Acute Myocarditis Mimicking Hypertrophic Cardiomyopathy in Marfan Syndrome and Morphologically Abnormal Mitral Valve.
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ABSTRACT:
SUBMITTER: Sharrack N
PROVIDER: S-EPMC8784724 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description: Not available
| S-EPMC7451885 | biostudies-literature
Project description:Costello syndrome is a rare congenital disease caused by activating germline mutations, and it is often associated with cardiac abnormalities. A 17-year-old male with a history of Costello syndrome presented with persistent fever. Vegetation attached to anterior mitral leaflet was detected, and antibiotic therapy was administered as treatment for infectious endocarditis. However, it was difficult to manage his heart failure owing to the worsening of mitral valve regurgitation. Therefore, mitral valve repair with vegetation resection was performed. His hypertrophic cardiomyopathy and systolic anterior motion of mitral anterior leaflet caused left ventricle outflow tract obstruction. A floating stitch on the anterior mitral leaflet from the posterior ring annulus was effective. Herein, we report a successfully repaired case of infectious endocarditis on the mitral valve with hypertrophic cardiomyopathy complicated by Costello syndrome. <Learning objective: Costello syndrome is a rare congenital disease caused by activating germline mutations, and is often associated with cardiac abnormalities. However, there are few reports about cardiac surgery in a patient with Costello syndrome. Thus, we report a successfully repaired case of infectious endocarditis on the mitral valve with hypertrophic cardiomyopathy complicated by Costello syndrome. A floating stitch on the anterior mitral leaflet from the posterior ring annulus was effective.>
| S-EPMC9168975 | biostudies-literature
Project description:AimsMitral valve (MV) abnormalities are recognized features of hypertrophic cardiomyopathy (HCM), and there is preliminary evidence suggesting they are intrinsic phenotypic manifestations of sarcomere mutations, present in mutation carriers without left ventricular (LV) hypertrophy (subclinical HCM). However, further study is required to characterize the nature of these changes and their functional impact. Thus, we performed comprehensive echocardiographic analysis of MV structure and function on a genotyped population.Methods and resultsMV and papillary muscle echocardiographic parameters were measured in 192 genotyped individuals, including 50 overt HCM, 79 subclinical HCM, and 63 mutation-negative, healthy relatives as normal controls. Compared to controls, subclinical HCM subjects had elongated anterior MV leaflets relative to LV end-diastolic volume index (0.57 ± 0.02 vs. 0.51 ± 0.02 mm/mL/m2, P = 0.013) and anteriorly displaced papillary muscles [decreased papillary-septal separation (31.1 ± 0.7 vs. 34.2 ± 0.9 mm, P = 0.004) and relative antero-posterior position ratio of the papillary muscles (0.67 ± 0.01 vs. 0.71 ± 0.01, P = 0.011]. Similar findings were identified comparing overt HCM to controls. These MV changes were associated with an increased prevalence of systolic anterior motion (SAM) of the MV amongst subclinical HCM subjects.ConclusionsSarcomere mutations are associated with primary abnormalities of the MV apparatus, specifically excess anterior leaflet length relative to LV cavity size and anterior displacement of the papillary muscles; both features predisposing to SAM. These abnormalities appear to be early phenotypic consequences of sarcomere mutations, observed in mutation carriers with normal LV wall thickness.
| S-EPMC6148328 | biostudies-literature
Project description:Patients with Marfan syndrome (MFS), a multisystem disorder caused by mutations in the gene encoding the extracellular matrix (ECM) protein fibrillin 1, are unusually vulnerable to stress-induced cardiac dysfunction. The prevailing view is that MFS-associated cardiac dysfunction is the result of aortic and/or valvular disease. Here, we determined that dilated cardiomyopathy (DCM) in fibrillin 1-deficient mice is a primary manifestation resulting from ECM-induced abnormal mechanosignaling by cardiomyocytes. MFS mice displayed spontaneous emergence of an enlarged and dysfunctional heart, altered physical properties of myocardial tissue, and biochemical evidence of chronic mechanical stress, including increased angiotensin II type I receptor (AT1R) signaling and abated focal adhesion kinase (FAK) activity. Partial fibrillin 1 gene inactivation in cardiomyocytes was sufficient to precipitate DCM in otherwise phenotypically normal mice. Consistent with abnormal mechanosignaling, normal cardiac size and function were restored in MFS mice treated with an AT1R antagonist and in MFS mice lacking AT1R or β-arrestin 2, but not in MFS mice treated with an angiotensin-converting enzyme inhibitor or lacking angiotensinogen. Conversely, DCM associated with abnormal AT1R and FAK signaling was the sole abnormality in mice that were haploinsufficient for both fibrillin 1 and β1 integrin. Collectively, these findings implicate fibrillin 1 in the physiological adaptation of cardiac muscle to elevated workload.
