Coronary-to-Pulmonary Artery Fistula in Adults: Natural History and Management Strategies

Abstract

ObjectiveTo evaluate the natural course of coronary-to-pulmonary artery fistula (CPAF) detected on coronary computed tomography angiography (CCTA) and to propose potential treatment strategies.Materials and MethodsIn this retrospective multicenter study, we assessed the CCTA reports of 188 CPAF patients evaluated between March 2009 and June 2016. Fifty-seven patients were excluded because their follow-up (FU) periods were less than 2 years. Information regarding demographic characteristics, past history, treatment method, and the occurrence of major adverse cardiac events (MACE) during the FU period was collected. We analyzed the morphologic features of CPAF and the various factors associated with surgical treatment. Patients who had undergone FU CCTA after being diagnosed with CPAF were assessed for the presence of morphological changes on FU imaging.ResultsThe median age of the study population was 63.0 years (range, 57.0–72.0 years), and the median FU period was 5.72 years (range, 4.08–6.96 years). The most common origin of the CPAF was both coronary arteries in 76 (58.0%) cases. An aneurysm or aneurysms was/were present in 41 (31.3%) cases. Fifty-four (41.2%) fistulas were less than 2 mm in size. Eight patients underwent surgery, and 123 (93.9%) patients received optimal medical treatment (OMT). The fistula size was significantly different between the two treatment groups (p = 0.013) and was the only factor associated with surgical treatment (odds ratio = 1.14, p = 0.021). Only one patient in the OMT group reported MACE during the FU period due to preexisting coronary artery disease. Twenty-nine patients (22.1%) underwent FU CCTA after CPAF diagnosis, with a median FU period of 3.81 years. None of the patients in the OMT group demonstrated morphological changes in the CPAF on FU imaging.ConclusionMost CPAFs identified on CCTA have a favorable prognosis. Observation with OMT is usually an appropriate strategy. Fistula size is a possible determinant for surgical treatment.

Congenital coronary artery-to-pulmonary fistula with giant aneurysmal dilatation and thrombus formation: a case report and review of literature

“…While CPAF is primarily congenital, it can also result from iatrogenic procedures, trauma, chest radiation, and several diseases like myocardial infarction, Kawasaki disease, and Takayasu arteritis [7,8]. Patients with CPAF are usually asymptomatic due to the small size of the left-toright shunts and carry a seemingly benign prognosis [2,5]. Nevertheless, CPAF will cause symptoms suggestive of myocardial ischemia or even induce myocardial infarction when the shunts are large enough to create the coronary steal phenomenon [7,9].…”

“…Endovascular embolization might also be adopted if CPAF locates proximally and consists of a single narrow drainage site without concomitant cardiac disorders requiring surgery [3,18]. Although no consensus exists regarding the oral medication regimen, several cases utilize antiplatelet medicine, anticoagulants, beta-blockers, and calcium channel blockers to prevent thrombosis and control symptoms [2].…”

“…Coronary artery-to-pulmonary artery fistula (CPAF) is a rare coronary artery anomaly defined as the abnormal communication between the coronary artery and pulmonary artery [1]. It mostly remains asymptomatic before incidentally discovered by coronary computed tomography angiography (CCTA) [2]. However, it could also manifest as angina pectoris, congestive heart failure, pulmonary hypertension, or even sudden cardiac death depending on the severity of the left-right shunt and whether a concomitant ruptured aneurysm exists [2].…”

“…It mostly remains asymptomatic before incidentally discovered by coronary computed tomography angiography (CCTA) [2]. However, it could also manifest as angina pectoris, congestive heart failure, pulmonary hypertension, or even sudden cardiac death depending on the severity of the left-right shunt and whether a concomitant ruptured aneurysm exists [2]. The treatment regimen for CPAF, including oral antiplatelet or anticoagulant medications, interventional endovascular procedures, and ultimately, surgical ligation, should be individually tailored [3].…”

A Glimpse on Trends and Characteristics of Recent Articles Published in the Korean Journal of Radiology

“…KJR increasingly publishes meta-analyses (251525354555657585960), and guidelines (193161626364656667686970717273747576777879), and KJR also publishes consensus statements and recommendations (67808182) in collaboration with Korean Society of Radiology or other societies (68788384). Trends show increasing number of published articles with a prospective design and/or multicenter involvement in the studies (220214950858687888990919293949596979899100101102103104105106107108109110111112113114115116117118119120121122123124125126127). The numbers of articles with guidelines or consensus statements/recommendations were one in 2016, three in 2017, four in 2018, and 12 in 2019.…”