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Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult.

ABSTRACT: We present the case of a symptomatic young woman with mitral stenosis and regurgitation due to a congenital mitral arcade. Multimodality imaging with echocardiography and computed tomography were used for diagnosis and surgical planning. The patient underwent successful bioprosthetic valve replacement. (Level of Difficulty: Intermediate.).

SUBMITTER: Ong C

PROVIDER: S-EPMC8387843 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature

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Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult.

Ong Caroline C Brown Rachel-Maria RM Gershon Adam A Trost Biana B Hosseini Hossein H Pirelli Luigi L

JACC. Case reports 20210819 10

We present the case of a symptomatic young woman with mitral stenosis and regurgitation due to a congenital mitral arcade. Multimodality imaging with echocardiography and computed tomography were used for diagnosis and surgical planning. The patient underwent successful bioprosthetic valve replacement. (<b>Level of Difficulty: Intermediate.</b>). ...[more]

PMID: 34471883

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Project description:ObjectiveArrhythmogenic mitral valve prolapse (MVP) is an important cause of sudden cardiac death characterized by fibrosis of the papillary muscles or left ventricle (LV) wall, and an association between late gadolinium enhancement (LGE) of the LV papillary muscles and ventricular arrhythmia in MVP has been reported. However, LGE of the papillary muscles may be observed in other causes of mitral regurgitation, and it is not limited to patients with MVP. This study was to evaluate the association of LGE of the LV papillary muscles or ventricular wall on cardiac magnetic resonance imaging (CMR) and ventricular arrhythmia in patients with mitral regurgitation.Materials and methodsThis study included 88 patients (mean age ± standard deviation, 58.3 ± 12.0 years; male, 42%) with mitral regurgitation who underwent CMR. They were allocated to the MVP (n = 43) and non-MVP (n = 45) groups, and their LGE images on CMR, clinical characteristics, echocardiographic findings, and presence of arrhythmia were compared.ResultsLV myocardial wall enhancement was more frequent in the MVP group than in the non-MVP group (28% vs. 11%, p = 0.046). Papillary muscle enhancement was observed in 7 (7.9%) patients. Of the 43 patients with MVP, 15 (34.8%) showed LGE in the papillary muscles or LV myocardium, including 12 (27.9%) with LV myocardial wall enhancement and 4 (9.3%) with papillary muscle enhancement. One patient with bilateral diffuse papillary muscle enhancement experienced sudden cardiac arrest due to ventricular fibrillation. Univariable logistic regression analysis showed that high systolic blood pressure (BP; odds ratio [OR], 1.05; 95% confidence interval [CI], 1.01-1.09; p = 0.027) and ventricular arrhythmia (OR, 6.84; 95% CI, 1.29-36.19; p = 0.024) were significantly associated with LGE of the papillary muscles.ConclusionLGE of the papillary muscles was present not only in patients with MVP, but also in patients with other etiologies of mitral regurgitation, and it was associated with high systolic BP and ventricular arrhythmia. Papillary muscle enhancement on CMR should not be overlooked.

Dataset Information

Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult.

Publications

Mitral Arcade With Anomalous Papillary Muscles: Rare Cause of Mitral Stenosis in a Young Adult.

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