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

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          Abstract

          Objective

          To 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 Methods

          In 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.

          Results

          The 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.

          Conclusion

          Most 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.

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          Most cited references34

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          2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines.

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            Coronary artery fistulas: clinical and therapeutic considerations.

            Coronary artery fistulas vary widely in their morphological appearance and presentation. These fistulas are congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel, or other structure, bypassing the myocardial capillary network. The majority of these fistulas arise from the right coronary artery and the left anterior descending coronary artery; the circumflex coronary artery is rarely involved. Clinical manifestations vary considerably and the long-term outcome is not fully known. The patients with coronary fistulas may present with dyspnea, congestive heart failure, angina, endocarditis, arrhythmias, or myocardial infarction. A continuous murmur is often present and is highly suggestive of a coronary artery fistula. Differential diagnosis includes persistent ductus arteriosus, pulmonary arteriovenous fistula, ruptured sinus of Valsalva aneurysm, aortopulmonary window, prolapse of the right aortic cusp with a supracristal ventricular septal defect, internal mammary artery to pulmonary artery fistula, and systemic arteriovenous fistula. Although noninvasive imaging may facilitate the diagnosis and identification of the origin and insertion of coronary artery fistulas, cardiac catheterization and coronary angiography is necessary for the precise delineation of coronary anatomy, for assessment of hemodynamics, and to show the presence of concomitant atherosclerosis and other structural anomalies. Treatment is advocated for symptomatic patients and for those asymptomatic patients who are at risk for future complications. Possible therapeutic options include surgical correction and transcatheter embolization. Historical perspectives, demographics, clinical presentations, diagnostic evaluation, and management of coronary artery fistula are elaborated.
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              Coronary arteriovenous fistulae: a review.

              Coronary arteriovenous fistulae are a coronary anomaly, presenting in 0.002% of the general population. Their etiology can be congenital or acquired. We present a review of recent literature related to their epidemiology, etiology, pathophysiology, clinical presentation, diagnostic approach, and therapeutic management.
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                Author and article information

                Journal
                Korean J Radiol
                Korean J Radiol
                KJR
                Korean Journal of Radiology
                The Korean Society of Radiology
                1229-6929
                2005-8330
                November 2019
                17 September 2019
                : 20
                : 11
                : 1491-1497
                Affiliations
                [1 ]Department of Radiology, Seoul St. Mary's Hospital, School of Medicine, The Catholic University of Korea, Seoul, Korea.
                [2 ]Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
                Author notes
                Corresponding author: Jung Im Jung, MD, PhD, Department of Radiology, Seoul St. Mary's Hospital, School of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 06591, Korea. Tel: (822) 2258-1432, Fax: (822) 599-6771, jijung@ 123456catholic.ac.kr
                Author information
                https://orcid.org/0000-0003-0884-7946
                https://orcid.org/0000-0002-9262-1001
                https://orcid.org/0000-0002-9983-048X
                https://orcid.org/0000-0001-8264-9388
                Article
                10.3348/kjr.2019.0331
                6791815
                31606954
                9bec3f86-7607-49af-8dbd-45a97ccc9ea7
                Copyright © 2019 The Korean Society of Radiology

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 11 May 2019
                : 15 July 2019
                Categories
                Cardiovascular Imaging
                Original Article

                Radiology & Imaging
                ct angiography,coronary artery,pulmonary artery,vascular fistula,prognosis,coronary artery anomaly

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