A Rare Combination of the Left Circumflex Coronary Artery Fistula Connecting a Dilated Coronary Sinus with Persistent Left Superior Vena Cava and Multiple Arteriovenous Fistulae
A 71-year-old woman without significant medical and family history visited the local hospital with exertional dyspnea and palpitations for 1 month. She had transferred to our hospital due to an unusual finding on transthoracic echocardiography (TTE), which was suspicious of CAF. On physical examination, the blood pressure was 125/80 mm Hg and chest auscultation revealed a continuous murmur over the left anterior chest, which was graded as Levine 2/VI. Chest radiography showed an increased cardiothoracic ratio and electrocardiography (ECG) showed atrial fibrillation. The heart rate was 75 beats/min. On TTE, a dilated LMT connecting to a dilated vessel was seen. Turbulent flow was observed on color Doppler behind the aortic root; moreover, a dilated CS with a PLSVC was identified by injection of agitated saline (). Also, TTE revealed a dilation of both the atrium and the pulmonary artery. A provisional diagnosis of aneurysm of the LMT with CAF and PLSVC was made. The patient underwent conventional coronary artery angiography, which showed a giant aneurysm of the LMT (9.05 mm) with tortuous and aneurysmal CAF (). Also, a right conal branch connecting to the pulmonary trunk was revealed. The right heart catheterization documented a pulmonary to the systemic blood flow ratio (Qp/Qs) of 1.72, which indicated the presence of a significant left to the right shunt. As the distal drainage site was not well identified on the conventional coronary artery angiography, ECG-gated multi-detector computed tomogram angiography (CTA, Brilliance 64 CT scanner, Philips Medical Systems, Cleveland, OH, USA) was performed. The CTA clearly demonstrated an aneurysmal change of the LMT, the proximal segment of the left circumflex artery and the obtuse marginal branch that connected to the dilated CS. The CTA showed that the distal segment of the left circumflex artery and the left bronchial artery were connected to the CS. Also, the CTA revealed the presence of a fistulous connection between the right conal branch and the pulmonary trunk, as well as the sinoatrial nodal artery originating from the LMT connected to the CS through the tortuous vessel (). CTA revealed a PLSVC. However, the connection between the two superior vena cavas (SVCs) was not observed; neither stenosis nor occlusion of CS ostium was seen. Due to multiple fistulae, severe dilation of the native coronary artery, extreme fistula tortuosity and difficulty in cannulating the distal fistula, the patient was considered not to be a candidate for percutaneous trans-catheter closure (TCC) and was recommended for surgical ligation of the fistula by a cardiothoracic surgeon. However, the patient refused surgery and requested to be managed conservatively. Her advanced age and the risks of surgical ligation were taken into consideration, and medications such as aspirin, ramipril and digoxin were prescribed for management of the atrial fibrillation. She was asymptomatic at the 1-year follow-up.