Dynamic LVOT Obstruction and Aortic Stenosis in the Same Patient: A Case of Challenging Doppler Hemodynamics

We present a patient with both dynamic left ventricular outflow tract obstruction and valvular aortic stenosis. The aortic valve was calcified, and velocities and gradients measured by continuous‐wave Doppler met standard criteria for severe aortic stenosis. The increased subvalvular velocities invalidated assumptions of the simplified Bernoulli equation; correction using the longer form of the Bernoulli equation suggested a lower but still significant gradient. The complex shape of the subvalvular spectral Doppler envelope indicated supranormal systolic function and dynamic left ventricular outflow obstruction. Left heart catheterization with an end‐hole catheter was required to determine the subvalvular and valvular components of the obstruction.     

The Usefulness of Transesophageal Echocardiography in the Surgical Management of Older Children with Subaortic Stenosis

Subaortic stenosis is a complex lesion that often presents in older children and adolescents. A clear depiction of the lesion is required for optimization of surgery. Due to the large size of these patients, is not always possible from surface echocardiography. Intraoperative multiplane echocardiography (MTEE) has been performed at our institute in older children for several different congenital heart lesions including many patients with subaortic stenosis. A retrospective analysis of our experience with MTEE in patients with subaortic stenosis was performed to assess its usefulness in the preoperative diagnosis and postoperative assessment of repair. Our results show that intraoperative MTEE was useful preoperatively by correcting or confirming suspected diagnosis, and giving additional details of the lesion in many patients. Postoperatively, MTEE was highly useful in the assessment of repair. We strongly recommend the use of intraoperative MTEE in older children and adolescents with subaortic stenosis.     

Left ventricular outflow obstruction at multiple levels: Occurrence in a single patient

Combined forms of left ventricular outflow obstruction are being recognized with increasing frequency. A patient who had coarctation of the aorta, valvular aortic stenosis, and fixed and dynamic subvalvular obstruction is described. The importance of recognizing multiple levels of obstruction in such patients is emphasized.     

An unusual cause of left ventricular outflow tract obstruction

Left ventricular outflow tract obstruction (LVOTO) has been reported with bio-prosthetic and mechanical mitral valves (MV), though it is more common with the former. The obstruction can be dynamic or fixed. We hereby report a case of fixed LVOTO following bio-prosthetic MV replacement (MVR).     

The use of MitraClip to prevent posttranscatheter aortic valve replacement left ventricular “suicide”

Patients with concomitant severe aortic stenosis (AS) and left ventricular outflow tract (LVOT) obstruction undergoing transcatheter aortic valve replacement (TAVR) are at risk for hemodynamic collapse due to a sudden decrease in afterload causing worsening LVOT obstruction. We present a case of an 88-year-old female with symptomatic, severe AS, and LVOT obstruction with systolic anterior motion (SAM) of the mitral leaflet in whom alcohol septal ablation was contraindicated secondary to a chronic total occlusion of the right coronary artery that filled retrograde via septal collaterals. MitraClip at the time of TAVR was successfully performed to treat SAM with subsequent stabilization of LVOT gradients despite treatment of the patient's AS. This novel approach may represent a feasible option to prevent hemodynamic complications after TAVR in patients with significant LVOT obstruction secondary to SAM and AS.     

Aortic and Mitral Valve Stenosis with Regurgitation: Not Due to Rheumatic Heart Disease

The patient is a 13‐year‐old Mexican–American immigrant who had no previous medical care. Upon arrival to the United States she was diagnosed with severe mitral valve stenosis and regurgitation. In addition she had severe aortic stenosis with mild to moderate aortic valve regurgitation, which was thought to be rheumatic heart disease. On the basis of the clinical and echocardiographic findings she was taken to the operating room for both mitral and aortic valve replacement. Her operation was complicated by the discovery that her aorta was completely calcified, as were her coronary arteries, mitral valve and aortic valve. She underwent aortic and mitral valve replacement as well as replacement of her aortic arch. Her coronary arteries were patch augmented and reimplanted into the newly created ascending aorta. Based on the pathologic examination of the surgical tissue a diagnosis of Gaucher disease was made. (Echocardiography 2011;28:E24‐E27)     

