Anomalous right coronary origin from pulmonary artery in a 54 year old

51 yo African American presented to the hospital with chest pain. Her chest pain was atypical in nature without associated symptoms and was ruled out for myocardial infarction by enzymes. She has a past history of congestive heart failure with depressed ejection fraction of 25-30% but no further evaluation after treatment of congestive heart failure. 

Exam: Vitals stable, Alert, looks well, Chest: Clear, CVS: S1 S2 no extra heart sounds, elevated JVD at 12 cm. 

Lab Studies: EKG: Normal sinus rhythm, leftward axis, possible left and right atrial enlargement ECHO: Global depressed LV, EF: 30%, moderate TR, moderate pulmonary hypertension Right heart cath: RA 12, RV 60/28, PA mean 30, PCWP 26, no step up during saturation run Cath: mild disease at diagonal, anomalous right pulmonary artery from pulmonary artery CT Chest: With knowledge of the cath images, anomalous origin of the right coronary from the pulmonary artery was visible. 


NOTES:



Clinical Course
Medical treatment with ACE inhibitors, diuretics and beta-blocker was initiated. After discussion with the patient, cardiac surgery was performed to correct her anomalous right coronary artery. The anomalous right coronary artery was identified and reimplantation of the right coronary artery to the aorta was performed without complications. Patient was discharged 4 days post operatively. She returned at three weeks with a pericardial effusion that required drainage. Repeat echo at that time showed no improvement to that point in her depressed LV function. 
Comment
Anomalous origin of the right coronary artery from the pulmonary trunk is a rare congenital anomaly. Many cases have been reported in the literature but the incidence of the disease is unknown. Many patients with the anomaly are asymptomatic, however, risks of adverse outcome exist because of the abnormality of coronary blood flow. Intercoronary anastomoses are created blood flowing from the distal left coronary system to the right coronary artery in a retrograde fashion. These anastomoses represent low-resistance pathways between the aorta and pulmonary artery so that left to right shunting exists. This low-resistance channels that bypass the capillary bed also leads to coronary steal and promoting myocardial ischemia. 


Image 1

 Long axis transthoracic echo shows global left ventricular hypokinesis.

ECG in this patient whose right coronary arteru originates from the pulmonary artery.

CT scan shows aortic origin of the left main and pulmonary origin of the right coronary artery.