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Severe mitral regurgitation caused by eosinophilic endocarditis.

ABSTRACT: We describe a patient with symptoms of heart failure caused by severe mitral regurgitation. Echocardiography revealed an intracardiac mass embedding the posterior mitral valve leaflet, and cardiac magnetic resonance imaging showed two intracardiac thrombi and endomyocardial fibrosis. Eosinophil count kept rising and a mutation in the gene for platelet-derived growth factor receptor alpha was found. The combination of these findings led to the diagnosis of Loeffler's endocarditis. Treatment with prednisone and a tyrosine kinase inhibitor resulted in complete remission of the hypereosinophilia and mitral valve regurgitation was only mild at 9-month follow-up visit. .

SUBMITTER: van Dongen IM

PROVIDER: S-EPMC6281715 | biostudies-literature | 2014 Sep

REPOSITORIES: biostudies-literature

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Severe mitral regurgitation caused by eosinophilic endocarditis.

van Dongen I M IM van Kraaij D J W DJW Schalla S S Brunner-La Rocca H P HP Driessen R G H RGH

Journal of cardiology cases 20140707 3

We describe a patient with symptoms of heart failure caused by severe mitral regurgitation. Echocardiography revealed an intracardiac mass embedding the posterior mitral valve leaflet, and cardiac magnetic resonance imaging showed two intracardiac thrombi and endomyocardial fibrosis. Eosinophil count kept rising and a mutation in the gene for platelet-derived growth factor receptor alpha was found. The combination of these findings led to the diagnosis of Loeffler's endocarditis. Treatment with ...[more]

PMID: 30546520

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Project description:Eosinophilic myocarditis (EM) is an under-diagnosed inflammatory heart disease that often leads to severe left ventricular (LV) dysfunction. Meanwhile, severe secondary mitral regurgitation (MR) with valve disruption, possibly requiring mitral valve repair, is rarely concomitant with EM. We present the case of a 64-year-old female diagnosed with heart failure with severe LV dysfunction and localized asynergy. Echocardiography revealed severe secondary MR with mitral valve disruption. Cardiac magnetic resonance imaging (CMR) showed transmural late-gadolinium enhancement localized in the anterior wall and diffuse high-signal areas on T2-weighted images, suggesting non-ischemic and inflammatory heart disease. Although the peripheral eosinophil count was not elevated on admission, it gradually increased during hospitalization. These findings encouraged us to perform endomyocardial biopsy, which confirmed myocardial eosinophilic infiltration with mild fibrosis and necrosis, leading to the diagnosis of EM. Immunosuppressive treatment with oral corticosteroids improved LV dysfunction and completely resolved severe secondary MR. The current case highlighted that comprehensive assessment of laboratory, imaging, and pathological examinations including CMR is crucial to develop the appropriate therapeutic strategy for refractory heart failure. Immunosuppressive treatment should be considered as the first therapeutic option even in EM cases with severe secondary MR, possibly requiring mitral valve repair. <Learning objective:The gradual increase in peripheral eosinophils during hospitalization, without significant peripheral eosinophilia on admission, is crucial for the diagnosis of eosinophilic myocarditis. Comprehensive assessment of laboratory, imaging, and pathological examinations including cardiac magnetic resonance imaging is mandatory when building an appropriate therapeutic strategy for refractory heart failure. Severe secondary mitral regurgitation with mitral valve disruption can be completely resolved via immunosuppressive treatment in cases of eosinophilic myocarditis.>.

Project description:OBJECTIVES:The Cardiothoracic Surgical Trials Network recently reported no difference in the primary end point of left ventricular end-systolic volume index at 1 year postsurgery in patients randomized to repair (n = 126) or replacement (n = 125) for severe ischemic mitral regurgitation. However, patients undergoing repair experienced significantly more recurrent mitral regurgitation than patients undergoing replacement (32.6% vs 2.3%). We examined whether baseline echocardiographic and clinical characteristics could identify those who will develop moderate/severe recurrent mitral regurgitation or die. METHODS:Our analysis includes 116 patients who were randomized to and received mitral valve repair. Logistic regression was used to estimate a model-based probability of recurrence or death from baseline factors. Receiver operating characteristic curves were constructed from these estimated probabilities to determine classification cut-points maximizing accuracy of prediction based on sensitivity and specificity. RESULTS:Of the 116 patients, 6 received a replacement before leaving the operating room; all other patients had mild or less mitral regurgitation on intraoperative echocardiogram after repair. During the 2-year follow-up period, 76 patients developed moderate/severe mitral regurgitation or died (53 mitral regurgitation recurrences, 13 mitral regurgitation recurrences and death, and 10 deaths). The mechanism for recurrent mitral regurgitation was largely mitral valve leaflet tethering. Our model (including age, body mass index, sex, race, effective regurgitant orifice area, basal aneurysm/dyskinesis, New York Heart Association class, history of coronary artery bypass grafting, percutaneous coronary intervention, or ventricular arrhythmias) yielded an area under the receiver operating characteristic curve of 0.82. CONCLUSIONS:The model demonstrated good discrimination in identifying patients who will survive 2 years without recurrent mitral regurgitation after mitral valve repair. Although our results require validation, they offer a clinically relevant risk score for selection of surgical candidates for this procedure.

