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

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.

Complete and incomplete bridge type double-orifice mitral valve: Diagnosis and characterization with three-dimensional transesophageal echocardiography.

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.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Double orifice mitral valve: a report of two cases with different severity and treatment.

Background: Double orifice mitral valve (DOMV), a rare congenital heart disease, is characterized by a 2-orifice mitral valve (MV) separated by a tissue bridge, causing a spectacles-like morphology. DOMV can present with various severity ranging from asymptomatic to severe valvular dysfunction including mitral regurgitation (MR) and mitral stenosis (MS), as well as symptoms from coexisting congenital anomalies. Echocardiography is the mainstay of the investigation for a DOMV. We described two cases with DOMV who presented with different disease severity resulting in different treatment decisions. Case Description: In the first case, a 52-year-old woman presented with overt left-sided heart failure. The echocardiogram revealed DOMV with ruptured chordae tendineae of the anterior mitral valve leaflet (AMVL) causing severe MR which led the patient to undergo surgical MV replacement. Intraoperative findings confirmed a diagnosis of DOMV. After surgery, the patient could perform daily activities and light exercises without recurrent heart failure. In the second case, on the other hand, a 36-year-old woman was incidentally diagnosed with DOMV from an echocardiographic workup for symptomatic premature ventricular contraction (PVC). After controlled PVC with radiofrequency ablation, her symptom completely resolved and DOMV was classified as asymptomatic which led to the decision of a watchful waiting strategy. Conclusions: These cases highlight the diversity of DOMV manifestations and the importance of appropriate investigations, particularly echocardiography, to evaluate valvular pathology and contemplating the treatment strategy.

Repair of double orifice mitral valve with an atrioventricular septal defect in a girl with Ellis-Van Creveld syndrome.

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.

Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve, Bicuspid Aortic Valve, and Aortic Coarctation.

Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.

Case report: Echocardiographic diagnosis of double orifice mitral valve in an asymptomatic woman.

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.

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

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.

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.

BACKGROUND: 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. CASE PRESENTATION: 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. CONCLUSIONS: 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.

Echocardiographic diagnosis of congenital double orifice mitral valve malformation: A case report.

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.

Double outlet right atrium-A "bridge" across the bridging leaflets.

INTRODUCTION: 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. CASE REPORT: 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. DISCUSSION: 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. CONCLUSION: 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!

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

BACKGROUND: 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. CASE SUMMARY: 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. DISCUSSION: Two-dimensional echocardiography is the best detection method, the parasternal short-axis view being most useful to show DOMV.

Double orifice mitral valve: A rare cause of isolated severe congenital mitral regurgitation.

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.

Stenotic double-orifice mitral valve after surgical repaired partial atrioventricular septal defect.

Double-orifice mitral valve (DOMV) is an uncommon congenital anomaly account for 1% of congenital heart disease. However, accurate diagnosis and evaluation of valve stenosis or regurgitation and other concomitant congenital anomalies due to DOMV are required to obtain suitable treatment. Two- and three-dimensional echocardiography can contribute valuable functional and anatomic information that can support to reach this goal. Here, we present a case of complete bridge-type DOMV that causes mitral stenosis after surgical repair of the partial atrioventricular septal defect in childhood.

Double orifice mitral valve in a patient with bicuspid aortic valve and coarctation of the aorta: A rare presentation.

Double orifice mitral valve is a rare condition and may be accompanied by a bicuspid aortic valve (sometimes normal functioning) and coarctation of the aorta. Echocardiography is valuable in detecting the accompanied anomalies. Management depends on the severity of mitral valve function and the severity of associated anomalies.

Value of Three-Dimensional Echocardiography in Assessing Double-Orifice Mitral Valve in an Asymptomatic Patient.

Double-orifice mitral valve (DOMV) is a rare congenital anomaly consisting of an accessory bridge of fibrous tissue, which divides the mitral valve (MV) into two orifices. The mitral leaflets are essentially normal in most cases, but they can be regurgitant or stenotic. It is most commonly associated with a variety of other cardiac anomalies. Isolated DOMV with normal MV function is very rare. We present here a rare case of congenital DOMV in a 25-year-old female diagnosed by real-time three-dimensional echocardiography (RT3DE). RT3DE enabled complete anatomical and functional assessment of MV apparatus. It added much valuable information over conventional 2DE that helped in establishment of the diagnosis, identification of the anatomical type, and selection of the proper management.

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.

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.