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      Case Report: Multimodality Imaging as a Lifeline for Fatal Localization of Valsalva Sinus Fibroelastoma

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          Abstract

          Background: Papillary fibroelastomas are rare benign heart tumors, and is most likely to involve the cardiac valves. We will present an extremely rare localization of a large Valsalva sinus fibroelastoma, with occasional left coronary artery ostial obstruction presented as an acute coronary syndrome. The tumor was removed surgically and histologically confirmed as papillary fibroelastoma. This review points to the crucial importance of multidisciplinary team decision and multimodality imaging methods for diagnosing the fibroelastoma, determination of size, and localization, which avoided complications of fatal embolization during an invasive procedure.

          Case Summary: A healthy 55-year-old male with vigorous physical daily training and exercise was admitted to the acute coronary syndrome emergency department. Shortly after admission, expert transthoracic echocardiography was performed. Computed tomography of the chest observed a large irregular hypodense tumor-like lesion in the bulbar aorta that was occasionally prolapsing into the left main coronary artery ostium and which corresponded to fibroelastoma. A few hours after admission, an emergency cardiac surgery was performed with the excision of a Valsalva sinus tumor (size 2 × 2 cm) located between the right and left coronary cusp of the aortic valve.

          Conclusions: Focus cardiac ultrasound should be performed for any acute coronary syndrome because of the possible Valsalva sinus fibroelastoma etiology. Its localization next to the left main coronary artery ostium is rare, and dangerous. The timely diagnosis can be made by the multimodality imaging method, however, the final diagnosis will be made pathohistologically. Early cardiac surgery may be a necessitated recourse for these patients in order to prevent a fatal outcome.

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          Most cited references8

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          Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases.

          With the advent of echocardiography, cardiac papillary fibroelastoma (CPF) is being increasingly reported. The demographics, clinical characteristics, pathological features, treatment, and prognosis of CPF are examined. Cases, case series and related articles on the subject in all languages were identified through a comprehensive literature search. Seven hundred twenty-five cases of CPF were identified. Males comprised 55% of patients. Highest prevalence was in the 8th decade of life. The valvular surface was the predominant locations of tumor. The most commonly involved valve was the aortic valve, followed by the mitral valve. The left ventricle was the predominant nonvalvular site involved. No clear risk factor for development of CPF has been reported. Size of the tumor varied from 2 mm to 70 mm. Clinically, CPFs have presented with transient ischemic attack, stroke, myocardial infarction, sudden death, heart failure, presyncope, syncope, pulmonary embolism, blindness, and peripheral embolism. Tumor mobility was the only independent predictor of CPF-related death or nonfatal embolization. Symptomatic patients should be treated surgically because the successful complete resection of CPF is curative and the long-term postoperative prognosis is excellent. The symptomatic patients who are not surgical candidates could be offered long-term oral anticoagulation, although no randomized controlled data are available on its efficacy. Asymptomatic patients could be treated surgically if the tumor is mobile, as the tumor mobility is the independent predictor of death or nonfatal embolization. Asymptomatic patients with nonmobile CPF could be followed-up closely with periodic clinical evaluation and echocardiography, and receive surgical intervention when symptoms develop or the tumor becomes mobile.
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            Focus cardiac ultrasound core curriculum and core syllabus of the European Association of Cardiovascular Imaging†

            There is a growing trend of using ultrasound examination of the heart as a first-line diagnostic tool for initial patient evaluation in acute settings. Focus cardiac ultrasound (FoCUS) is a standardized but restricted cardiac ultrasound examination that may be undertaken by a range of medical professionals with diverse backgrounds. The intention of this core curriculum and syllabus is to define a unifying framework for educational and training processes/programmes that should result in competence in FoCUS for various medical professionals dealing with diagnostics and treatment of cardiovascular emergencies. The European Association of Cardiovascular Imaging prepared this document in close cooperation with representatives of the European Society of Anaesthesiology, the European Association of Cardiothoracic Anaesthesiology, the Acute Cardiovascular Care Association of the European Society of Cardiology and the World Interactive Network Focused On Critical Ultrasound. It aims to provide the key principles and represents a guide for teaching and training of FoCUS. We offer this document to the emergency and critical care community as a reference outline for teaching materials and courses related to FoCUS, for promoting teamwork and encouraging the development of the field.
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              Papillary fibroelastoma of the aortic valve: analysis of 21 cases, including a presentation with cardiac arrest.

