Fistulous Communication Between Coronary Sinus and Left Atrium
A 61-year-old man with dilated cardiomyopathy presented with progressive biventricular decompensation. Two years before admission, the patient had a dual-chamber pacemaker implanted in another hospital because of “sick-sinus-syndrome.” Physical examination showed a heart rate of 110 bpm, with a blood pressure of 150/100 mm Hg, inspiratory crepitant rales over both lung fields, and moderate jugular venous distension. Additional findings included a mitral insufficiency murmur and a tender enlarged liver.
The 12-lead ECG showed atrial flutter with negative p-waves in II, III, and aVF (cycle length 270 ms), with 2:1-AV-conduction and wide QRS-complex (165 ms) with left-bundle-branch-block-morphology (Figure 1). An echocardiogram demonstrated that the left ventricle was markedly dilated (72.5 mm end-diastolic diameter, 69 mm end-systolic diameter), and global hypokinesia with asynchronic movement of the septum. The mitral anulus was extended with moderate to severe mitral insufficiency (grade III). Coronary artery disease was excluded by cardiac catheterization. Left ventricular end-diastolic pressure was 20 mm Hg and cardiac index 2.2 L per min/m2. We decided to implant a defibrillator with additional left ventricular stimulation and, as a second intervention, we decided to ablate the isthmus as therapy for atrial flutter.
We made a coronary venogram to facilitate positioning of the left ventricular electrode. Therefore, a Swan-Ganz-catheter was advanced into the middle part of the coronary sinus, guided by a soft-tip wire. The balloon was carefully insufflated (0.5 to 1 mL) and contrast medium (5 to 8 mL) was given. The angiogram demonstrated normal antegrade flow over the normal dimensioned coronary sinus into the right atrium and retrograde flow through a fistula entering the left atrium just below the ostium of the left superior pulmonal vein. Regurgitation of contrast medium into the pulmonary veins could be observed because of severe mitral regurgitation (Figure 2A and 2B). This finding was confirmed by transesophageal echocardiogram (Figure 3A and 3B). The diameter of the fistula was 7 mm. The measured left to right shunt volume was less than 10%. Angiogram of the left subclavian vein revealed no persistent left superior vena cava. According to the classification of Mantini et al1 and Edwards,2 the described malformation corresponds to the type AII/1a of coronary sinus anomalies.
Implantable cardioverter-defibrillator–implantation was performed by positioning the right atrial and right ventricular electrodes (with shock coil) in typical manner. To avoid any complication concerning the coronary sinus anomaly, the left ventricular electrode was implanted epicardially with an anterior thoracotomy. Some days later, isthmus ablation was successfully performed. At first follow-up visit 8 weeks later, the patient had a stable sinus rhythm with improved cardiac function and exercise capacity (New York Heart Association functional class II), and mitral insufficiency was reduced to grade I.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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- ↵Mantini E, Grondin CM, Lillehei CW, et al. Congenital anomalies involving the coronary sinus. Circulation. 1966; 33: 317–327.
- ↵Edwards JE. Congenital malformations of the heart and great vessels. In: Gould SE, ed. Pathology of the Heart and Blood Vessels. 3rd ed. Springfield, Ill: C. Thomas; 1968: 262.