An accessory mitral valve leaflet causing left ventricular outflow tract obstruction and associated with severe aortic incompetence

This case report describes a 20-year-old woman with Turner's syndrome who presented with reduced effort tolerance limited by dyspnea. She had previously been on pediatric cardiology follow-up for congenital subvalvular aortic stenosis first diagnosed at age 7. Unfortunately she defaulted after two visits before any intervention could be done. Transthoracic echocardiography demonstrated severe aortic incompetence (AI) with a membrane-like structure in the left ventricular outflow tract (LVOT). The mean pressure gradient across the LVOT on continuous wave Doppler was 41 mmHg. The membranous interventricular septum appeared aneurysmal and it was observed that the "subaortic membrane" had a connection to the anterolateral papillary muscle via a strand of chordal tissue. Further images were captured using two-dimensional and three-dimensional transthoracic and transesophageal echocardiography (iE33, Philips Medical Systems, Andover, MA, USA). After a review of the literature it was concluded that this appeared to be an accessory mitral valve (AMV) leaflet causing LVOT obstruction associated with AI. AMV tissue is a rare congenital malformation causing LVOT obstruction. Because it is so unusual, it may not be immediately recognizable even in a high volume echocardiography laboratory. The clue which helped with the diagnosis was the strand of chordal tissue which connected the mass to the papillary muscle. This anomaly is often associated with LVOT obstruction.     

Critical left ventricular outflow tract obstruction due to accessory mitral valve tissue

Left ventricular outflow tract (LVOT) obstruction due to anomalous tissue tag arising from the mitral valve is a rare congenital cardiac anomaly. It generally becomes symptomatic during the first decade of life as exercise intolerance, chest pain, or syncope at effort. To date, only a few cases of critical systemic obstruction due to isolated mitral valve anomaly in neonates have been reported. We report the case of a neonate who was a few hours old and was referred in severe clinical condition due to critical left ventricular outflow obstruction resulting from an anomalous tissue tag of mitral valve origin.     

Malaligned bioprosthetic valve causing left ventricular outflow tract obstruction

Left ventricular outflow tract obstruction resulting from strut impingement upon the interventricular septum is a rare complication of bioprosthetic mitral valve insertion. Obstruction is more likely to develop when a small, high profile prosthetic valve is inserted into a patient with a small outflow tract. The likelihood of this complication may be reduced by appropriate modification of surgical technique.     

Transient left ventricular outflow tract obstruction with systolic anterior motion of the mitral valve: A stunning cause

Left ventricular outflow tract obstruction (LVOTO) and systolic anterior motion (SAM) of the mitral valve may have various etiologies, of which hypertrophic cardiomyopathy is the most common. More rarely, an acute coronary syndrome, myocardial stunning, and takotsubo cardiomyopathy may give rise to LVOTO and SAM. Here, we present a 70‐year‐old female patient with a non‐ST‐elevation acute coronary syndrome treated with percutaneous coronary intervention. Echocardiography the day after, because of dyspnea and hypotension, revealed apical akinesia, LVOTO, and SAM, which proved completely reversible after treatment with a β‐blocker and a 2‐month follow‐up period. It was concluded that postischemic apical stunning had caused LVOTO and SAM.     

Mechanisms,pathophysiology, and diagnostic imaging of left ventricular outflow tract obstruction following mitral valve surgery and transcatheter mitral valve replacement

Left ventricular outflow tract obstruction is a serious complication of mitral valve surgery (repair and replacement) and transcatheter mitral valve replacement. An appreciation of the various mechanisms which cause outflow obstruction in these settings is critical to avoiding this complication and to initiating appropriate treatment. This article discusses the mechanisms, pathophysiology, and imaging of left ventricular outflow tract obstruction which can arise following insertion of a variety of mitral valve prosthetics.     

Unusually large left atrial myxoma presenting with severe mitral valve obstruction symptoms

A 13-year-old girl with the complaint of severe mitral valve obstruction symptoms was diagnosed as having an unusually large left atrial tumor by echocardiography. The giant mass was surgically removed and the postoperative course was uneventful. Histologic examination confirmed the mass was a benign atrial myxoma.     

Ischemic mitral valve repair: predictive significance of restrictive left ventricular diastolic filling

