congenital coronary arteriovenous fistula ( cavf ) has been described to consist of a diagnostic triad : an abnormal localization for a to - and - fro murmur thought to be due to a patent ductus arteriosus ; a left to right shunt at the atrial or ventricular level ; and a large , tortuous coronary artery on coronary angiography.1 ) known complications of cavf are heart failure , endocarditis , arrhythmias , stroke , myocardial ischemia or myocardial infarction .
many centers have reported surgical corrections or closure of congenital cavf to prevent the complications.2 - 7 ) but choosing an appropriate method of cavf treatment still remains a controversial issue.8 ) we report a case of congenital giant right coronary artery which had a fistulous connection to the coronary sinus ( cs ) and left persistent superior vena cava treated with medical therapy .
a 63-year - old female presented with dyspnea on exertion , orthopnea , and facial edema .
she had underlying type 2 diabetes , parkinsonism , as well as osteoporosis . at the time of admission , the patient 's blood pressure was 100/60 mm hg , pulse rate was 67/min , and
initial echocardiogram revealed marked dilatation of the right coronary artery ( 15 mm in diameter ) from the initial segment , running along the posterior wall of the left ventricle and terminating around the cs with shunt flow ( fig .
ct revealed a right coronary artery to cs fistula with persistent left superior vena cava ( plsvc ) , low lying left innominate vein , and right atrial enlargement possibly due to a left to right shunt .
cardiac catheterization revealed significant oxygen step - up in the right atrium ( ra ) { oxygen saturations were as follows : right jugular vein , 51.4% ; left subclavian vein , 70.3% ; superior vena cava 64.2% ; plsvc ( upper ) 82.8% ; plsvc ( lower ) 86.7% ; cs ostium 75.7% , high ra 66.4% ; mid ra 74.7% ; low ra 73.3% ; right ventricle 75.8% ; pulmonary artery 75.8% , inferior vena cava 61.5%}. the estimated ratio of pulmonary blood flow to systemic blood flow ( qp / qs ) was approximately 1.53 . based on a multidisciplinary team approach taking into account the patient age and activity level as well as the amount of shunt flow , we concluded to treat her with combination therapy using angiotensin converting enzyme inhibitor and diuretics .
after approximately 10 months from the date of discharge , the patient has been doing well without symptom .
coronary arteriovenous fistula is rare cardiac anomaly with a known incidence of 0.3 - 0.8%.9 ) low - pressure structures such as pulmonary artery , superior vena cava , and cs are frequent termination sites of fistulous connections.9 ) possible complications include heart failure , angina , endocarditis , arrhythmias , stroke , myocardial ischemia or myocardial infarction due to coronary steal.10 ) thus the antiplatelet therapy , especially for patients with distal coronary artery fistulas , abnormally dilated coronary arteries , and prophylactic precautions of subacute bacterial endocarditis , is recommended.8 ) we were able to find eight cases of congenial giant right coronary artery to cs reported in the literature ( table 1).3 - 6)11 - 14 ) five out of 8 cases were repaired with surgery .
some authors insist , in order to prevent potential complications , that early surgical intervention is the best treatment.5 ) but surgical intervention for cavf is still a matter of debate . some postoperative complications such as myocardial infarction , ischemia ,
post ligation thrombosis and death have been reported.10)15)16 ) liberthson et al.10 ) reviewed 13 cases of congenital cavf with surgical therapy and found that postoperative complications increase in patients over 20 years of age . in our case ,
the patient was treated with loop diuretics and spironolactone for several days after admission after which her facial edema and dyspnea gradually improved .
initially surgical correction or catheter - based closure of fistula was considered , however for symptom improvement , we came across an opportunity to treat with medical therapy .
this case suggests that heart failure symptom with qp / qs over 1.5 may not be a clear - cut indication for surgery.14 ) in conclusion , we report a rare case of cavf from giant right coronary artery presented with dyspnea and facial edema . to determine the appropriate therapeutic strategy for a rare case such as this one presenting with congenital anomalies
, we need to take into account the overall conditions and well - being of the patient .
 the combination of coronary arteriovenous fistula to the coronary sinus ( cs ) , dilatation of the entire length of coronary artery , coronary aneurysm and persistent left superior vena cava ( plsvc ) is very rare . 
 we present the case of a 63-year - old female admitted for dyspnea on exertion , orthopnea , and facial edema . 
 echocardiography detected a giant coronary artery with shunt flow , dilated cs and plsvc and a coronary angiography reaffirmed these findings . 
 the calculated ratio of pulmonary blood flow to systemic blood flow by cardiac catheterization was 1.53 . 
 after multidisciplinary review considering old age , hypoactivity due to underlying parkinsonism and relatively small amount of shunt flow , medical therapy was chosen . 
 the patient remained asymptomatic for 10 months after discharge without intervention . 
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