Double-orifice Mitral Valve Research Articles

Complete atrioventricular septal defect with double orifice mitral valve in a calf with congestive heart failure

AbstractA 3–4‐week‐old, suckler, bull calf from Durham County, United Kingdom died due to congestive heart failure. Postmortem examination identified a Type C complete atrioventricular septal defect with concurrent double orifice mitral valve as the cause of the heart failure. Both atrioventricular septal defects and double orifice mitral valves have only rarely been reported in cattle, and never concurrently, to the authors’ knowledge.

Double orifice mitral valve: rare disease with a wide range of presentation

Double orifice mitral valve: rare disease with a wide range of presentation

Risk factors for mitral valve dysfunction, reoperation and death following repair of the primary mitral valve disease in children.

After primary mitral valve (MV) repair, residual mitral valve regurgitation (MR) and recurred mitral valve stenosis (MS) are the principal occurrences. This study's purpose is to identify the risk factors of MV dysfunction, reoperation and death following repair of primary MV diseases. We retrospectively reviewed 98 patients (47 males and 51 females) with primary MV diseases between January 2013 and December 2021. The median age was 34 months [interquartile range (IQR), 11.4-59] for male and 24 months (IQR, 7.35-72) for female. The left ventricular ejection fraction (LVEF), the left ventricular end-diastolic volume index (LVEDVI) and left ventricular end-systolic volume index (LVESVI) were assessed to evaluate patient's left ventricular function. Risk factors that increased the likelihood of MV dysfunction, reoperation and death after surgery were investigated. During the 23.5 months (IQR, 9-44.5) of follow-up, 5 (5.1%) patients died, including one early death and two late deaths (n=3; 3.9%) in the MR group and one early death and one late death (n=2; 9.1%) in the MS group. Seven (9.2%) patients in the primary MR disease group and 2 (9.1%) patients in the primary MS disease group required a second MV operation for a total reoperation rate of 9.2% (9/98). As of the most recent follow-up, 34 patients experienced MV dysfunction. No significant difference was recorded between primary MR and MS disease groups in Kaplan-Meier freedom from MV dysfunction and reoperation. Mixed MV pathology (P=0.014) acted as an independent risk factor for MV dysfunction, and ≥ moderate MR at 24 h after first surgery (P=0.014) an independent risk factor for MV reoperation. Double-orifice MV technique (P=0.002), MV reoperation (P=0.023) and severe MR at 24 h after first surgery (P=0.028) were independent risk factors for death. The Kaplan-Meier freedom from MV dysfunction and reoperation were comparable between primary MR and MS disease groups. A high probability of MV dysfunction was predicted due to the mixed MV pathology. Patients with ≥ moderate MR at 24 h after first surgery had a higher risk of MV reoperation. Double-orifice MV technique, MV reoperation and severe MR at 24 h after first surgery had a higher risk for death.

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

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. 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. 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.

Novel arm-width-expandable transcatheter edge-to-edge repair system: Preclinical experiment and first-in-human study.

The ValveClasp system is a novel transcatheter edge-to-edge repair (TEER) device with an arm-width-expandable clip that allows treatment of patients with only one clip more frequently. This study aimed to evaluate the feasibility and safety of a novel TEER device in porcine models and patients. Fourteen young adult pigs were enrolled. A clip with an expanded arm was implanted under epicardial echocardiography and fluoroscopy guidance. Five patients with at least moderate-to-severe mitral regurgitation underwent TEER using the ValveClasp system to test the safety and effectiveness of the device. The device success rate was 100% (14/14) in the animal experiments, and all clips were deployed at the A2P2 segments, forming a double-orifice mitral valve. Gross observations on day 180 showed a wide and continuous tissue bridge between the leaflets. The acute procedural success rate was 100% (5/5). Only one clip was required in all patients, and all achieved effective postoperative endpoints (grade ≤2+). During 30-day follow-up, no adverse events occurred. All patients'vena Contracta width (from 8.04 0.71 mmto 3.84 ± 1.18 mm, p = 0.012), mitral regurgitation area (from 12.75 ± 3.13 cm2 to 3.50 ± 1.66 cm2 , p = 0.008), and left ventricular end diastolic diameter (from 52.00 ± 2.92 mm to 46.00 ± 3.08 mm, p = 0.040) were considerably decreased, without obvious mitral stenosis. The novel arm-width-expandable ValveClasp device is safe for TEER for treating severe mitral regurgitation.

