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Echocardiographic Assessment and Successful Valvular Repair of Congenital Mitral Arcade.

ABSTRACT: Anomalous mitral arcade is a rare congenital anomaly affecting the mitral tensor apparatus. This condition causes a restrictive movement of the leaflets and interferes with the normal closure of the valve during ventricular systole. We report a case of a 15-year-old female with this condition and initial left ventricular dysfunction, who successfully underwent mitral valve repair. This reparative technique is more technically challenging then valve replacement and only few cases are described in literature.

SUBMITTER: Sormani P

PROVIDER: S-EPMC5353431 | biostudies-literature | 2015 Apr-Jun

REPOSITORIES: biostudies-literature

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Echocardiographic Assessment and Successful Valvular Repair of Congenital Mitral Arcade.

Sormani Paola P Chiara Benedetta De B Taglieri Corrado C Moreo Antonella A

Journal of cardiovascular echography 20150401 2

Anomalous mitral arcade is a rare congenital anomaly affecting the mitral tensor apparatus. This condition causes a restrictive movement of the leaflets and interferes with the normal closure of the valve during ventricular systole. We report a case of a 15-year-old female with this condition and initial left ventricular dysfunction, who successfully underwent mitral valve repair. This reparative technique is more technically challenging then valve replacement and only few cases are described in ...[more]

PMID: 28465933

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Project description:BackgroundMitochondrial diseases are a group of genetic disorders caused by nuclear or mitochondrial DNA gene mutations and characterized by multiorgan disorders, including cardiomyopathy. Mitochondrial cardiomyopathy is occasionally complicated by hypertrophic cardiomyopathy with/without left ventricular systolic dysfunction, dilated cardiomyopathy, and left ventricular non-compaction. In such cases, the dilated left ventricle impairs coaptation of the mitral leaflets and leads to functional mitral regurgitation. To date, valvular interventions in patients with mitochondrial cardiopathy have not been investigated.Case summaryA 64-year-old woman with mitochondrial cardiopathy was referred to our hospital owing to dyspnoea. She experienced her first admission with heart failure at age 60 years. At 62 years old, she was diagnosed with maternally inherited diabetes and deafness with mitochondrial cardiomyopathy based on mitochondrial DNA sequencing. Despite administration of guideline-directed medical therapy and high-dose taurine supplementation, she was repeatedly hospitalized for heart failure. At admission, transthoracic echocardiography revealed severe functional mitral regurgitation due to left ventricular dilatation. Surgical risk was considered high (Society of Thoracic Surgeons score of 12.6%); therefore, transcatheter edge-to-edge repair with the MitraClip system was performed. Two devices deployed at the middle segment of the anterior and posterior leaflet successfully reduced mitral regurgitation. The patient was free from cardiovascular events during the 2-year follow-up period.DiscussionTranscatheter edge-to-edge repair is a less invasive and effective treatment for severe drug-refractory mitral regurgitation in patients with mitochondrial disease. Given the limited therapeutic options for mitochondrial cardiopathy, further studies are required to uncover the mechanism underlying mitochondrial diseases and establish disease-specific treatments.

Dataset Information

Echocardiographic Assessment and Successful Valvular Repair of Congenital Mitral Arcade.

Publications

Echocardiographic Assessment and Successful Valvular Repair of Congenital Mitral Arcade.

Similar Datasets

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