A 22-month-old boy was referred to pediatric cardiology for evaluation of a heart murmur. He was asymptomatic except for occasional wheezing with activity. On evaluation, he was found to have both systolic and diastolic murmurs. Electrocardiography demonstrated possible left ventricular hypertrophy, with no evidence of ST-segment abnormalities. Echocardiography and cardiac catheterization showed an anomalous origin of the patient’s right coronary artery from his left ventricle, just inferior to his aortic valve annulus.
Anomalous origins of the coronary arteries are rare but are known to occur in some typical forms including anomalous origin of the left coronary artery from the pulmonary artery, the right coronary artery, and the right sinus of Valsalva. The right coronary artery is known to arise anomalously from the left main coronary artery and the left sinus of Valsalva. Various coronary artery fistulous connections have also been documented and typically include fistulous connections from the right or left coronary artery to low-pressure chambers such as the right ventricle and the pulmonary artery. We report an unusual case of a patient whose right coronary artery originated from the left ventricular cavity, just inferior to the aortic valve annulus.
A 22-month-old boy was referred to pediatric cardiology for evaluation of a heart murmur. He was asymptomatic except for occasional wheezing with exertion. His family history was negative. Growth and development were normal. On physical examination, the patient’s weight was 15.7 kg (97th percentile), and his length was 86 cm (>50th percentile). The blood pressure was 96/62 mm Hg in his right arm and 94/48 mm Hg in the left leg. On cardiac auscultation, there was a II-III/VI systolic ejection murmur followed by a II/IV diastolic decrescendo murmur at the right upper sternal border. The distal pulses were normal. Electrocardiography showed possible left ventricular hypertrophy, with no evidence of ST-segment abnormalities. Echocardiography demonstrated that the origin of the patient’s right coronary artery was from his left ventricle, just inferior to the aortic valve annulus ( Figure 1 , Video 1 ). There was retrograde flow from the right coronary artery coursing into the body of the left ventricle ( Figures 2 and 3 , Videos 2 and 3 ) The left coronary artery originated normally. Both coronary artery systems were diffusely dilated.
Cardiac catheterization was performed for further delineation of the anatomy and hemodynamics. The left ventricular pressure was 84/10 mm Hg, and the remainder of the hemodynamics were normal. Selective contrast injection into the left main coronary artery demonstrated antegrade filling of the left coronary artery system coursing via collaterals to retrograde filling of the right coronary artery system, with subsequent emptying into the left ventricle ( Figure 4 , Video 4 ).