Anomalous coronary artery connecting with the right ventricle associated with pulmonary stenosis and atrial septal defect

Abstract We have described a case of multiple and complex malformations of the coronary arterial system, i.e., single coronary artery which gave off several branches, one of which connected abnormally with the right ventricular cavity so as to produce a shunt. In addition, there was severe pulmonary stenosis with a high right ventricular pressure. This, in turn, maintained a right-to-left shunt through the abnormal coronary vessel. Another, similar shunt was present at the atrial level through an atrial septal defect. These malformations were suspected clinically. Specialized studies (catheterization and angiocardiography) confirmed the diagnosis. The patient was operated upon for ligation of the abnormal coronary vessel and correction of the pulmonary stenosis. He died as a result of cardiac arrest. The hemodynamic pattern of this unusual case is discussed.