Congenital coronary artery anomaly in an adult: Double Barrel Left Coronary System and Coronary Artery Fistula Connecting a giant left circumflex artery to the coronary sinus

ABSTRACT

Introduction@#Coronary anomalies are rare congenital anatomic patterns affecting approximately one percent of the general population. Most of the cases may be asymptomatic and are never detected. Rarely, they result in severe life threatening consequences, thus, the diagnostic challenge is recognizing the abnormality early. Cardiac imaging modalities such as echocardiography, coronary computed tomography angiography (CTA), and coronary angiography can help us in the diagnosis.@*Case@#A 49-year-old female who initially presented with dyspnea and managed as rheumatic heart disease and hypertension. An echocardiogram finding prompted further investigation with coronary CTA and coronary angiography which identified the coronary artery anomalies. She was advised surgery but was unable to comply. Accordingly, medical management of her heart failure symptoms was pursued. She eventually succumbed to multiorgan failure as the complications of the fistula set in, three years after she had her initial presentation.@*Discussion@#The normal coronary anatomy consists of the the right and left coronary arteries that originate from the aorta. Our patient has two coronary artery anomalies: one is the double barrel left coronary system - her left anterior descending artery and left circumflex artery (LCX) originate directly from the aorta instead of from the left coronary artery. Second, is the coronary artery fistula (CAF), with her LCX draining into the right atrium through the coronary sinus. CAFs tend to grow with age, and if untreated, can cause clinical symptoms in older patients. The consequences of the patient’s abnormal connection may have caused volume overload to the right sided chambers resulting to heart failure. In adults with a congenital heart disease such as the CAF, it is recommended that if a continuous murmur is present, it should be defined either by echocardiography, magnetic resonance imaging, CTA, or cardiac catheterization.@*Conclusion@#A high level of suspicion for a CAF as a cause of an adult patient’s progressive heart failure can lead to timely diagnosis and optimal interventions.