SummaryCoronary to pulmonary artery fistulas (CPAFs) are abnormal communications between the coronary and pulmonary arteries. They are an uncommon congenital heart disease and usually remain asymptomatic until later in life. However, there is no consensus on their management. We present four adult patients who required surgery for coronary to pulmonary artery fistulas to illuminate this issue. The clinical presentations were variable depending on the anatomical features of coronary to pulmonary artery fistulas and the presence or absence of other cardiac diseases. We successfully performed surgical closure of the coronary to pulmonary artery fistulas in each of the cases. In this report, we describe our experience with these cases and outline the available therapeutic strategies and treatment options for coronary to pulmonary artery fistulas.(Int Heart J 2017; 58: 1012-1016) Key words: Cardiac surgery, Coronary artery fistula, Coronary artery anomaly, Coronary aneurysm A coronary artery fistula (CAF) is an abnormal communication between a coronary artery and either a cardiac chamber or a great vessel including pulmonary artery (PA). The reported incidence is 0.002% among the general population, 1) and these fistulas account for approximately 0.4% of all congenital cardiac abnormalities.2) Though they usually remain asymptomatic in childhood, they may lead to congestive heart failure, myocardial ischemia, infective endocarditis, or rupture of an associated aneurysm later in life.3) Recently, multidetector computed tomography (MDCT) has been used to delineate the complex anatomy of CAF 4) or coronary aneurysm, 5) with recent studies indicating that coronary to pulmonary artery fistulas (CPAFs) are the most common type.6-8) It has been argued that the embryological basis of CPAF is different from other types of CAF, which drain into cardiac chambers, 9,10) and that the resulting left-toright shunt is relatively small through a CPAF.11) To date, the optimal management for small and asymptomatic CPAFs remains a matter of debate. In this report, we present our experience with surgical repair of CPAF at our institution between January 2001 and June 2016.
Case ReportsCase 1: An asymptomatic 70-year-old woman with a history of hypertension and hyperlipidemia was referred to our hospital for evaluation after a mass adjoining the left cardiac border had been identified on chest X-ray. Enhanced CT of the chest demonstrated a large aneurysm measuring 34 mm in diameter on the left side of the main PA ( Figure 1A). The aneurysm had increased in size by 6 mm over a 3-year period. Coronary angiography (CAG) revealed a fistula arising from diagonal branch that was supplying a large aneurysm draining into the PA through two fistulas ( Figure 1B). Electrocardiography (ECG) showed no ischemic changes, and echocardiography revealed preserved left ventricular systolic function with no significant valvular disease. From these findings, she was diagnosed as having a CPAF with a concomitant large aneurysm that was considered to have enla...