An unusual co-anomaly: multiple coronary-cameral fistulas and left main coronary artery arising from the right sinus of Valsalva

Abstract

A 70-year-old female patient was referred to the Cardiology Department for preoperative evaluation before undergoing total hysterectomy. She had no chest pain, but had experienced exertional dyspnea over the past several months. Besides age, she had no additional risk factors for coronary artery disease. Electrocardiogram showed normal sinus rhythm and nonspecific T wave changes, with a heart rate of 81 beats per minute. The physical examination was unremarkable, and exercise stress test was non-diagnostic. Coronary angiography revealed anomalous left main coronary artery (LMCA) arising from the right sinus of Valsalva (Fig. A, Video 1*) and the presence of multiple coronary-cameral fistulas (CCF) between the left ventricle and septal perforating branches of the left anterior descending (LAD) and right coronary arteries (Fig. B, Video 2; Fig. C, Video 3). According to the eye-dot method (Fig. B) and given the septal perforator branch before the LMCA bifurcation (Fig. A), its course was considered as septal, which was confirmed by computed tomography coronary angiography (CTCA) (Fig. D). Because of the benign nature of the abnormal septal course of the LMCA and clinically insignificant CCF, the patient was treated conservatively with β-blockers. She did not experience any chest pain during the hysterectomy and had an uneventful postoperative course. CCF is an uncommon clinical entity, which is defined as an anomalous communication between any epicardial coronary artery and the cardiac chambers. The functional significance and treatment of these fistulas remain unclear. As in our case, CCF originating from both coronary arteries and terminating in cardiac chambers is one of the least common forms. The anomalous origin of LMCA arising from the right sinus of Valsalva is quite rare, with an incidence of 0.1%. It has four possible courses: inter-arterial, retro-aortic, septal, and anterior. The clinical significance and management strategies in these arteries depend on the initial course of the anomalous vessel. In 1990, Serota et al. defined the ‘Dot and Eye’ method for identification of the initial course of anomalous coronary arteries in coronary angiography. In a septal course, the circumflex artery (Cx) will form an ellipse to the left of the aorta, similar to an ‘eye’, with the LMCA forming the inferior portion and the Cx forming the superior portion during 30 RAO injection. In conclusion, CTCA can visualize coronary anomalies accurately; however, a correct diagnosis can be made safely in selected cases by conventional angiography in the presence of clues. 782