Double-orifice Mitral Valve Research Articles

Double-orifice mitral valve associated with bicuspid aortic valve: a rare case of incomplete form of Shone's complex

Double-orifice mitral valve (DOMV) is a rare congenital malformation characterized by two separate valve orifices of varying sizes in association with the abnormalities of the subvalvular apparatus. It has been reported to be associated with a variety of other congenital malformations. We report a rare case of incomplete form of Shone's complex composed of a complete bridge-type DOMV and bicuspid aortic valve, well demonstrated by both transthoracic and transoesophageal echocardiography and cardiac magnetic resonance imaging.

Double Orifice Mitral valve with Muscular Ventricular Septal defect: A case report

Double orifice mitral valve is a rare congenital heart anomaly. We present here a case in which this anomaly was detected during routine echocardiography done for evaluation of a systolic murmur.

Double orifice mitral valve: a report of two cases

Double orifice mitral valve is rarely reported; and no report of it was encountered in medical literature from Nigeria. To document the existence of double orifice mitral valve co-existing with situs in versus in our environment. Two young men who were initially diagnosed as cases of rheumatic mitral incompetence presented for echocardiography. The investigation was for the purpose of confirming the clinical diagnosis, and to determine the functional states. They both underwent 2D and M mode echocardiographic assessment. At echocardiography, both cases turned out to have double orifice mitral valves. There were two orifices between the left atria and ventricles on gross appearance at 2 D echo; with multiple M shaped mitral valve tracings on M mode echo. The second case in addition, had situs in versus discovered on Chest X-ray and confirmed on abdominal ultrasonography. Rare as it seems, double orifice mitral valve also occurs in our environment. It can also co-exist with situs in versus as a congenital abnormality. Since it is surgically remediable, all children with cardiac murmurs should be availed of echocardiography for diagnosis and early treatment to avert cardiac dysfunction.

Echocardiographic findings in double orifice mitral valve

目的 应用彩色多普勒超声心动图研究双孔二尖瓣畸形的特点.方法 采用HDI3500超声诊断仪,对三例年龄为35～64岁的男性患者进行超声心动图检查.结果 左室短轴见二尖瓣口呈眼镜样双孔改变,每一孔口的腱索均附着到一组乳头肌上,心尖四腔观见两组瓣叶同时开放和关闭.彩色多普勒显示舒张期两股彩色血流自二尖瓣两个孔分别返流入左房. 结论超声心动图是诊断双孔二尖瓣畸形的可靠方法。

Double orifice mitral valve by real-time three-dimensional echocardiography

Double orifice mitral valve (DOMV) is a rare congenital malformation described as division of mitral orifice into two anatomically distinct orifices separated by an accessory bridge of fibrous tissue. In 85% of cases, both orifices are unequal in size. It is usually associated with other congenital defects such as atrioventricular septal defect and complex congenital heart disease. Most of cases could be diagnosed by two-dimensional echocardiography (2DE). The real-time three-dimensional echocardiography (RT3DE) helped in more detailed structure and function. Presented here RT3DE used for orientation of DOMV that allowed detailed and comprehensive assessment incremental to that obtained by 2DE.

Percutaneous and off-pump treatments for functional mitral regurgitation

A new era in the treatment of functional mitral regurgitation is emerging with new devices that can be placed percutaneously or minimally invasively without cardiopulmonary bypass. These devices are categorized into three groups: annuloplasty, edge-to-edge repair, and ventricular reshaping. Percutaneous annuloplasty devices, implanted via the coronary sinus, mimic surgical annuloplasty by reducing the mitral annular anterior-posterior (or septal-lateral) dimension. Several devices, such as the PTMA, CARILLON, Monarch, and PS3 systems, are in clinical trials. Percutaneous edge-to-edge repair devices mimic the surgical Alfieri edge-to-edge repair technique, creating a double-orifice mitral valve; of these, MitraClip is in clinical trials. Ventricular reshaping devices treat both mitral annular dilatation and papillary muscle displacement (and thus leaflet tethering). The surgical Coapsys device is currently in clinical trials, and its percutaneous "interventional" version, iCoapsys, is being prepared for a clinical trial. Numerous issues need to be addressed before these devices can become standard therapies for functional mitral regurgitation. Device safety and efficacy must be demonstrated in carefully designed clinical trials with the goal of achieving outcomes equal to or better than those of surgical repair.

