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      Imaging Findings of Coronary Artery Fistula in Children: A Pictorial Review

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          Abstract

          Coronary artery fistula, defined as an abnormal communication between the coronary arteries and a cardiac chamber (most commonly) or a thoracic great vessel, may result in hemodynamically significant problems due to vascular shunting in children. Echocardiography, cardiac catheterization, cardiac MRI, and cardiac CT may be used to evaluate coronary artery fistula in children. Recently, CT has played a pivotal role for the accurate diagnosis of coronary artery fistula in children. Surgical or interventional treatment is performed for hemodynamically significant coronary artery fistulas. In this pictorial review, the detailed imaging findings of coronary artery fistula in children are described.

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          Coronary artery anomalies in 126,595 patients undergoing coronary arteriography.

          O Yamanaka, R. E. Hobbs (1990)
          Coronary artery anomalies were found in 1,686 patients (1.3% incidence) undergoing coronary arteriography at the Cleveland Clinic Foundation from 1960 to 1988. Of the 1,686 patients, 1,461 (87%) had anomalies of origin and distribution, and 225 (13%) had coronary artery fistulae. Most coronary anomalies did not result in signs, symptoms, or complications, and usually were discovered as incidental findings at the time of catheterization. Eighty-one percent were "benign" anomalies: 1) separate origin of the left anterior descending and circumflex from the left sinus of Valsalva; 2) ectopic origin of the circumflex from the right sinus of Valsalva; 3) ectopic coronary origin from the posterior sinus of Valsalva; 4) anomalous coronary origin from the ascending aorta; 5) absent circumflex; 6) intercoronary communications; and 7) small coronary artery fistulae. Other anomalies may be associated with potentially serious sequelae such as angina pectoris, myocardial infarction, syncope, cardiac arrhythmias, congestive heart failure, or sudden death. Potentially serious anomalies include: 1) ectopic coronary origin from the pulmonary artery; 2) ectopic coronary origin from the opposite aortic sinus; 3) single coronary artery; and 4) large coronary fistulae. Coronary artery anomalies require accurate recognition, and at times, surgical correction.
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            Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history.

            M Vavuranakis, H Boudoulas, Paul C. Bush (1995)
            The incidence, angiographic characteristics, and natural history of coronary artery fistulas in patients undergoing diagnostic cardiac catheterization have not been well defined. Of 33,600 patients who had diagnostic cardiac catheterization, 34 (0.1%) had coronary artery fistula. Nineteen fistulas originated from the right, 11 from the left anterior descending, and 4 from the circumflex coronary arteries, respectively. The mean ratio of pulmonary to systemic flow was 1.19 +/- 0.33. Only one patient with coexistent atrial septal defect had a pulmonic to systemic flow ratio > 1.5. Right and left heart pressures, with the exception of three patients in whom left ventricular end-diastolic pressures was > 12 mm Hg, were within normal limits. During a mean follow-up period of 6.3 years (range 2-14 years), there were no complications related to coronary artery fistula. It was concluded that the incidence of coronary artery fistulas detected during diagnostic coronary angiography is very low. Coronary artery fistulas originate predominantly from the right coronary artery and are not associated with hemodynamic abnormalities or other congenital heart diseases. The prognosis of coronary artery fistulas in adults is good.
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              Coronary Artery Fistulas: Pathophysiology, Imaging Findings, and Management.

              Gabin Yun, Tae Nam, Eun Chun (2018)
              Coronary artery fistulas (CAFs) are abnormal communications of coronary arteries whereby venous circuits bypass the normal capillaries within the myocardium. CAFs are rare, and most affected patients are asymptomatic. However, these fistulas are the most common coronary artery anomalies that can alter coronary hemodynamic parameters. Although most CAFs are asymptomatic in young patients, symptoms and complications become more frequent with increasing age. CAFs are characterized by variable clinical manifestations that are based on the size, origin, and drainage site of the fistula. In symptomatic cases, surgical ligation or percutaneous transcatheter closure is often recommended. Although CAFs historically have been evaluated with conventional invasive angiography, electrocardiographically gated cardiac computed tomographic (CT) angiography has emerged as the noninvasive alternative modality of choice owing to the high spatial and temporal resolution and short acquisition time. Furthermore, three-dimensional volume-rendered CT angiograms facilitate accurate assessment of the complex anatomy of CAFs, including their origin, drainage site, and complexity and the number and size of fistulous tracts. Knowledge of these characteristics is crucial for therapeutic planning. Radiologists must be aware of the pathophysiology, clinical manifestations, and characteristic CT angiographic findings of CAFs; appropriate CT angiographic protocols for evaluation of various CAFs; and the role of CT angiography in preprocedural planning and follow-up. Online supplemental material is available for this article. ©RSNA, 2018.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Korean J Radiol
                Journal ID (iso-abbrev): Korean J Radiol
                Journal ID (publisher-id): KJR
                Title: Korean Journal of Radiology
                Publisher: The Korean Society of Radiology
                ISSN (Print): 1229-6929
                ISSN (Electronic): 2005-8330
                Publication date (Print): December 2021
                Publication date (Electronic): 13 September 2021
                Volume: 22
                Issue: 12
                Pages: 2062-2072
                Affiliations
                Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
                Author notes
                Corresponding author: Hyun Woo Goo, MD, PhD, Department of Radiology and Research Institute of Radiology, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea. ghw68@ 123456hanmail.net
                Author information
                https://orcid.org/0000-0001-6861-5958
                Article
                DOI: 10.3348/kjr.2021.0336
                PMC ID: 8628148
                PubMed ID: 34564965
                SO-VID: 33fb96bd-dc85-44dc-8aa4-cfeba064fbcf
                Copyright © Copyright © 2021 The Korean Society of Radiology
                License:

                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
                Date received : 22 April 2021
                Date revision received : 09 June 2021
                Date accepted : 21 July 2021
                Categories
                Subject: Pediatric Imaging
                Subject: Pictorial Essay

                ScienceOpen disciplines: Radiology & Imaging
                Keywords: cardiac catheterization,children,cardiac ct,coronary artery anomalies,echocardiography
                Data availability:
                ScienceOpen disciplines: Radiology & Imaging
                Keywords: cardiac catheterization, children, cardiac ct, coronary artery anomalies, echocardiography

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