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      Clinical presentation of coronary arteriovenous fistula according to age and anatomic orientation

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          Abstract

          Background: Coronary arteriovenous fistulas (CAVFs) are direct connections from one or more coronary arteries to cardiac chambers or a large vessel. They are mostly of congenital origin. The aim of this study was to describe clinical presentation and also delineate the course and management of CAVF.

          Methods: Clinical data, chest x-rays, echocardiographic and angiographic evaluation of 40 patients with congenital CAVF during 1990 to 2008 were reviewed retrospectively.

          Results: Seventeen patients were ≤ 20 years old (42.5%) were mostly asymptomatic, and twenty tree cases were older than 20 years old (57.5%), mostly symptomatic (P<0.05). Twenty one (52.5%) patients had pure CAVF and nineteen (47.5%) patients with associated intarcardiac congenital heart disease (15%) or acquired valvular and coronary arteries diseases (32.5%). CAVFs mostly originated from left anterior descending artery (LAD) (42.5%) and mostly drained into the main pulmonary artery (MPA) (35 %). Twenty-four patients underwent CAVF surgical ligation. From twenty-one patients with pure CAVF, eight (38%) patients were complicated by congestive heart failure and aneurism formation of fistula.

          Conclusion: Unlike some previous reports, in our study, the most prevalent origin site for CAVFs was the left anterior descending (LAD). Most patients with CAVFs especially those who went first diagnosed before 20 years old were asymptomatic. On the other hand, as the continuous murmur is not always detected in children or infants, consequently, cases of spontaneous closure may remain undetected. All symptomatic and asymptomatic patients with moderate to severe shunting should be operated on and minimal morbidity and good surgical results could be expected.

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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 artery fistula.

            Although coronary arterial fistula is rare, it is one of the most common among the coronary artery anomalies. Coronary arterial fistula most commonly affects the right side of the heart. It may occur isolated or along with congenital heart diseases. Angiography remains the best investigation for diagnosing the disease. Unless very large and hemodynamically significant, it is usually asymptomatic in younger patients. With increasing age, symptoms begin to appear, and the incidence of complication rises. Treatment by transcatheter or surgical closure gives the best results, provided this is performed early in the course of the disease. This review was prepared by searching the terms "coronary artery fistula," "coronary cameral fistula," "surgical management of coronary arterial fistula," "MDCT in coronary artery fistula," and "multiple coronary artery fistulae" in Google Scholar, PubMed, and PubMed Central and exploring the related articles shown on the side of page. Copyright © 2012 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.
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              Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management.

              Thirteen new patients and 174 patients previously reported with coronary arteriovenous fistula (CAVF) were reviewed to delineate the course and management of CAVF and to clarify the role of surgical ligation in the young asymptomatic patient. Patients were grouped according to age: 99 patients (four new and 95 reported) were less than 20 years old and 88 (nine new and 79 reported) were greater than or equal to 20 years old. Of those under 20 years of age, 19% had preoperative symptoms or CAVF-related complications, including congestive heart failure (CHF) in 6%, subacute bacterial endocarditis in 3% and death in one patient. Seventy-six patients younger than 20 years old had CAVF ligation with only one significant complication. In contrast, 63% of the older group and all of our nine older patients had preoperative symptoms or complications, including CHF in 19%, SBE in 4%, myocardial infarction (MI) in 9%, death in 14% and fistula rupture in one patient. Of the 43 ligated older patients, 23% had surgical complications, including MI in three and death in three. Mean pulmonic-to-systemic flow in the entire group was 1.6:1 and did not differ significantly in those with or without symptoms or complications. One of our patients and one previously reported had spontaneous CAVF closure. In summary, early elective ligation of CAVF is indicated in all patients because of the high incidence of late symptoms and complications and the increased morbidity and mortality associated with ligation in older patients.
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                Author and article information

                Journal
                Caspian J Intern Med
                Caspian J Intern Med
                CJIM
                Caspian Journal of Internal Medicine
                Babol University of Medical Sciences (Babol, Iran )
                2008-6164
                2008-6172
                Spring 2015
                : 6
                : 2
                : 108-112
                Affiliations
                [1 ]Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, IR Iran.
                [2 ]Pediatric Cardiology Department of Shahid Rajaie Heart Center, Iran University of Medical Sciences, Tehran, Iran.
                [3 ]Mashhad University of Medical Sciences, Mashhad. Iran.
                [4 ]Mazandaran university of medical sciences, Sari, Iran.
                Author notes
                [* ]Correspondence: Hassan Zamani, Non-Communicable Pediatric Diseases Research Center, Babol University of Medical Sciences, Babol, IR Iran. E-mail: zamanihassan@yahoo.com ,Tel: 0098 11 32346963 ,Fax: 0098 11 32346963
                Article
                cjim-6-108
                4478461
                26221510
                fe1a0c82-8b11-4ea5-91be-90dfc1949dc3
                © 2015: Caspian Journal of Internal Medicine

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License, ( http://creativecommons.org/licenses/by/3.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 January 2014
                : 13 July 2014
                : 17 December 2014
                Categories
                Original Article

                congenital heart disease,coronary arteriovenous fistula,cavf.

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