was evaluated for shortness of breath and chest heaviness. On physical examination, she was found to be morbidly obese. Her blood pressure was 160/90 mmHg. There were no carotid bruits or jugular venous distension. Cardiac auscultation showed distant heart sounds with no audible murmurs. Lower extremity examination showed +1 edema with weak pedal pulses. ECG showed non-specific ST segment and T-wave changes. Echocardiogram demonstrated dysfunction grade I with preserved ejection fraction. An adenosine nuclear study showed an area of reversible ischemia of the inferior wall. Selective left coronary angiography showed the left coronary artery (LCA) originating from the left sinus of Valsalva. From the LCA, the left anterior descending and the left circumflex coronary arteries arose in a typical course. The right coronary artery (RCA) was visualized in a retrograde fashion via collaterals originating from the left coronary system and it drained into the pulmonary artery. On aortic root angiography, the origin of the RCA could not be determined. The patient’s surgical risk was deemed unacceptably high and she was not considered a surgical candidate. Her symptoms were controlled conservatively.Conclusions:By reporting this case, we shed some light on a rare congenital anomaly involving the coronary arteries. Variable presentations have been described for ARCAPA, however, many patients remain asymptomatic. Diagnosis can be confirmed by coronary angiography. Surgical correction is the definitive treatment.

Incidental finding of an ARCAPA during angiography

“…5 Symptoms are usually related to the direction of coronary flow (usually from the coronary to the pulmonary artery, left to right, due to pressure difference) and to collateral circulation. 5,7…”

“…The onset of most symptoms is in the first decade of life, or there are no symptoms throughout the whole life, characterizing, in some cases, the malformation as a casual finding. [2][3][4][5][6][7][8][9] In the aforementioned case, the patient is oligosymptomatic from the cardiovascular point of view except for family complaint due to the advanced dementia. It is worth mentioning that he remained completely asymptomatic until the sixth decade of life, an uncommon fact to individuals with such findings.…”

“…Among the congenital heart diseases, the anomalous coronary artery origin is even more uncommon, and one of the most severe conditions, ranging from 0.2 to 1.5% in the general population, [1][2][3] while the anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is even rarer, with a prevalence of 0.002%. 1,[4][5][6][7][8][9] ARCAPA was first described in 1885 by St. John Brooks, in two cases of autopsy. [5][6][7][8] The natural course may be insidious if there is collateral coronary circulation, which, due to the low pulmonary resistance, allows the flow from the left to the right.…”

“…1,[4][5][6][7][8][9] ARCAPA was first described in 1885 by St. John Brooks, in two cases of autopsy. [5][6][7][8] The natural course may be insidious if there is collateral coronary circulation, which, due to the low pulmonary resistance, allows the flow from the left to the right. 2 In general, the main symptoms of the anomalous origin of the coronary arteries are angina, heart failure, dyspnea, myocardial infarction and sudden death.…”

“…2 In general, the main symptoms of the anomalous origin of the coronary arteries are angina, heart failure, dyspnea, myocardial infarction and sudden death. 1,[5][6][7][8] This anomaly can be present in association with other structural and congenital heart diseases, such as tetralogy of Fallot or aorto-pulmonary window in 22% of cases. 9 This case report describes an incidental finding of an ARCAPA during angiography in an oligosymptomatic 76-years-old male patient.…”