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A rare case of double orifice mitral valve: A case report.

ABSTRACT: Double-orifice mitral valve is a rare congenital anomaly being associated with other cardiac defects and rarely presented in isolation. Valve function can be preserved for long and it is usually an incidental finding. We present an unusual case of double-orifice mitral valve with mitral regurgitation in a middle-aged man associated with the atrial septal defect, highlighting the role of three-dimensional transesophageal echocardiography.

SUBMITTER: Kandpal SS

PROVIDER: S-EPMC8617400 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Congenital double mitral orifice with severe mitral regurgitation-associated rheumatoid arthritis: a case report.

Project description:BackgroundA double orifice mitral valve (DOMV) represents a rare congenital malformation characterized by two valve orifices with two separate subvalvular apparatus. Double orifice mitral valve is congenital anomaly of the subvalvular mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices.Case summaryA 30-year young male presented with dyspnoea and palpitation for 4 years, joint pain for 2 years and weakness of right upper limb and lower limb for 6 months. On clinical examination, Boutonniere, Swan neck, and Z-deformity of hand and foot metatarsal bone deformities are noted, on further evaluation, patient was diagnosed as a case of DOMV and was managed conservatively since patient was not willing for surgery.DiscussionTwo-dimensional echocardiography is the best detection method, the parasternal short-axis view being most useful to show DOMV.

| S-EPMC7501888 | biostudies-literature

Hammock mitral valve: a rare cause of congenital mitral regurgitation--a case report.

Project description:Hammock valve, also known as anomalous mitral arcade is a rare mechanism for congenital mitral insufficiency. We report a case of a two-week-old neonate who presented with features of heart failure and an apical systolic murmur. Echocardiogram showed severe mitral regurgitation and abnormal mitral valve with direct attachment of mitral leaflets to papillary muscle without intervening chordae tendinae, typical of hammock valve. Heart failure was controlled with ionotrpes and diuretics. The literature on the hammock mitral valve is reviewed.

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Transcatheter Tricuspid and Mitral Valve Edge-to-Edge Repair: The Double Double Orifice.

Project description:Highlights•Tricuspid regurgitation with mitral valve disease is associated with poor outcomes.•Transcatheter edge-to-edge repair of the tricuspid valve can be done successfully.•Intraprocedural echocardiography is essential for transcatheter tricuspid therapies.

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Transcatheter Edge-to-Edge Repair for Severe Mitral Regurgitation With Double-Orifice Mitral Valve.

Project description:Double-orifice mitral valve (DOMV), a rare congenital heart disease, is the inspiration behind the transcatheter edge-to-edge repair (TEER). Here we report a successful TEER case in a patient with DOMV with severe regurgitation. The patient's symptoms were greatly improved after the procedure.

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Double-Orifice Mitral Valve in an Adult with Atrioventricular Reentrant Tachycardia.

Project description: Not available

| S-EPMC6058762 | biostudies-literature

Double orifice mitral valve and bicuspid aortic valve: pieces of the same single puzzle?

Project description:Double orifice mitral valve is a very rare congenital abnormality. Well known associations of this pathology with other congenital lesions point to a complex and central pathophysiological mechanism leading to a sequence of pathologies. These associations have long been realized and arbitrarily defined as Shone complex. We would like to present a 21-year-old patient with double orifice mitral valve associated with bicuspid aortic valve, with a brief review of the literature on possible central mechanisms leading to different subsets of congenital abnormalities involving these two.

| S-EPMC4408625 | biostudies-literature

Perioperative management of a patient with double orifice mitral valve with supramitral ring with subaortic membrane with ventricular septal defect and severe pulmonary hypertension: Report of a rare case.

