Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl
A 3-year-old girl, weighing 17 kg, was referred to Chonnam National University Hospital for evaluation of cardiomegaly. At 2-month of her age, the patient was evaluated for heart murmur and was diagnosed with CCAF. Because she did not show any symptoms of heart failure, medication was not given and serial echocardiography performed. On physical examination, grade 2/6 continuous murmur at the left lower sternal border was heard and she had no previous symptoms of tachypnea, poor feeding, cold sweat, cyanosis, etc. Chest radiography revealed cardiomegaly with normal pulmonary vascular markings (). Resting electrocardiogram (ECG) showed mild right ventricular hypertrophy and artery pressures were 90 mmHg systolic and 50 mmHg diastolic. Two-dimension and color Doppler ECG showed a coronary artery fistula arising from a dilated right coronary artery (RCA) and draining into the right atrium through the aneurysmal sac in the right atrium (). Computed tomography showed an approximately 21×16 mm aneurysmal dilatation in a dilated sinoatrial nodal branch of the RCA, and a fistula was located between the distal portion of the aneurysmal sac and right atrium ().\nAfter parental informed consent, cardiac catheterization was performed. The right femoral vein and artery were cannulated and the patient was heparinized intravenously (50 IU/kg). A RCA angiogram revealed a dilated proximal RCA and 4 mm-sized coronary fistula originating from the proximal portion of the RCA emptying into the right atrium with an aneurysm having a maximal diameter of 23 mm; the sizes of the right marginal branch and RAC (originating distal to the fistula) were normal (). After an unsuccessful attempt to guide the wire loop through the extensively tortuous fistula, we decided to change the approach to a transvenous approach. A Progreat 0.019 inch microcatheter (Terumo, Tokyo, Japan) was introduced into the right atrium through the right femoral vein, passing the aneurysm sac, coronary fistula, RCA and ascending aorta. A 0.014 inch runthrough wire (Terumo) was inserted along with the Progreat wire and was replaced with a 4F multipurpose catheter. It was then placed with its tip at the proximal coronary fistula. An 8-mm AVP, which is approximately twice as large as the diameter of the proximal fistula, was inserted and placed immediately proximal to the coronary fistula (at its origin from the normal RCA) in order to minimize the "blind pouch."\nAfter confirmation of the correctly positioned AVP by a RCA angiogram without evidence of ischemic changes in an ECG, the device was released. In repeated RCA angiography 10 minutes after AVP insertion, no shunt lesion was noted (). A repeat echocardiography 3 days after intervention showed no shunt, although the fistula and thrombus were formed in the distal aneurysmal portion of the fistula (). We did not attempt to occlude the distal fistula with another device anymore because distal portion was already naturally closed by the thrombus. The follow-up chest posteroanterior view showed decreased heart size after the procedure.\nThe patient was prescribed aspirin 3 mg/kg daily for 6 months to prevent thromboembolic events. Follow-up echocardiography at 1, 3, 6 months and 1 year after the procedure showed a decreasing size of aneurysm of fistula with thrombus formation (). The patient remained completely asymptomatic including no thromboemboic event during the follow-up period of 1 year and 4 months.