Anatomical and physiological assessment of a symptomatic anomalous origin of the right coronary artery from the pulmonary artery by noninvasive imaging examinations

Abstract

A 58-year-old female visited our hospital complaining of fatigue and repetitive pre-syncope during exercise. She had suffered with those symptoms for decades and had visited some hospitals where she did not get conclusive diagnosis. She was ultimately diagnosed as having subclinical ventricular septal defect unrelated to her symptoms. Echocardiography revealed normal ventricular function, but color Doppler showed multiple abnormal intramyocardial blood flow signals in ventricular septum and moderator band, which suggested abnormally developed coronary perfusion. Dilated left main trunk and abnormal color Doppler signal running into the main pulmonary trunk were also observed. Coronary computed tomography angiography revealed that right coronary artery originated from pulmonary artery and made connection to distal left anterior descending artery, which led to the final diagnosis of anomalous origin of the right coronary artery from the pulmonary artery. Exercise stress echocardiography showed wall motion abnormalities in inferior region which suggested ischemia in right coronary artery (RCA) territory. Her symptoms could be derived from the myocardial ischemia. She was referred to surgical intervention considering the possible risk of cardiac events including sudden death. Re-implantation of the abnormal RCA to the aorta was performed successfully. Her long-year persistent symptoms were completely alleviated, and repeat exercise stress echocardiography showed no evidence of ischemia. <Learning objective: We report an adult case of symptomatic anomalous origin of the right coronary artery from the pulmonary artery, who had not been given the correct diagnosis. Noninvasive diagnostic examinations including transthoracic Doppler echocardiography, exercise stress echocardiography and coronary computed tomography angiography contributed to the anatomical and physiological assessment of the rare congenital heart disease before and after the successful coronary re-implantation surgery.>.

Keywords: Congenital heart disease; Coronary artery disease; Coronary artery malformation; Exercise stress echocardiography; Noninvasive diagnosis.