| S-EPMC3934180 | biostudies-literature
Project description:Backgroundboth myocarditis and mitral valve prolapse (MVP) are known uncommon causes of ventricular arrhythmias in young patients.Aimto report the first clinical case of endomyocardial biopsy (EMB)-proven autoimmune myocarditis and associated arrhythmogenic MVP in a patient with recurrent ventricular fibrillation (VF) episodes.Methodsmyocarditis was diagnosed both by cardiac magnetic resonance (CMR) and EMB. Arrhythmogenic MVP was documented by transthoracic echocardiogram, CMR, and electroanatomical mapping of the trigger premature ventricular contractions (PVCs).Resultsa 22-year-old woman underwent immunosuppressive therapy after EMB-proven diagnosis of autoimmune myocarditis with VF onset and early implantable cardioverter defibrillator (ICD) placement. Three years later, she experienced two VF recurrences and persistent PVCs, despite no signs of myocarditis recurrence. An echocardiogram revealed bileaflet MVP with high arrhythmic risk features. Finally, electroanatomical mapping and ablation of the trigger PVC were successfully performed.Conclusionin patients with recurrent VF episodes despite evidence-based medical treatment for myocarditis, MVP should be considered as an alternative arrhythmogenic substrate, and warrants early ICD implant and PVC-targeted therapy.
| S-EPMC8619916 | biostudies-literature
Project description:Background and objectiveThe prevalence of hypertrophic cardiomyopathy (HCM) is estimated to be 1 in 200 to 500 individuals, with systolic anterior motion (SAM) of the mitral valve (MV) and left ventricular outflow tract (LVOT) obstruction present in 60% to 70%. In this narrative review, we aim to elucidate the pathophysiology of SAM-septal contact and LVOT obstruction in HCM by presenting a detailed review on the anatomy of the MV apparatus in HCM, examining the various existing theories pertaining to the SAM phenomenon as supported by cardiac imaging, and providing a critical assessment of management strategies for SAM in HCM.MethodsA literature review was performed using PubMed, EMBASE, Ovid, and the Cochrane Library, of all scientific articles published through December 2021. A focus was placed on descriptive studies, reports correlating echocardiographic findings with pathologic diagnosis, and outcomes studies.Key content and findingsThe pathophysiology of SAM involves the complex interplay between HCM morphology, MV apparatus anatomic abnormalities, and labile hemodynamic derangements. Echocardiography and cardiac magnetic resonance (CMR) vector flow mapping have identified drag forces, as opposed to the "Venturi effect", as the main hydraulic forces responsible for SAM. The degree of mitral regurgitation with SAM is variable, and its severity is correlated with degree of LVOT obstruction and outcomes. First line therapy for the amelioration of SAM and LVOT obstruction is medical therapy with beta-blockers, non-dihydropyridine calcium-channel blockers, and disopyramide, in conjunction with lifestyle modifications. In refractory cases septal reduction therapy is performed, which may be combined with a 'resect-plicate-release' procedure, anterior mitral leaflet extension, surgical edge-to-edge MV repair, anterior mitral leaflet retention plasty, or secondary chordal cutting.ConclusionsRecent scientific advances in the field of HCM have allowed for a maturation of our understanding of the SAM phenomenon. Cardiac imaging plays a critical role in its diagnosis, treatment, and surveillance, and in our ability to apply the appropriate therapeutic regimens. The increasing prevalence of HCM places an emphasis on continued basic and clinical research to further improve outcomes for this challenging population.