Project description:A 67-year-old man with myelodysplastic syndrome, disseminated histoplasmosis, and mitral valve replacement presented with dyspnea and peripheral edema. Transthoracic echocardiography demonstrated abnormal pulmonic valve with possible vegetation. Color flow imaging showed laminar flow from main pulmonary artery into right ventricular outflow tract (RVOT) in diastole. The continuous wave Doppler signal showed dense diastolic envelope with steep deceleration slope. These findings were consistent with severe pulmonic valve regurgitation, possibly due to endocarditis. Transesophageal echocardiography demonstrated an echodense mass attached to the pulmonic valve. The mitral valve bioprosthesis appeared intact. Bacterial and fungal blood cultures were negative; however, serum histoplasma antigen was positive. At surgery, the valve appeared destroyed by vegetations. Gomori methenamine silver-stains showed invasive fungal hyphae and yeast consistent with a dimorphic fungus. Valve cultures grew one colony of filamentous fungus. Itraconazole was continued based on expert infectious diseases diagnosis. After surgery, dyspnea and ankle edema resolved. To the best of our knowledge, histoplasma endocarditis of pulmonic valve has not been previously reported. Isolated pulmonic valve endocarditis is rare, accounting for about 2% of infectious endocarditis (IE) cases. Fungi account for about 3% of cases of native valve endocarditis. Characterization of pulmonary valve requires thorough interrogation with 2D and Doppler echocardiography techniques. Parasternal RVOT view allowed visualization of the pulmonary valve and assessment of regurgitation severity. As an anterior structure, it may be difficult to image with transesophageal echocardiography. Mid-esophageal right ventricular inflow-outflow view clearly showed the pulmonary valve and vegetation.Identification and characterization of pulmonary valve abnormalities require thorough interrogation with 2D and Doppler echocardiography techniques.Isolated pulmonary valve IE is rare and requires high index of suspicion. Histoplasma capsulatum IE is rare and requires high index of suspicion.

Project description:BackgroundIschemic mitral regurgitation is associated with a substantial risk of death. Practice guidelines recommend surgery for patients with a severe form of this condition but acknowledge that the supporting evidence for repair or replacement is limited.MethodsWe randomly assigned 251 patients with severe ischemic mitral regurgitation to undergo either mitral-valve repair or chordal-sparing replacement in order to evaluate efficacy and safety. The primary end point was the left ventricular end-systolic volume index (LVESVI) at 12 months, as assessed with the use of a Wilcoxon rank-sum test in which deaths were categorized below the lowest LVESVI rank.ResultsAt 12 months, the mean LVESVI among surviving patients was 54.6±25.0 ml per square meter of body-surface area in the repair group and 60.7±31.5 ml per square meter in the replacement group (mean change from baseline, -6.6 and -6.8 ml per square meter, respectively). The rate of death was 14.3% in the repair group and 17.6% in the replacement group (hazard ratio with repair, 0.79; 95% confidence interval, 0.42 to 1.47; P=0.45 by the log-rank test). There was no significant between-group difference in LVESVI after adjustment for death (z score, 1.33; P=0.18). The rate of moderate or severe recurrence of mitral regurgitation at 12 months was higher in the repair group than in the replacement group (32.6% vs. 2.3%, P<0.001). There were no significant between-group differences in the rate of a composite of major adverse cardiac or cerebrovascular events, in functional status, or in quality of life at 12 months.ConclusionsWe observed no significant difference in left ventricular reverse remodeling or survival at 12 months between patients who underwent mitral-valve repair and those who underwent mitral-valve replacement. Replacement provided a more durable correction of mitral regurgitation, but there was no significant between-group difference in clinical outcomes. (Funded by the National Institutes of Health and the Canadian Institutes of Health; ClinicalTrials.gov number, NCT00807040.).

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Severe mitral regurgitation caused by eosinophilic endocarditis.

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Severe mitral regurgitation caused by eosinophilic endocarditis.

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