Double orifice mitral valve with dysplastic tricuspid valve and intact interatrial septum: a three-dimensional echocardiographic study

Double orifice mitral valve is a rare congenital heart disease. We present a case in which 3D echocardiography helped clarify the underlying anatomy. The need exists for more extensive use of this technique to allow for surgical and pathological confirmation.

Congenital Double-Orifice Mitral Valve with Mitral Valve Prolapse and Severe Mitral Regurgitation

Introduction Double-orifice mitral valve (DOMV) is a rare congenital malformation. This anomaly is usually detected at an early age because it is frequently associated with other cardiac malformations. However, there also have been a few reports of isolated DOMV. In these patients, the mitral valve function is usually normal, and most of the patients are asymptomatic at the time of diagnosis. We experienced a case of mitral valve prolapse and severe regurgitation of congenital DOMV.

Two Cases of Double Orifice Mitral Valve Whose Three Dimensional Echocardiography Was Useful for The Classification

Two Cases of Double Orifice Mitral Valve Whose Three Dimensional Echocardiography Was Useful for The Classification

Transesophageal Echocardiography of the Edge-to-Edge Technique of Mitral Valve Repair

A 66-year-old man with a history of a large anteroapical myocardial infarction presented to the operating room for three-vessel coronary artery bypass grafting and left ventricular (LV) reconstructive surgery (Dor procedure). His baseline intraoperative transesophageal echocardiography demonstrated a dilated LV with an end diastolic diameter 7 cm and an ejection fraction 20%. A central jet of severe mitral regurgitation (MR) was present, causing systolic flow reversal in the pulmonary veins. The mechanism appeared to be dilation of the mitral valve (MV) annulus and tethering of both the anterior and posterior leaflets. A coaptation defect was clearly seen along the entire commissural line (Video clip 1, Please see video clip available at www.anesthesia-analgesia.org). After initiation of cardiopulmonary bypass (CPB) and completion of the coronary artery bypass grafting, the LV was opened and a pericardial patch sewn into place to exclude the infarcted area of myocardium. Because of the high risk of the procedure and an already long bypass time (more than 2.5 h), the decision was made not to open another area of the heart to deal with the patient’s severe MR. Instead, before closing the LV, an Alfieri stitch was placed, using the double-orifice technique (Fig. 1). This was done to increase the area of coaptation between the two leaflets. The patient was successfully weaned from CPB with inotropes and an intraaortic balloon pump. Transesophageal echocardiography examination after chest closure showed only mild MR (Video

Mitral insufficiency with a double-orifice mitral valve in an adult patient

Mitral insufficiency with a double-orifice mitral valve in an adult patient

Echocardiographic Guidance and Assessment of Percutaneous Repair for Mitral Regurgitation With the Evalve MitraClip: Lessons Learned From EVEREST I

Percutaneous mitral repair is rapidly developing as an alternative to cardiac surgery in select patients. The Evalve percutaneous E2E system uses the MitraClip to replicate the surgical suture-based approach. This procedure requires real-time echocardiographic guidance in a unique and significant collaboration between echocardiographer and interventionalist. transesophageal echocardiography (TEE) is used as the primary imaging modality to guide this procedure and is essential to its success. In EVEREST I, the US multicenter phase I safety and feasibility trial, 47 patients with 3 or 4+ mitral regurgitation (MR) were enrolled. The trial involved a standardized echocardiographic imaging protocol with a standardized anatomic-based vocabulary, predetermined standard TEE views, preprocedural strategy meetings, and display of echocardiographic aids to optimize communication and procedural efficiency during placement of the clip. TEE guidance facilitated the creation of a double-orifice mitral valve in all 47 patients enrolled (100%), and 40 patients were discharged with 1 or more clips (85%). At discharge, successful placement of a clip and <or= 2+ MR was present in 34 patients (74%). The standardized approach contributed to a reduction in the time to perform the procedure over the course of the trial at both initial and new sites. TEE is essential to the guidance of percutaneous MitraClip E2E repair. A streamlined approach to echocardiographic guidance, using predetermined standardized views, a common anatomic-based vocabulary, preprocedural strategy meetings, and a display of echocardiographic aids in the catheterization laboratory shortens the procedure time and allows for efficient percutaneous repair.