AIM: The aim of the present study was to evaluate the impact of left ventricular (LV) diastolic filling impairment on postoperative results in patients with low LV ejection fraction (EF) undergoing combined coronary artery bypass grafting (CABG) and mitral valve (MV) repair. METHODS AND RESULTS: Study population consisted of 53 patients with ischemic MV incompetence and LV systolic dysfunction (mean EF-26.1 +/- 6%), who underwent CABG with MV repair. Patients were divided into three groups according to the LV diastolic filling pattern. Study protocol included evaluation of perioperative mortality (30 days inhospital mortality), NYHA functional class, and two-dimensional Doppler echocardiographic examination preoperatively, 10-14 days, and 12 months after surgery. The highest perioperative mortality rate (33.3%), unimproved functional status (in 78.5% of the patients, NYHA functional class remained unchanged late after surgery), and hemodynamic deterioration (LV dilatation, progression of mitral regurgitation (MR) was observed in the restriction group). Though early after surgery, MR reduction was significant in this group, at even one year after surgery 85.7% of patients presented with >grade 1 of MR (P < 0.05). Logistic regression analysis showed that restrictive LV diastolic filling is an important independent preoperative marker (P = 0.035) of progression of MR late after MV repair. CONCLUSION: In patients with severe LV dysfunction undergoing combined CABG and MV repair, restrictive LV diastolic filling pattern is an important preoperative marker of high perioperative mortality rate, further negative remodeling of LV, and progression of mitral regurgitation late after MV repair.     

Subvalvular left ventricular pseudoaneurysm: A rare complication of mitral valve replacement

A patient who developed a subvalvular left ventricular paeudoaneurysm following mitral valve replacement is presented, the fourth such case in the literature. The etiology of this uncommon complication is felt to be caused by excessive surgical resection of the diseased nitral valve, which may weaken or cause rupture of the heart at the mitral annulus. The diagnosis can only be made by left ventriculography. Surgical treatment is indicated, because rupture of the pseudoaneurysm can occur.     

二尖瓣修复术治疗左室功能减退的二尖瓣关闭不全患者效果的随访观察

目的探讨二尖瓣修复术（MVr）对左室功能减退的二尖瓣关闭不全（MR）患者的治疗效果。方法回顾性分析近9年来62例中重度MR并发左室功能减退患者的临床资料、手术方法和超声心动图围术期观察及术后随访对比。结果全组无手术死亡,术后无重大并发症;随访（58±13）月,晚期死亡2例（3%）;失访7例,随访患者恢复心功能Ⅰ级29例（53%）,Ⅱ级26例（47%）;1年后复查49例超声心动图结果显示二尖瓣返流0～轻微36例（73%）,轻度10例（20%）、轻～中度3例（7%）,术后2周、1年心脏超声观察左室舒张末期直径（LVEDD）、左室收缩末期直径（LVESD）及左房直径（LAD）较术前明显缩小,左室射血分数（LVEF）及短轴缩短率（LVFS）明显改善（P<0.05）。结论二尖瓣修复术,对于并发左心功能不全患者,有利于术后心功能的恢复和改善。     

Quantitative frame by frame analysis of regional contraction and lengthening on left ventricular cineangiograms: application to the study of normal left ventricles and left ventricles with mitral valve prolapse

This work attempted to study the segmental wall motion on left ventriculograms, in terms of segmental shortening, velocity of segmental shortening, and temporal sequences of various events in systole as well as in diastole. The ability of such a method to characterize patterns of normal regional wall motion and to detect mild abnormalities such as isolated asynchronisms, was tested on two groups of patients. Group I included 25 patients presenting evidence of a normal left ventricle (LV) after left heart catheterization. Group II consisted of 21 patients suffering from an isolated pure idiopathic mitral valve prolapse (MVP), with no mitral insufficiency and with an unaffected global LV function. In all patients left ventriculography was filmed in the right anterior oblique view at a rate of 50 frames/s. For each patient a cycle was chosen, distant from any premature beat, with acceptably contrasted outlines, and a quantitative frame by frame study of the motion of 10 segments was performed using a semiautomated method derived from the Stanford method. In the control group (Group I), analysis of the segmental motion by means of this method demonstrates a mild nonuniformity of the normal wall motion. This is principally marked by a stronger and faster contraction in anterolateral segments (segments 7, 8, 9) and by a shorter duration of the contraction in this region. In contrast the MVP group (Group II), exhibited a frank asynergy of the anterolateral region occurring from end systole to early diastole.(ABSTRACT TRUNCATED AT 250 WORDS)     

Echocardiographic predictors of left ventricular outflow tract obstruction and systolic anterior motion of the mitral valve after mitral valve reconstruction for myxomatous valve disease

OBJECTIVE

To determine predictors of systolic anterior motion and left ventricular outflow tract obstruction (SAM/LVOTO) after mitral valve repair (MVRep) in patients with myxomatous mitral valve disease.

BACKGROUND

Mechanisms for the development of SAM/LVOTO after MVRep have been described; however, predictors of this complication have not been explored. We hypothesize that pre-MVRep transesophageal echocardiography (TEE) can predict postrepair SAM/LVOTO.

METHODS

Using TEE, the lengths of the coapted anterior (AL) and posterior (PL) leaflets and the distance from the coaptation point to the septum (C-Sept) were measured before and after MVRep in 33 patients, including 11 who developed SAM/LVOTO (Group 1) and 22 who did not (Group 2).

RESULTS

Group 1 patients had smaller AL/PL ratios (0.99 vs. 1.95, p < 0.0001) and C-Sept distances (2.53 vs. 3.01 cm, p = 0.012) prior to MVRep than those in Group 2. Resolution of SAM/LVOTO was associated with increases in AL/PL ratio and C-Sept distance. This reflects a more anterior position of the coaptation point in those who developed SAM/LVOTO.