Project description:Double-orifice mitral valve (DOMV) is an unusual congenital anomaly characterized by a mitral valve with a single fibrous annulus with two orifices or rarely two orifices with two separate mitral annuli opening into the left ventricle. We present a first report of a patient with a DOMV with supramitral ring (SMR), subaortic membrane (SAM), a large ventricular septal defect (VSD) with more than 50% aortic override, and severe pulmonary arterial hypertrophy (PAH). This patient underwent excision of the SAM, and SMR, with closure of the VSD together under cardiopulmonary bypass (CPB). However postoperatively, the patient developed an irreversible fatal pulmonary hypertensive crisis (PHC), immediately after transferring the patient to the cardiac intensive care unit from the operating room (OR). The PHC was refractory to intravenous and inhaled milrinone and nitroglycerine and intravenous adrenaline, dobutamine, norepinephrine, vasopressin, patent foramen oval (PFO), and CPB support. The management of DOMV and perioperative pulmonary hypertension is discussed.

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Rare Case of Unileaflet Mitral Valve.

Project description:Unileaflet mitral valve is the rarest of the congenital mitral valve anomalies and is usually life threatening in infancy due to severe mitral regurgitation (MR). In most asymptomatic individuals, it is mostly due to hypoplastic posterior mitral leaflet. We present a 22-year-old male with palpitations, who was found to have an echocardiogram revealing an elongated anterior mitral valve leaflet with severely hypoplastic posterior mitral valve leaflet appearing as a unileaflet mitral valve without MR. Our case is one of the 11 reported cases in the literature so far. We hereby review those cases and conclude that these patients are likely to be at risk of developing worsening MR later in their lives.

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A rare finding of giant accessory mitral valve tissue: a case report.

Project description:Background:Accessory mitral valve tissue (AMVT) is a rare anomaly that can be detected in the first decade. It is associated with other congenital cardiac abnormalities, such as ventricular septal defect. When detected in adulthood, it is usually an incidental finding on echocardiography. Symptomatic individuals can present with breathlessness, syncope, and features of distal tissue embolization. Cardiac surgery is indicated in those with significant left ventricular outflow tract obstruction. Case summary?:A 45-year-old man without any significant medical history was referred due to an abnormal electrocardiogram. He was asymptomatic from a cardiac perspective. Echocardiography revealed the presence of a giant mobile mass attached to the anterior mitral valve leaflet and prolapsing into the left ventricular outflow tract (LVOT). This was classified as Type IIB2 AMVT. As there was no dynamic outflow tract obstruction on subsequent treadmill stress echocardiography, and in the absence of other coexistent congenital abnormality, surgical excision was not performed. Discussion?:It is important to exclude significant obstruction when a large AMVT is seen to be prolapsing into the LVOT. Three-dimensional echocardiography is the tool of choice for anatomical classification and to assess for concomitant congenital cardiac abnormalities.

| S-EPMC7047063 | biostudies-literature

Rare double orifice mitral valve malformation associated with bicuspid aortic valve in Turner syndrome: diagnosed by a series of novel three-dimensional echocardiography and literature review.

Project description:BackgroundPatients with both double orifice mitral valve (DOMV) and bicuspid aortic valve (BAV) malformation are rare. Although DOMV or BAV can be detected in some genetic syndromes, it has not been reported to simultaneously appear in Turner syndrome (TS). TrueVue, TouchVue, and TrueVue Glass are the latest technologies in advanced three-dimensional echocardiography (3DE), which is an important information supplement to two-dimensional echocardiography (2DE) for the diagnosis of congenital cardiac malformations. Herein we report the novel use of the above-mentioned technologies in the diagnosis and evaluation of a rare, combined valve malformation. Meanwhile, we also reviewed the literature for cases involving both DOMV and BAV and their association with various genetic syndromes.Case presentationWe present the case of a 5-year-old girl diagnosed with TS because of a developmental delay. DOMV and BAV were found through echocardiographic examination. Three-dimensional transthoracic echocardiography as well as a series of novel advanced techniques were applied to clearly display the spatial structure of all tiers of the mitral valve apparatus, aortic valve, and arch to facilitate an accurate diagnosis.ConclusionsThis is the first case in which both DOMV and BAV were associated with TS. Innovative TrueVue and TrueVue Glass offer unprecedented photographic stereoscopic images, while TouchVue technology greatly improved the ultrasonic diagnostic workflow and the diagnostic performance of rare valve malformations by adding virtual light sources to display realistic light-shadow effects.

| S-EPMC8336376 | biostudies-literature

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