Double Orifice Mitral Valve Associated with Bicuspid Aortic Valve: A Case Report

Double Orifice Mitral Valve Associated with Bicuspid Aortic Valve: A Case Report

Windows, Wings and Wonder: Advanced Cardiac Imaging Via Transesophageal Two-Dimensional and Transthoracic Three-Dimensional Echocardiography for the Accurate Diagnosis of Double-Orifice Mitral Valve in the Background of Primum Atrial Septal Defect: A Case Report

The assessment of mitral valve anatomy can be difficult or even misleading on standard cardiac imaging procedures. Such is the scenario in a 40-year-old woman with an incidental finding of atrial septal defect (ASD) and consideration of a trileaflet versus double-orifice mitral valve (DOMV) on transthoracic two-dimensional echocardiography. Further interrogation using transesophageal two-dimensional and transthoracic three-dimensional (3D) echocardiography (to allow more accurate assessment and simultaneous viewing of multiple imaging planes) revealed the typical “seagull wing” configuration of the mitral valve on long-axis view and two distinct but nearly equal-sized orifices on short-axis view, one oriented anteriorly and the other posteriorly. The patient’s DOMV was classified as an incomplete bridge type as confirmed on 3D imaging, with each orifice having its own set of papillary muscles and chordal attachments. The mitral regurgitation was graded as severe, resulting from leaflet prolapse of the anterior orifice. No left ventricular outflow tract obstruction was demonstrated in this case, and the overall left ventricular systolic function was preserved. The ASD was of the primum type, with the configuration and exact location verified through 3D imaging. The accurate detection of congenital anomalies via noninvasive techniques and complementary advanced modalities is vital for preoperative planning, as well as anticipation of potential complications related to the structural anomalies. To our knowledge, this is the first local report of DOMV with primum ASD in an adult. KEYWORDS: double-orifice mitral valve, primum atrial septal defect, transesophageal and three dimensional echocardiography

Two Holes in One: A Rare Case of Congenital Mitral Valve Anomaly

Double orifice mitral valve (DOMV) is a rare anomaly, characterised by a mitral valve with a single fibrous annulus resulting in two orifices. Since its discovery in the late 19th century, around 200 cases have been reported. In the Philippines, this mitral valve anomaly has not been reported in literature. The authors are reporting this case to provide information in identifying DOMV using 2D and 3D imaging. The case involves a 26-year-old Filipino man with an incidental finding of an isolated double-orifice mitral valve. Additional imaging using 2D and 3D transoesophageal echocardiography was performed to assess the mitral valve morphology, function and possible associated anomalies. The en face view of the mitral valve showed a complete bridge of tissue extending from the middle of the anterior to the posterior leaflet, creating medial and lateral orifices. The patient presented with an isolated DOMV; hence, no intervention was necessary. However, long-term follow-up is advised.

Double orifice mitral valve in an elderly patient.

Journal Article Corrected proof Double orifice mitral valve in an elderly patient Get access May Khei Hu, May Khei Hu Department of Cardiology, Birmingham City Hospital, Dudley Road, Birmingham B18 7QH, United Kingdom Corresponding author. E-mail: maykhei.hu@nhs.net Search for other works by this author on: Oxford Academic PubMed Google Scholar Nina Huppertz, Nina Huppertz Department of Cardiology, Birmingham City Hospital, Dudley Road, Birmingham B18 7QH, United Kingdom Search for other works by this author on: Oxford Academic PubMed Google Scholar Stavros Apostolakis, Stavros Apostolakis Department of Cardiology, Birmingham City Hospital, Dudley Road, Birmingham B18 7QH, United Kingdom Search for other works by this author on: Oxford Academic PubMed Google Scholar Fairoz Abdul Fairoz Abdul Department of Cardiology, Birmingham City Hospital, Dudley Road, Birmingham B18 7QH, United Kingdom Search for other works by this author on: Oxford Academic PubMed Google Scholar European Heart Journal - Cardiovascular Imaging, jead084, https://doi.org/10.1093/ehjci/jead084 Published: 04 May 2023

The great performance of the edge-to-edge repair in the anterior leaflet prolapse: a surprise?

In the meta-analysis of Khairallah et al. [1], 2 surgical techniques (the edge-to-edge repair and the neochordal repair) to correct mitral regurgitation (MR) due to isolated anterior leaflet prolapse are compared. A relevant finding is that a lower incidence of recurrent MR was observed in the follow-up when the lesion was corrected using the edge-to-edge repair. The first edge-to-edge mitral valve repair was carried out by us about 30 years ago in a patient with severe MR due to an isolated anterior leaflet prolapse. The functional result was perfect: the newly created double-orifice mitral valve was totally competent, and the global mitral valve area after implantation of a prosthetic ring was well above 3 cm2. At that time (early 1990s), the overall mitral valve repair rate in the surgical community was rather low, particularly in some subsets of lesions due to the complexity and/or ineffectiveness of the available surgical techniques. Triangular resection, chordal transposition and chordal shortening for anterior leaflet prolapse were not uncommonly associated with haemodynamically significant residual or recurrent MR and suboptimal results, even in experienced hands, were repeatedly reported [2–5]. On the contrary, the prolapse of the posterior leaflet, by far the most common lesion in degenerative MR, already at that time was associated with very satisfactory short- and long-term outcomes.