CONCLUSIONS

These data suggest that TEE analysis of the mitral apparatus can identify patients likely to develop SAM/LVOTO after MVRep for myxomatous valve disease. The findings are consistent with the concept that SAM of mitral leaflets is due to anterior malposition of slack mitral leaflet portions into the LVOT. The position of the coaptation point of the mitral leaflets is dynamic and a potential target and end point for surgical designs to prevent SAM/LVOTO post MVRep.     

二尖瓣置换术并发左心室破裂7例分析

目的探讨二尖瓣置换术并发左心室破裂的易发因素,并总结手术经验。方法对1473例行二尖;瓣膜置换术的患者中并发左心室破裂的病例资料进行回顾性分析。有7例并发左心室破裂．发生率0．47（7／1473）,其中4例在鱼精蛋白中和肝素时发生过敏反应出现左心室破裂：2例在停止体外循环后血压突：升高而发生左心室破裂;1例术后8h在监护室内出现心包纵隔引流管引流突然增多．血压迅速下降。考虑：心室破裂并紧急床边开胸探查,发现左心室后壁破裂。发生左心室破裂后紧急建立体外循环．探查左心室破位置及局部病变情况,采取心内、心外联合修补,术后严密监测。结果7例左心室破裂的患者中,死亡5例,抢救成功2例,病死率71．4％（5／7）,成功率28．6％（2／7）。结论二尖瓣置换术术前对左心室破裂高危因素积极预防,手术操作得当,避免机械性损伤,一旦发生左心室破裂及时建立体外循环,停跳心脏,心内、心外联一修补,严密监测,保持血流动力学稳定至关重要。     

Isolated non-obstructive accessory mitral valve tissue in an adult mimicking ruptured chordae

Accessory mitral valve tissue is commonly associated with other congenital heart diseases and is usually detected in children causing left ventricular outflow tract obstruction. We present an adult patient with isolated non-obstructive accessory mitral valve tissue that was mimicking ruptured chordae of the mitral valve. Accessory mitral valve tissue in adults is very rare and can mimick various causes of left ventricular outflow tract obstruction. This patient represents the first case in literature wherein an unobstructive accessory mitral valve tissue simulated a ruptured chordae. This case illustrates that in patients with suspected mitral valve chordae rupture without any mitral regurgitation, this diagnosis should be considered, which can have therapeutic implications.     

Mitral valve aneurysm revealed by cross-sectional echocardiography in a patient with mitral valve prolapse

We report a cerebral ischemia in a 23-year-old woman, in whom a mitral valve aneurysm with thrombotic masses was diagnosed by cross-sectional echocardiography. A prolapsing mitral leaflet was also visualized.     

组织多普勒对缘对缘二尖瓣成形术后左心室舒张功能评价

目的:EMVP技术将脱垂的二尖瓣叶相对应的前后叶缝合起来,形成一个双孔二尖瓣,从而改变了二尖瓣的解剖形态,本文旨在研究组织多普勒在缘对缘二尖瓣成形术后左心室舒张功能的作用.方法:30例二尖瓣关闭不全患者根据成形方式被分成A、B组,A组:15例二尖瓣后叶脱垂患者接受后叶楔形切除;B组:15例前叶或双叶脱垂患者接受缘对缘二尖瓣成形术,所有患者均同时接受二尖瓣环成形.术前及术后1周,运用彩色多普勒、组织多普勒测定患者左心室舒张功能指标,包括:术前和术后E峰与A峰血流速比值(E/A),舒张期E峰血流速度与舒张早期二尖瓣环的最大运动速度的比值(E/Em), 舒张早期二尖瓣环的最大运动速度与舒张晚期二尖瓣环的最大运动速度的比值(Em/Am).结果:A组与B组成形术后二尖瓣口面积及二尖瓣反流程度均明显减小,E/A、E/Em及Em/Am在手术前后组间及组内之间均无明显变化.结论:组织多普勒能较好客观地评价缘对缘二尖瓣成形后左心室舒张功能,缘对缘二尖瓣成形技术效果良好,对左心室的舒张功能无明显影响.     

Left ventricular outflow tract obstruction after mitral valve repair treated with alcohol septal ablation

Left ventricular outflow tract (LVOT) obstruction due to systolic anterior motion of the mitral valve (SAM) occurs in 4–5% of patients after mitral valve repair. If conservative treatment is unsuccessful, reoperation is indicated. Treatment options include repeated mitral valve repair or valve replacement and septal myectomy if hypertrophy is present. We report a case of a patient with pre‐operatively undiagnosed hypertrophic cardiomyopathy who suffered from progressive dyspnea post‐operation due to severe LVOT obstruction with SAM. This case was successfully treated with catheter‐based alcohol septal ablation. © 2013 Wiley Periodicals, Inc.