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Double orifice mitral valve is a rare congenital anomaly that is often associated with other congenital cardiac abnormalities. Patients may present with valvular dysfunction or may be asymptomatic and have an incidental diagnosis of double outlet mitral valve. Whether symptoms due to this pathology are present or not it is important to accurately characterize this lesion. Three-dimensional imaging is a powerful diagnostic modality for diagnosing and characterizing lesions such as this as highlighted in this case image series where we describe how three-dimensional transesophageal echocardiography was utilized to characterize both complete and incomplete bridge type double orifice mitral valve.

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Double orifice mitral valve (DOMV) is a rare congenital anomaly that is often associated with cardiac malformation. Valve dysfunction usually presents in childhood; therefore, most cases are diagnosed with DOMV in childhood. Its prevalence and prognostic relevance in adulthood are unknown. Here, we report a case of a 38-year-old woman who presented to the outpatient clinic with an abnormal electrocardiogram and was found to have an isolated double orifice mitral valve malformation on transthoracic echocardiography. Echocardiography is the diagnostic tool of choice for patients with double orifice mitral valves. We should familiarize ourselves with the echocardiographic features of DOMV in order to improve the detection.

Ellis-van Creveld syndrome is a rare autosomal recessive disorder caused by mutations in the EVC and EVC2 genes. The four principal manifestations are chondrodysplasia, polydactyly, ectodermal dysplasia, and congenital heart defects. We describe the case of a 7-year-old girl with Ellis-van Creveld Syndrome with the diagnosis of common atrium and partial atrioventricular septal defect. She underwent a successful surgical repair, and intraoperatively, a double orifice mitral valve was diagnosed as well. The correct diagnosis of this disorder in early life is essential in the overall prognosis of the syndrome. Clinical follow-up at regular intervals is very important in these patients to institute proper managements and prevent further complications.

Patients 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.
We 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.
This 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.

Double orifice mitral valve (DOMV) is a rare congenital valve malformation. Due to the insufficient understanding and awareness of its echocardiographic characteristics, there is a risk of misdiagnosis. Here, we report a case of echocardiographic diagnosis of congenital DOMV malformation in a 56-year-old male patient who visited our hospital for systematic physical examination, during which DOMV was not diagnosed. The following year, the patient visited our hospital again, and echocardiography showed two approximately circular orifices on the short axis view of the mitral valve. During diastole, two red Doppler blood flow signals were observed entering the left ventricle simultaneously. A small amount of blue Doppler signal revealed reflux into the left atrium during systole, leading to the diagnose of congenital heart disease with DOMV malformation, without other cardiac structural abnormality. Because the patient did not have obvious discomfort, he did not receive any special treatment and is regularly followed-up.

BACKGROUND: Mal-alignment between the inter-atrial septum (IAS) and the inter-ventricular septum (IVS) during cardiac embryogenesis results in abnormal atrioventricular (AV) connections ranging from doubleoulet left atrium (DOLA) to double outlet right atrium (DORA)1.
METHODS: We report DORA resulting from partial override of the interatrial septum (IAS) across the cleft between the bridging leaflets of the left atrioventricular (AV) valve.
RESULTS: Successful surgical management with preservation of the orifice is described, leading to an unusual type of double orifice left AV valve.
CONCLUSIONS: The mal-alignment of the interatrial septum, resulting in it bridging the bridging leaflets, leads to an additional orifice between the RA and the LV and a unique form of right to left shunt. DORA can cause cyanosis depending on the size of the shunt and the compliance of the ventricles. Our patient was acyanotic, possibly due to the small size of the orifice and low LV compliance secondary to hypertrophy of the LV.
CONCLUSIONS: our case adds to the spectrum of atrial septal mal-alignment variant of DORA in the setting of AVSD without atrial and ventricular components, with RA to LV communication occurring via the smaller orifice of a left atrioventricular valve divided by the abnormal insertion of a partially overriding, mal-aligned atrial septum; a mechanism not described previously - the \'bridge\' across the bridging leaflets!

UNASSIGNED: A 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.
UNASSIGNED: A 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.
UNASSIGNED: Two-dimensional echocardiography is the best detection method, the parasternal short-axis view being most useful to show DOMV.

Double orifice mitral valve (DOMV) is a rare congenital anomaly of the mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices. It usually occurs as an associated lesion. Encountering a DOMV as an isolated finding meriting intervention is indeed rare. We, here, report a case of \"complete bridging type DOMV\" occurring as an isolated entity and presenting as severe congenital MR requiring surgery.