Isolated double orifice mitral valve: An extremely rare cardiac anomaly

Isolated double orifice mitral valve: An extremely rare cardiac anomaly

Neonatal Blues: Cyanosis and Failure to Thrive in a Newborn

Congenital heart disease is a spectrum of structural anatomic defects, and clinical manifestations arise from a combination of fixed anatomy and dynamic physiologic processes. We describe a newborn with acyanotic congenital heart disease who rapidly developed hypoxemia, cyanosis, respiratory distress, and failure to thrive. Less than 2 weeks after birth, an unusual constellation of cardiac anatomy (a large ventricular septal defect, double orifice mitral valve, and supramitral ring) cumulatively yielded cyanotic physiology. The acute clinical change prompted immediate anti-congestive therapy and urgent cardiothoracic surgery. This case is the first report of successful neonatal cardiac surgery for this cyanotic constellation of defects, which are independently classified as acyanotic structural defects. It is an important reminder that newborns with multiple intracardiac lesions may behave unpredictably throughout the neonatal transition period, and the cardiac differential diagnosis need not be strictly tied to the more common cyanotic anatomy (i.e., limited to one of the “classic five T’s” and single ventricle). Int J Clin Pediatr. 2022;11(2):51-55 doi: https://doi.org/10.14740/ijcp486

Real-time 3D transoesophageal echocardiography visualization of congenital double orifice mitral valve.

Real-time 3D transoesophageal echocardiography visualization of congenital double orifice mitral valve.

A Rare Combination of Double Orifice Mitral Valve With Noncompaction of Left Ventricle

A 52-year-old gentleman, with a diagnosis of triple vessel coronary artery disease, underwent coronary artery bypass grafting. Intraoperative transesophageal echocardiography revealed an incidental diagnosis of double orifice mitral valve (DOMV) along with left ventricular noncompaction (LVNC). Three-dimensional zoom views showed the medial and lateral orifices of the DOMV and its relationship with adjacent valves (Figure 1, left atrial view; Figure 2, left ventricular view). In the midesophageal 2-chamber view (Figure 3A), the Chin index confirmed the LVNC.

A Young Girl with Barely Symptomatic Congenital Mitral Valve Disease

Congenital mitral valve disease or malformation may be isolated or associated with other cardiac anomalies. Mitral valve may affect its different segments during development. This anomaly is due to mitral valve with a single fibrous annulus with two orifices (Double orifices mitral valve) that open into the left ventricle. It may be associated with stenosis and or mitral regurgitation. Here we describe a 17 years young girl with palpitation and shortness of breath on moderate to severe exertion. Examination of the cardiovascular system, chest including precordium revealed no abnormality. She is acyanotic and there is no clubbing. On Trans thoracic Echocardiogram isolated double orifice mitral valve was found with no other associated congenital anomalies. Cardiovasc j 2021; 14(1): 79-81

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

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.

Isolated double-orifice mitral valve: an atypical morphology in adult patient.

Isolated double-orifice mitral valve: an atypical morphology in adult patient.

A Case of Double Orifice Mitral Valve in a Patient with Bicuspid Aortic Valve: Coincidental or a Missed Finding?

A Case of Double Orifice Mitral Valve in a Patient with Bicuspid Aortic Valve: Coincidental or a Missed Finding?

Abstract 14338: Prevalence, Associations and Outcomes of Double Orifice and Parachute Mitral Valve

Background: The incidence of congenital mitral valve disease is 0.4%; Double Orifice Mitral Valve (DOMV) and Parachute Mitral Valve (PMV) are two morphologic pathologies that may result in mitral valve dysfunction. The objectives of this study are 1) To describe valve function and progression and 2) To define factors contributing to disease progression. Methods: Retrospective database review. Fyler codes for DOMV, PMV and text search was performed. Echocardiographic images, echo reports, and chart review were used to identify mitral regurgitation (MR), mitral stenosis (MS), morphology, and associated lesions. Results: 39 patients with DOMV and 76 patients with PMV were identified. In the DOMV cohort, 51% were male, median age at diagnosis was 0.17 years (IQR 0.01, 3.88); median follow-up of 5.92 years (IQR 0.46, 10.22). In the PMV cohort, 44% were male, median age at diagnosis at was 0.01 years (IQR 0, 0.34); median follow-up of 2.56 years (IQR 0.25, 9.55). 41% of DOMV and 23% of patients with PMV had normal valve function at initial visit. DOMV was associated with MR (p=0.04), and PMV with MS (p&lt;0.0001). 23% of patients in the PMV cohort had progressive MS compared to 5% of patients in the DOMV cohort (p&lt;0.0001). There was no significant difference in MR progression between both groups (p=0.02). Papillary muscle (PM) morphology was evaluated in 37 (excluding canals) of 76 patients in the PMV cohort. 5 had true PMV (single PM), 32 had variant PMV with two PM groups of which 62.5% had dominant posterior medial PM. 67% of those with posterior medial PM dominance had progressive MS irrespective of association with Shone’s complex. The anterolateral PM muscle group dominant PMV were not associated with Shone’s complex and progressive MS. Conclusion: DOMV are more likely to have MR while PMV are more likely to have MS. DOMV has non progressive MR and MS. Posterior medial PM dominance in PMV is more likely to have progressive MS.

Double-orifice mitral valve associated with multivalvular involvement: a new entity?

Double-orifice mitral valve associated with multivalvular involvement: a new entity?