A Unique Case of Quadricuspid Aortic Valve With Coronary Artery and Descending Aorta-to-Pulmonary Artery Fistulae

Abstract

46-year-old man with well-controlled hypertension pre-sented with dyspnea, palpitations, and a murmur of aortic regurgitation. Echocardiography revealed a quadricuspid aortic valve (QAV) with 4 equal-sized cusps and enlarged left main coronary artery ostium (Figure 1A). On additional imaging, an unusual flow was seen entering the proximal main pulmonary artery from the lateral aspect just distal to the pulmonic valve (Figure 2A and Movie I in the online-only Data Supplement). A QAV with moderate aortic regurgitation (Movie II in the online-only Data Supplement) and a coronary-pulmonary artery fistula were diagnosed by echocardiography. Computed tomography confirmed the presence of QAV (Figure 1B) and a small-cal-iber vessel that originated from the left main coronary artery, coursed superiorly (Figure None.2B), and ended in a larger confluence of anomalous vessels that communicated with the pulmonary artery (Figure 2C). Other vessels contributing to this conflu-ence originated from the descending aorta and followed a very tortuous course (Figure 2D1 through 2D3). A 3-dimensional reconstruction shown in Figure 2E demonstrates the descend-ing aorta-to-pulmonary artery fistula and coronary artery-to-pulmonary artery fistula draining into a large confluence. Thus, a unique anomaly in combination with QAV was diagnosed.QAV is a rare malformation, with an estimated incidence of 0.003% to 0.043% of all congenital heart disease.