neurysmal circumflex coronary artery (Cx) with fistulous connection to the coronary sinus is a rare clinical condition, 1 and usually remains asymptomatic until later in life. 2 The therapeutic strategy, including the timing of surgical treatment, is not well defined, especially in asymptomatic patients, and surgical repair is quite challenging. 3 Regarding surgical intervention, whether to leave or exclude a diffusely aneurysmal Cx, in addition to ligation of the fistula, is a big issue, considering the risk of later rupture when leaving the aneurysm and sacrifice of the native coronary circulation when excluding the aneurysm. 1 We report a case of an aneurysmal Cx, which finally ruptured into the left atrium after surgical ligation of its fistulous connection to the coronary sinus.
Case ReportIn 2001, an asymptomatic 59-year-old man with a continuous heart murmur underwent cardiac catheterization, which revealed an aneurysmal Cx with fistulous connection to the coronary sinus. The pulmonic-to-systemic flow ratio (Qp/Qs) was 2.05 with an anomalous oxygen step-up at the right atrium. However, he rejected any intervention, because of the lack of symptoms. In 2006, he started to feel worsening dyspnea on exertion, and his Qp/Qs became 4.1. The aneurysmal Cx was grossly dilated from its origin and its maximum diameter was 2.5 cm. It pursued a tortuous course along the left posterior atrioventricular groove (Fig 1). In another institution, he underwent external ligation of the fistula and closure of its distal opening into the coronary sinus under cardiopulmonary bypass. He became symptom-free with an equal Qp/Qs and was discharged without any additional dilatation of the aneurysmal Cx in early angiographs.However, at 6 weeks after the operation, he complained of severe, abrupt chest and back pain and was transferred to our institution as an emergency case. On arrival, his blood pressure was 70 mmHg with ST depression in all leads of the electrocardiogram. Several cardioversions were needed to treat the ventricular tachycardias. He was emergently intubated and required intra-aortic balloon pump support, followed by establishment of percutaneous cardiopulmonary support. Echocardiography revealed akinetic inferior and posterolateral left ventricular walls with generalized hypokinesis and an anomalous penetrated shunt from the aneurysmal Cx to the left atrium. Emergency cardiac repair was undertaken under cardiac arrest using cardiopulmonary bypass. The aneurysmal Cx was opened after transection of the pulmonary trunk (Fig 2). The dilated Cx contained a massive adherent thrombus. Saphenous vein grafts were individually bypassed to the left anterior descending coronary artery and a posterolateral branch of the Cx. A right-side left atriotomy clearly showed perforation of the aneurysmal Cx into the left atrium with a 3×4-cm fragile-ended foramen, which was successfully closed using a bovine pericardial patch. After ensuring that there was no bleeding from the excluded portion, the operation Circ J 2007; 71: 1996 -1998 ...