446 Double orifice mitral valve. Clinical and echocardiographic features

446 Double orifice mitral valve. Clinical and echocardiographic features

Double-Orifice Mitral Valve Associated with Noncompaction of Left Ventricular Myocardium

Double-orifice mitral valve (DOMV) is a rare anomaly commonly associated with other congenital heart diseases. We present two patients with DOMV and noncompaction of the left ventricular myocardium (NLVM). Case 1 was a 5-year-old male diagnosed with dilated cardiomyopathy. His echocardiogram showed thin myocardium with dilatation at the basal of the left ventricle, thick noncompacted myocardium around the apex of the left ventricle, and DOMV (complete bridge type) with mild mitral regurgitation. Case 2 was an 11-year-old male diagnosed with complete atrioventricular block. His echocardiogram showed thick noncompacted myocardium with mild hypokinesis from the posterior to lateral wall and DOMV (complete bridge type) with mild mitral regurgitation. DOMV is commonly associated with congenital anomaly and always has an abnormal subvalvar apparatus. The mitral valve and its apparatus embryologically originate from the endomyocardium, which is thought to be the origin of noncompacted myocardium. We speculate that patients with DOMV may have NLVM.

Real-time three-dimensional echocardiography in assessment of congenital double orifice mitral valve

The application of real-time three-dimensional echocardiography (RT 3DE) in the diagnosis of double orifice mitral valve (DOMV) was explored. Five cases of DOMV were examined by using 2-dimensional echocardiography (2DE) and RT 3DE. The spatial morphology of malformed mitral valve and its change in hemodynamics were observed. DOMV associated with partial atrioventricular septal defect was found in 3 cases (in which 2 cases had cleft mitral valve) and isolated DOMV in 2 cases; and moderate to severe mitral regurgitation was detected in 3 cases, and mild mitral regurgitation in 1, and no regurgitation in 1 case; 1 case had complicated rhumatic heart disease. Three cases were preoperatively discovered by 2DE, while 2 missed (1 case was discovered postoperatively). Four cases were diagnosed by RT 3DE preoperatively, and 1 case was diagnosed postoperatively (not examined by RT 3DE preoperatively). It was suggested that RT 3DE is a reliable technique in the diagnosis of DOMV; it permitted comprehensive and noninvasive assessment of mitral valve and may supplement 2D TTE in the assessment of DOMV.

2382: Initial observation of double orifice mitral valve by real-time three-dimensional echocardiography

2382: Initial observation of double orifice mitral valve by real-time three-dimensional echocardiography

Double orifice mitral valve; a coincidental finding☆

A double orifice mitral valve (DOMV) represents a rare congenital malformation characterised by two valve orifices with two separate subvalvular apparatus. This case demonstrates the necessity of careful imaging of the mitral valve apparatus, not only in patients with atrioventricular septal defects, but also in patients with congenital left obstructive heart disease.

