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      Cardiac papillary fibroelastoma of a bicuspid aortic valve in an adolescent: A case report

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          Abstract

          Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.

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          Most cited references19

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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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            Clinical and echocardiographic characteristics of papillary fibroelastomas: a retrospective and prospective study in 162 patients.

            Cardiac papillary fibroelastoma (CPF) is a primary cardiac neoplasm that is increasingly detected by echocardiography. The clinical manifestations of this entity are not well described. In a 16-year period, we identified patients with CPF from our pathology and echocardiography databases. A total of 162 patients had pathologically confirmed CPF. Echocardiography was performed in 141 patients with 158 CPFs, and 48 patients had CPFs that were not visible by echocardiography (<0.2 cm), leaving an echocardiographic subgroup of 93 patients with 110 CPFs. An additional 45 patients with a presumed diagnosis of CPF were identified. The mean age of the patients was 60+/-16 years of age, and 46.1% were male. Echocardiographically, the mean size of the CPFs was 9+/-4.6 mm; 82.7% occurred on valves (aortic more than mitral), 43.6% were mobile, and 91.4% were single. During a follow-up period of 11+/-22 months, 23 of 26 patients with a prospective diagnosis of CPF that was confirmed by pathological examination had symptoms that could be attributable to embolization. In the group of 45 patients with a presumed diagnosis of CPF, 3 patients had symptoms that were likely due to embolization (incidence, 6.6%) during a follow-up period of 552+/-706 days. CPFs are generally small and single, occur most often on valvular surfaces, and may be mobile, resulting in embolization. Because of the potential for embolic events, symptomatic patients, patients undergoing cardiac surgery for other lesions, and those with highly mobile and large CPFs should be considered for surgical excision.
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              Cardiac Masses on Cardiac CT: A Review

              Cardiac masses are rare entities that can be broadly categorized as either neoplastic or non-neoplastic. Neoplastic masses include benign and malignant tumors. In the heart, metastatic tumors are more common than primary malignant tumors. Whether incidentally found or diagnosed as a result of patients’ symptoms, cardiac masses can be identified and further characterized by a range of cardiovascular imaging options. While echocardiography remains the first-line imaging modality, cardiac computed tomography (cardiac CT) has become an increasingly utilized modality for the assessment of cardiac masses, especially when other imaging modalities are non-diagnostic or contraindicated. With high isotropic spatial and temporal resolution, fast acquisition times, and multiplanar image reconstruction capabilities, cardiac CT offers an alternative to cardiovascular magnetic resonance imaging in many patients. Additionally, cardiac masses may be incidentally discovered during cardiac CT for other reasons, requiring imagers to understand the unique features of a diverse range of cardiac masses. Herein, we define the characteristic imaging features of commonly encountered and selected cardiac masses and define the role of cardiac CT among noninvasive imaging options.
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                Author and article information

                Journal
                Clin Pract
                CP
                Clinics and Practice
                PAGEPress Publications, Pavia, Italy
                2039-7275
                2039-7283
                07 August 2019
                02 August 2019
                : 9
                : 3
                : 1135
                Affiliations
                [1 ]Pediatric Cardiology, Department of Pediatrics, University Hospital of Liège
                [2 ]Department of Pediatrics, Regional Hospital Verviers
                [3 ]Department of Anatomic Pathology, University Hospital of Leuven , Belgium
                Author notes
                Pediatric Cardiology, Department of Pediatrics, University Hospital of Liège, Avenue de l’Hôpital, B. 4000 Liège, Belgium. +32.43679275. mcseghaye@ 123456chu.ulg.ac.be

                Contributions: SD, conception of the research work, acquisition and collection of the data, analysis and critical interpretation of the data; drafting the manuscript, analyzing literature and critically revising the manuscript; final approval of the manuscript; agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. MCS wrote the manuscript; BD and MCS diagnosed the lesion at echocardiography; MB confirmed the anatomopathological nature of the lesion. All of the authors contributed to the revision of the manuscript.

                Conflict of interest: the authors declare no potential conflict of interest.

                Article
                10.4081/cp.2019.1135
                6766684
                d7d9f1d7-0a43-4279-ba3e-4e209a425b5a
                ©Copyright: the Author(s), 2019

                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
                : 07 February 2019
                : 02 August 2019
                Page count
                Figures: 3, Tables: 0, Equations: 0, References: 19, Pages: 3
                Funding
                Funding: none.
                Categories
                Case Report

                cardiac papillary fibroelastoma,bicuspid aortic valve,children

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