| S-EPMC9264047 | biostudies-literature
Project description:BackgroundMitral valve (MV) prolapse is common in children with Marfan syndrome (MFS) and is associated with varying degrees of mitral regurgitation (MR). However, the three-dimensional (3D) morphology of the MV in children with MFS and its relation to the degree of MR are not known. The goals of this study were to describe the 3D morphology of the MV in children with MFS and to compare it to that in normal children.MethodsThree-dimensional transthoracic echocardiography was performed in 27 patients (3-21 years of age) meeting the revised Ghent criteria for MFS and 27 normal children matched by age (±1 year). The 3D geometry of the MV apparatus in midsystole was measured, and its association with clinical and two-dimensional echocardiographic parameters was examined.ResultsCompared with age-matched control subjects, children with MFS had larger 3D annular areas (P < .02), smaller annular height/commissural width ratios (P < .001), greater billow volumes (P < .001), and smaller tenting heights, areas, and volumes (P < .001 for all). In multivariate modeling, larger leaflet billow volume in MFS was strongly associated with moderate or greater MR (P < .01). Intra- and interuser variability of 3D metrics was acceptable.ConclusionsChildren with MFS have flatter and more dilated MV annuli, greater billow volumes, and smaller tenting heights compared with normal control subjects. Larger billow volume is associated with MR. Three-dimensional MV quantification may contribute to the identification of patients with MFS and other connective tissue disorders. Further study of 3D MV geometry and its relation to the clinical progression of MV disease is warranted in this vulnerable population.
| S-EPMC6218932 | biostudies-literature
Project description: Not available
| S-EPMC8617388 | biostudies-literature
Project description:BackgroundN-terminal pro B-type natriuretic peptide (NT-proBNP) and cardiac troponin-I (cTnI) are biomarkers commonly evaluated in cats with suspected heart disease. Many cats with hypertrophic cardiomyopathy (HCM) have systolic anterior motion of the mitral valve (SAM), but its influence on circulating NT-proBNP or cTnI concentrations is currently unknown.Hypothesis/objectivesCats with HCM and SAM (HCMSAM+ ) have higher NT-proBNP and cTnI concentrations than do cats with HCM but without SAM (HCMSAM- ).AnimalsOne hundred forty cats with HCM: 70 with SAM and 70 without SAM.MethodsRetrospective case-to-case study. Cats were recruited if diagnosed with HCM by echocardiography and results were available for NT-proBNP or cTnI concentrations or both. Cats with SAM were matched to those without SAM for clinical presentation, left atrial (LA) size and left ventricular (LV) fractional shortening.ResultsA total of 119 NT-proBNP and 123 cTnI results were available. The HCMSAM+ cats had higher median concentrations than did HCMSAM- cats for NT-proBNP (729 pmoL/L; interquartile range [IQR], 275-1467 versus 65 pmoL/L; IQR, 25-271; P < .001) and cTnI (0.27 ng/mL; IQR, 0.10-0.81 versus 0.07 ng/mL; IQR, 0.01-0.43; P = .002). In general linear models for both NT-proBNP and cTnI, the independent explanatory variables were SAM, congestive heart failure, maximal LV wall thickness, and LA size.Conclusions and clinical importanceFor cats with HCM and equivalent LA size and LV systolic function, those with SAM had higher NT-proBNP and cTnI concentrations than did those without SAM. Presence of SAM should be considered when interpreting biomarker concentrations in cats with HCM.
| S-EPMC7517492 | biostudies-literature
Project description:Preservation of the subvalvular apparatus has the merits of postoperative outcomes during mitral valve replacement for mitral regurgitation. We performed mitral valve replacement with anterior and posterior leaflet chordal preservation in a 65-year-old woman. On the 2nd postoperative day, routine postoperative trans-thoracic echocardiography showed an unknown aortic subvalvular mobile mass. We report a case of a remnant mitral subvalvular apparatus detected by echocardiography after chordal preserving mitral valve replacement which was confused with postoperative aortic valve vegetation.
| S-EPMC3324732 | biostudies-literature