              Cardiac papillary fibroelastoma is a rare, benign tumor, arising predominantly from cardiac valves. This tumor can cause a variety of symptoms due to thromboembolism. We describe our single-center surgical experience with papillary fibroelastoma of the aortic valve. From April 2004 through June 2013, 6,530 patients underwent cardiac surgery. Of those, 6,098 patients were included in the final analysis. Twenty-one patients (0.34%) underwent surgical resection of 30 papillary fibroelastomas of the aortic valve. Most patients (67%) were incidentally diagnosed to have cardiac papillary fibroelastoma. The usual symptom was cerebral infarction (in 5 of 7 symptomatic patients). A rare presentation of papillary fibroelastoma in one patient was cardiac arrest caused by left main coronary artery ostial obstruction. Tumor size was not related to patient age (Pearson correlation coefficient, 0.34; P=0.13). Neither the number of tumors (1.43 ± 0.72 vs 1.43 ± 0.62) nor tumor size (8.14 ± 2.42 vs 8.07 ± 3.31 mm) was significantly different between symptomatic and asymptomatic patients. All lesions were resected by means of the simple shave technique. There were no operative or 30-day deaths. Follow-up echocardiograms showed no tumor recurrence (mean follow-up duration, 17 ± 14 mo). We identified no significant relationship among tumor size, number of tumors, symptoms, or patient age. Because simple shave excision of the tumor can be safely achieved without evidence of tumor recurrence, we conclude that surgical resection can be reasonable in asymptomatic patients.
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                Author and article information

                Contributors
                Journal
                Front Cardiovasc Med
                Front Cardiovasc Med
                Front. Cardiovasc. Med.
                Frontiers in Cardiovascular Medicine
                Frontiers Media S.A.
                2297-055X
                31 May 2021
                2021
                : 8
                : 683534
                Affiliations
                [1] 1Faculty of Medicine, University of Novi Sad , Novi Sad, Serbia
                [2] 2Department of Electrocardiography, Institute of Cardiovascular Diseases of Vojvodina , Sremska Kamenica, Serbia
                [3] 3Faculty of Sport and Physical Education, University of Novi Sad , Novi Sad, Serbia
                Author notes

                Edited by: Dan Jones, Barts Heart Centre, United Kingdom

                Reviewed by: Krishnaraj Sinhji Rathod, Queen Mary University of London, United Kingdom; Mingxing Xie, Huazhong University of Science and Technology, China

                *Correspondence: Aleksandra Milovancev aleksandra.milovancev@ 123456mf.uns.ac.rs

                This article was submitted to Cardiovascular Imaging, a section of the journal Frontiers in Cardiovascular Medicine

                Article
                10.3389/fcvm.2021.683534
                8200485
                33a638aa-586f-43cb-bc02-a8f5091907a3
                Copyright © 2021 Tadic, Ilic, Stefanovic, Stojsic-Milosavljevic, Popov, Bjelobrk, Milovancev, Maksimovic and Drid.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 21 March 2021
                : 11 May 2021
                Page count
                Figures: 4, Tables: 1, Equations: 0, References: 8, Pages: 5, Words: 2507
                Categories
                Cardiovascular Medicine
                Case Report

                multimodality imaging,papillary fibroelastoma,valsalva sinus,acute coronary syndrome,ostium,left coronary artery,cardiac surgery

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