A 69-year-old morbidly obese Caucasian woman was seen in consultation owing to exertional dyspnea. She was found to have atrial fibrillation and a continuous murmur at the left upper sternal border. Echocardiography suggested the possibility of patent ductus arteriosus, with high velocity flow into the pulmonary artery (PA). Transesophageal echo suggested similar findings, but again imaging was less than optimal for a definitive diagnosis.
The patient underwent heart catheterization that revealed a right atrial mean pressure of 15 mmHg, right ventricular pressure of 49/15 mmHg, PA pressure of 51/34 mmHg, and a pulmonary capillary wedge pressure of 21 mmHg. Left ventricular systolic pressure was 148 mmHg, with an end-diastolic pressure of 24 mmHg and no aortic stenosis. Fick cardiac output was 5.3 L/min. The left ventriculogram showed a left ventricular (LV) ejection fraction of 70%, with no wall motion abnormalities nor mitral insufficiency.
However, while coronary arteriography showed a normal left main coronary artery, there was a large, 7 mm tortuous fistula arising from the proximal left anterior descending (LAD) coronary artery that drained into the PA (Figure 1). The left coronary artery was otherwise normal. The right coronary artery also gave rise to a smaller, tortuous fistula into the PA (Figure 2).
The patient underwent interventional therapy from the right femoral artery approach using an 8 French (Fr) Amplatz left 1 guide catheter for the right coronary artery. A Balance Middleweight (BMW) Universal .014-inch wire (Abbott Vascular) was placed into the fistulous track and a Progreat microcatheter (Terumo) placed over it well into the fistulous tract. The BMW wire was removed and a 7 mm x 24 mm Azur CX coil (Terumo) was placed into the fistulous tract. A second 7 mm x 24 mm Azur CX coil was placed into the fistulous track under fluoroscopic guidance. A 6 mm x 20 mm Azur CX coil was ultimately placed. There was a dramatic reduction of flow into the fistula (Figure 3). On subsequent imaging, no flow into the pulmonary artery was appreciated.
An 8 French Ikari L4 guide catheter (Terumo) was then placed into the left main coronary artery and the BMW .014-inch universal wire was placed into the LAD and into the fistulous track, followed by an 8 Fr GuideLiner (Teleflex) and the Progreat microcatheter. The BMW wire was removed. The fistula was closed with 5 Azur CX coils in the following sizes (listed in order of placement): 20 mm x 40 mm, 16 mm x 39 mm, 14 mm x 34 mm, 12 mm x 38 mm, and 12 mm x 38 mm. The coils were tightly packed into the fistula and resulted in a significant diminution of fistula tract flow into the PA (Figure 4).
Angiographic reevaluation 2 months later evidenced substantial reduction of flow with some sparse flow from residual feeder vessels for the LAD fistula (Figure 5) and the RCA fistula (Figure 6). The patient’s heart failure symptoms resolved within days after initial coil embolization, with normalization of her activities. She has done well with continued marked improvement of exercise tolerance.
Coronary angiography in adults has revealed the presence of a coronary artery fistula in 0.132-0.22% of cases.1-3 At least 75% of these incidentally diagnosed coronary fistulas were found to be small and clinically silent. Greater than 90% drain into the right side of the circulation. Over 50% of adult patients diagnosed with a coronary fistula have symptoms or complications resulting from the fistula (e.g., angina, myocardial infarction, congestive heart failure, arrhythmias, endocarditis). A very small number of large fistulas expand aneurysmally with dissection or rupture.4-6 The most common physical finding in a large coronary artery fistula is a continuous murmur that can mimic that of patent ductus arteriosus.
Patients with coronary fistulas who have heart failure or myocardial ischemia are candidates for treatment. First reported in 19837, multiple cases of transcatheter occlusion of coronary artery fistulas have been published. The results are generally comparable to reported surgical results, with an expected mortality of less than 1%. In 2007, Catheterization and Cardiovascular Interventions published a “Core Curriculum” report on coronary artery fistulas and their management.8
Herein, we describe the case of a 69-year-old woman with congestive heart failure, who was thought initially to have a patent ductus arteriosus, but was found to have a large LAD to PA fistula and a moderate size right coronary artery to PA fistula. Her fistulas were coil embolized with resolution of anomalous flow and of heart failure symptomatology.
1Clinical Professor, California Northstate University College of Medicine
Disclosures: Dr. Scott Baron reports he is on the speakers bureau for Abbott. Dr. Arvin Arthur and Mohammed Nazir, RCIS, report no conflicts of interest regarding the content herein.
Dr. Scott Baron can be contacted at firstname.lastname@example.org.
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