Anomalous Origin (Coronary Artery)
This opened left ventricular outflow tract reveals the aortic valve and the coronary artery orifices. The aortic cusps as depicted from left to right in the photo are left anterior, right anterior, and noncoronary (posterior). Both coronary arteries take their origin from the sinus of Valsalva associated with the left anterior cusp. The right coronary artery, the orifice of which is to the right in the photo, took off at a sharp angle and then coursed between the aorta and the pulmonary trunk. In this anatomical arrangement blood flow can be markedly diminished. The normal site of origin of the right coronary artery is from the sinus of Valsalva associated with the right anterior cusp from which it can easily follow its normal course in the AV groove to the posterior aspect of the heart without sharp twists or turns or being squeezed between the pulsating aorta and pulmonary trunk.
This patient was a 4 year old boy who collapsed while playing with his father in a swimming pool. Examination of the heart revealed cardiomegaly, an old anteroseptal infarct, and an apical aneurysm with a mural thrombus. The photo illustrates the opened right ventricular outflow tract with the pulmonic valve. The probe is in the left coronary artery which took its exit from the pulmonary trunk.
Anomalies of the coronary arteries can be associated with sudden death. Patients can present at any age but clinical manifestations including sudden death at a young age are not uncommon. The anomalies may involve origin of one or both vessels from the pulmonary trunk or origin of the vessel or vessels from an abnormal position in the aorta. For a good diagramatic illustration of the anomaly present in this case see figures 2B and 3A in Lipsett J et al. Anomalous coronary arteries. Pediatr Pathol 1994;14:287-300.
Contributed by Dr. Thomas Gilson