Clinical Applications of Echocardiography for Patients with Stenotic Double Orifice Mitral Valve Pre- and Post-balloon Mitral Valvuloplasty

In previous studies, double orifice mitral valve (DOMV) was found to be a rare congenital anomaly, especially in isolated DOMV stenosis. The aim of this study was to demonstrate the usefulness of transthoracic echocardiography, which includes two-dimensional and Doppler echocardiography, for diagnosing and evaluating stenotic DOMV pre- and post-balloon mitral valvuloplasty (BMV). This is believed to be the first study ever reported in Taiwan. We retrospectively reviewed the echocardiographic findings of eight symptomatic patients with stenotic DOMV who successfully underwent BMV between September 2000 and March 2005. We analyzed their echocardiographic characteristics of valve morphology, size, location and transmitral pressure gradient before BMV and valve restenosis after BMV. All patients had incomplete bridge type with larger posteromedial orifice. No associated lesions were noted in any of the patients. After medical treatment and evaluation, they all underwent BMV successfully. Follow-up echocardiography showed no mitral valve restenosis. Two-dimensional echocardiography is a dependable method for diagnosing DOMV and evaluating stenotic DOMV post-BMV.

A Case with Massive Aortic Regurgitation due to Bicuspid Aortic Valve Associated with Double Orifice Mitral Valve

重複僧帽弁口は稀な先天性奇形である. 20歳, 女性の大動脈二尖弁に重複僧帽弁口を合併した症例を報告する. 収縮期駆出性雑音と, 拡張期潅水様雑音を聴取した. 経胸壁心エコー図では, 大動脈弁は二尖弁で, 僧帽弁は2個の円形の弁口を呈し, カラードプラ法心尖四腔像において拡張期に左室流入血流が2本観察され, 高度大動脈弁逆流を認めた. 大動脈二尖弁と重複僧帽弁口の合併と診断し, 大動脈弁置換術を施行した. 手術所見では, 大動脈弁は二尖弁で両尖ともに高度な肥厚と逸脱を認めた. 僧帽弁はcomplete bridge typeの重複僧帽弁口であった. 大動脈二尖弁と重複僧帽弁口合併例は稀であり, 病態の診断上2D心エコー図とカラードプラ法が有用であった.

Current Management of Severe Congenital Mitral Stenosis

Severe congenital mitral stenosis (MS) is a rare anomaly that is frequently associated with additional left heart obstructions. Anatomic treatments for congenital MS include balloon mitral valvuloplasty (BMVP), surgical mitral valvuloplasty (SMVP), and mitral valve replacement (MVR), although the optimal therapeutic strategy is unclear. Between 1985 and 2003, 108 patients with severe congenital MS underwent BMVP or surgical intervention at a median age of 18 months (range 1 month to 17.9 years). Anatomic subtypes of MS were "typical" congenital MS in 78 patients, supravalvar mitral ring in 46, parachute mitral valve in 28, and double-orifice mitral valve in 11, with multiple types in approximately 50% of patients. Additional left heart anomalies were present in 82 patients (76%). The first MS intervention was BMVP in 64 patients, SMVP in 33, and MVR in 11. BMVP decreased peak and mean MS gradients by a median of 33% and 38%, respectively (P<0.001), but was complicated by significant mitral regurgitation in 28%. Cross-sectional follow-up was obtained at 4.8+/-4.2 years. Overall, Kaplan-Meier survival was 92% at 1 month, 84% at 1 year, and 77% at 5 years, with 69% 5-year survival during the first decade of our experience and 87% since (P=0.09). Initial MVR and younger age were associated with worse survival. Survival free from failure of biventricular repair or mitral valve reintervention was 55% at 1 year among patients who underwent BMVP and 69% among patients who underwent supravalvar mitral ring resection initially. Among patients who underwent BMVP, survival free from failure of biventricular repair or MVR was 79% at 1 month and 55% at 5 years, with worse outcome in younger patients and those who developed significant postdilation mitral regurgitation. BMVP effectively relieves left ventricular inflow obstruction in most infants and children with severe congenital MS who require intervention. However, surgical resection is preferable in patients with MS due to a supravalvar mitral ring. Five-year survival is relatively poor in patients with severe congenital MS, with worse outcomes in infants and patients undergoing MVR, but has improved in our more recent experience. Many patients have undergone second procedures for either recurrent/residual MS or mitral regurgitation resulting from dilation-related disruption of the mitral valve apparatus.