Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery in a Neonate with Turner Syndrome and Aortic Arch Hypoplasia

Abstract

Anomalous origin of the right coronary artery from the pulmonary artery, a rare congenital cardiac defect, is typically not diagnosed during infancy. On the other hand, Turner syndrome is usually diagnosed early, and it is classically associated with bicuspid aortic valve and aortic coarctation. Individuals with Turner syndrome are also at increased risk for coronary artery anomalies. We present a case of anomalous right coronary artery from the pulmonary artery in a week-old neonate who also had Turner syndrome, patent ductus arteriosus, transverse aortic arch hypoplasia, and impaired ventricular function. Prostaglandin therapy through the ductus increased the patient's myocardial perfusion. Four months after corrective surgery, she was doing well. We discuss the reperfusion phenomenon in our patient's case, as well as other considerations in this combination of congenital defects.

Challenging Diagnosis of Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery

“…Williams et al conducted a review of the literature on 70 patients diagnosed with ARCAPA in published case reports and described a possible association with other structural cardiac lesions in more than one-third of the reports [ 3 ]. The most common associated lesions were: aortopulmonary window and tetralogy of Fallot, but additional lesions were encountered: ventricular septal defect, atrial septal defect, double outlet right ventricle, patent ductus arteriosus, coarctation of the aorta, aortic arch hypoplasia, pulmonary stenosis, aortic stenosis, and left main coronary artery-to-pulmonary artery fistula [ 3 , 11 , 12 ].…”