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Obstructive accessory mitral valve tissue in an adult patient: a case report.

ABSTRACT: BACKGROUND:Accessory mitral valve tissue is a rare congenital anomaly that is commonly diagnosed in early childhood and rarely in adulthood. It is usually asymptomatic. However, it may cause left ventricular outflow tract obstruction in a way that mimics various other causes of obstruction. A 72-year-old Caucasian man complained of chest discomfort and exertional dyspnea for 3 months. There were no specific findings from a physical examination except systolic murmur. Transthoracic echocardiography demonstrated a mass on the mitral valve extending to the intraventricular septal, raising the pressure gradient flow across the aortic valve. Transesophageal echocardiography showed parachute-like tissue connected to the anterior leaflet of the mitral valve causing left ventricular outflow tract obstruction. During the surgery preparation period, he underwent coronary angiography and computed tomography to study the anatomy surrounding the mass. After surgery, biopsy showed non-specific findings. CONCLUSION:When facing a case of aortic valve stenosis, accessory mitral valve tissue should be kept in mind as one of the possible underlying causes despite its rarity. Although it is simple and noninvasive, echocardiography remains the best diagnostic procedure to make the correct decision about management and to define the golden time for surgical intervention.

SUBMITTER: Mardenli M

PROVIDER: S-EPMC6572748 | biostudies-literature | 2019 Jun

REPOSITORIES: biostudies-literature

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Obstructive accessory mitral valve tissue in an adult patient: a case report.

Mardenli Mahmoud M Samman Abdulla A Alkanj Hussein H Babi Amal A

Journal of medical case reports 20190617 1

<h4>Background</h4>Accessory mitral valve tissue is a rare congenital anomaly that is commonly diagnosed in early childhood and rarely in adulthood. It is usually asymptomatic. However, it may cause left ventricular outflow tract obstruction in a way that mimics various other causes of obstruction. A 72-year-old Caucasian man complained of chest discomfort and exertional dyspnea for 3 months. There were no specific findings from a physical examination except systolic murmur. Transthoracic echoca ...[more]

PMID: 31203815

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Project description:INTRODUCTION: A parachute mitral valve is defined as a unifocal attachment of mitral valve chordae tendineae independent of the number of papillary muscles. Data from the literature suggests that the valve can be distinguished on the basis of morphological features as either a parachute-like asymmetrical mitral valve or a true parachute mitral valve. A parachute-like asymmetrical mitral valve has two papillary muscles; one is elongated and located higher in the left ventricle. A true parachute mitral valve has a single papillary muscle that receives all chordae, as was present in our patient. Patients with parachute mitral valves during childhood have multilevel left-side heart obstructions, with poor outcomes without operative treatment. The finding of a parachute mitral valve in an adult patient is extremely rare, especially as an isolated lesion. In adults, the unifocal attachment of the chordae results in a slightly restricted valve opening and, more frequently, valvular regurgitation. CASE PRESENTATION: A 40-year-old Caucasian female patient was admitted to a primary care physician due to her recent symptoms of heart palpitation and chest discomfort on effort. Transthoracic echocardiography showed chordae tendineae which were elongated and formed an unusual net shape penetrating into left ventricle cavity. The parasternal short axis view of her left ventricle showed a single papillary muscle positioned on one side in the posteromedial commissure receiving all chordae. Her mitral valve orifice was slightly eccentric and the chordae were converting into a single papillary muscle. Mitral regurgitation was present and it was graded as moderate to severe. Her left atrium was enlarged. There were no signs of mitral stenosis or a subvalvular ring. She did not have a bicuspid aortic valve or coarctation of the ascending aorta. The dimensions and systolic function of her left ventricle were normal. Our patient had a normal body habitus, without signs of heart failure. Her functional status was graded as class I according to the New York Heart Association grading. CONCLUSIONS: A recently published review found that, in the last several decades, there have been only nine adult patients with parachute mitral valve disease reported, of which five had the same morphological characteristics as our patient. This case presentation should encourage doctors, especially those involved in echocardiography, to contribute their own experience, knowledge and research in parachute mitral valve disease to enrich statistical and epidemiologic databases and aid clinicians in getting acquainted with this rare disease.

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.

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Obstructive accessory mitral valve tissue in an adult patient: a case report.

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Obstructive accessory mitral valve tissue in an adult patient: a case report.

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