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      A rare case of Whipple’s disease with endocarditis in a patient with dextrocardia

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          Abstract

          We report a case of an elderly Caucasian male with past medical history of dextrocardia with situs inversus totalis, polymyalgia rheumatica, history of cryptogenic stroke, and severe mitral regurgitation with mitral valve prolapse, who presented with acute heart failure symptoms, including severe dyspnea on exertion and worsening lower extremity edema in the setting of immunosuppression with steroids for a year-old diagnosis of polymyalgia rheumatica. One month prior to this presentation, the patient suffered a transient ischemic attack and during the workup, his transthoracic echocardiography showed myxomatous degeneration of posterior mitral leaflet, partially flail, with severe mitral regurgitation, which required mitral valve replacement. Genome sequencing of mitral valve anterior leaflet pathology detected Tropheryma whipplei as a causal agent of culture-negative endocarditis. The patient was treated with 6 weeks of ceftriaxone and ampicillin–sulbactam and further continued trimethoprim–sulfamethoxazole for 1 year. He continued antibiotic treatment with resolution of shortness of breath along with arthralgia.

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          Most cited references14

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          Whipple's disease.

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            Infective endocarditis.

            Infective endocarditis (IE) is lethal if not aggressively treated with antibiotics alone or in combination with surgery. The epidemiology of this condition has substantially changed over the past four decades, especially in industrialized countries. Once a disease that predominantly affected young adults with previously well-identified valve disease--mostly chronic rheumatic heart disease--IE now tends to affect older patients and new at-risk groups, including intravenous-drug users, patients with intracardiac devices, and patients exposed to healthcare-associated bacteremia. As a result, skin organisms (for example, Staphylococcus spp.) are now reported as the pathogen in these populations more often than oral streptococci, which still prevail in the community and in native-valve IE. Moreover, progress in molecular diagnostics has helped to improve the diagnosis of poorly cultivable pathogens, such as Bartonella spp. and Tropheryma whipplei, which are responsible for blood-culture-negative IE more often than expected. Epidemiological data indicate that IE mostly occurs independently of medico-surgical procedures, and that circumstantial antibiotic prophylaxis is likely to protect only a minute proportion of individuals at risk. Therefore, new strategies to prevent IE--including improvement of dental hygiene, decontamination of carriers of Staphylococcus aureus, vaccination, and, possibly, antiplatelet therapy--must be explored.
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              High frequency of Tropheryma whipplei in culture-negative endocarditis.

              "Classical" Whipple's disease (cWD) is caused by Tropheryma whipplei and is characterized by arthropathy, weight loss, and diarrhea. T. whipplei infectious endocarditis (TWIE) is rarely reported, either in the context of cWD or as isolated TWIE without signs of systemic infection. The frequency of TWIE is unknown, and systematic studies are lacking. Here, we performed an observational cohort study on the incidence of T. whipplei infection in explanted heart valves in two German university centers. Cardiac valves from 1,135 patients were analyzed for bacterial infection using conventional culture techniques, PCR amplification of the bacterial 16S rRNA gene, and subsequent sequencing. T. whipplei-positive heart valves were confirmed by specific PCR, fluorescence in situ hybridization, immunohistochemistry, histological examination, and culture for T. whipplei. Bacterial endocarditis was diagnosed in 255 patients, with streptococci, staphylococci, and enterococci being the main pathogens. T. whipplei was the fourth most frequent pathogen, found in 16 (6.3%) cases, and clearly outnumbered Bartonella quintana, Coxiella burnetii, and members of the HACEK group (Haemophilus species, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella kingae). In this cohort, T. whipplei was the most commonly found pathogen associated with culture-negative infective endocarditis.
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                Author and article information

                Journal
                SAGE Open Med Case Rep
                SAGE Open Med Case Rep
                SCO
                spsco
                SAGE Open Medical Case Reports
                SAGE Publications (Sage UK: London, England )
                2050-313X
                30 June 2020
                2020
                : 8
                : 2050313X20936952
                Affiliations
                [1-2050313X20936952]Department of Internal Medicine, Reading Hospital—Tower Health System, PA, USA
                Author notes
                [*]Abigayle Sullivan, Department of Internal Medicine, Reading Hospital—Tower Health System, 420 South 5th Street West Reading, PA 19611, USA. Email: abigayle.sullivan@ 123456towerhealth.org
                Author information
                https://orcid.org/0000-0002-0876-8132
                https://orcid.org/0000-0002-8324-2827
                Article
                10.1177_2050313X20936952
                10.1177/2050313X20936952
                7328352
                2d6a12fc-f0c5-444d-a669-babcb5db7b36
                © The Author(s) 2020

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 16 September 2019
                : 31 May 2020
                Categories
                Case Report
                Custom metadata
                January-December 2020
                ts1

                tropheryma whipplei,culture-negative endocarditis,trimethoprim–sulfamethoxazole

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