Proximal Aortic Dissection Complicated by Cardiac Tamponade

Cardiac tamponade is a well recognized complication of acute proximal aortic dissection and is almost uniformly fatal if not immediately diagnosed and surgically treated. Pericardiocentesis has an ill-defined and perhaps deleterious role in the management of this condition. Severe concentric left ventricular hypertrophy and intravascular volume depletion may further impede ventricular filling in a patient with cardiac tamponade. We describe the management of a patient who presented in a confused state with an acute proximal aortic dissection complicated by cardiac tamponade and associated with evidence of left ventricular outflow tract obstruction. In such a patient, aggressive fluid administration and emergency surgery should be the treatment of choice.     

肥厚梗阻性心肌病与二尖瓣病变及二尖瓣反流

目的:探讨左心室流出道梗阻对二尖瓣叶及二尖瓣反流的影响。方法:采用数字化超声心动图技术分析左心室流出道梗阻患者二尖瓣前叶形态结构及二尖瓣反流的特点。结果:68例左心室流出道梗阻患者,其中2例二尖瓣前叶赘生物形成合并穿孔,66例二尖瓣前叶近瓣缘部分轻度增厚。全部患者均有明显二尖瓣前叶收缩期前向运动(SAM)及二尖瓣偏心反流。结论:左心室流出道梗阻可引起二尖瓣前叶病变。左心室流出道梗阻患者二尖瓣前叶SAM可导致二尖瓣反流。     

Noninvasive Evaluation of Membranous Subaortic Stenosis: Complimentary Roles of Echocardiography and Computed Tomographic Angiography

A 29‐year‐old Ethiopian woman that was referred to the Wisconsin Heart Hospital for treatment of subaortic stenosis, diagnosed 4 years earlier, in Ethiopia, using transthoracic echocardiography. Preoperative evaluation included transesophageal echocardiography, which showed severe membranous subaortic stenosis with a mean outflow gradient of 70 mmHg. Cardiac computed tomographic angiography also demonstrated a subaortic membrane, and additionally showed normal epicardial coronary arteries. The patient underwent uneventful surgical resection of the subaortic membrane without undergoing cardiac catheterization. (Echocardiography 2010;27:E34‐E35)     

Isolated Left Ventricular Apical Hypoplasia with Right Ventricular Outflow Tract Obstruction: A Rare Combination

Isolated left ventricular (LV) apical hypoplasia is a unusual and recently recognized congenital cardiac anomaly. A 19‐year‐old man was found to have an abnormal ECG and cardiac murmur identified during a routine health check since joining work. His ECG revealed normal sinus rhythm, right‐axis deviation, poor R wave progression, and T wave abnormalities. On physical examination, a 2/6～3/6 systolic murmur was heard at the second intercostal space along the left sternal border. Subsequent echocardiography and cardiac magnetic resonance imaging confirmed the LV apical hypoplasia. Of note, we first found that LV apical hypoplasia was accompanied by RV outflow tract obstruction due to exaggerated rightward bulging of the basal‐anterior septum during systole. A close follow‐up was performed for the development of heart failure, pulmonary hypertension, and potentially tachyarrhythmia.     

A Case of Aortic Stenosis and Hypertrophic Cardiomyopathy

A patient with severe aortic valvular stenosis and coexisting obstructive hypertrophic cardiomyopathy, in whom diagnostic difficulties and management dilemmas arose, is presented. The complex issues involved in such a combination are discussed.     

The Syndrome of Degenerative Calcific Aortic Stenosis: Prevalence of Multiple Pathophysiologic Disorders in Association with Valvular Stenosis and Their Implications

Background: We hypothesized that degenerative calcific aortic stenosis (DCAS) is a syndrome influenced by factors beyond aortic valve stenosis (AS). The aim of this study was to assess how frequently DCAS is complicated by increased vascular load, systolic and/or diastolic left ventricular (LV) dysfunction, and comorbid disorders. Methods: In 215 consecutive patients > 60 years of age with severe and moderate AS, we analyzed systemic arterial compliance, global hemodynamic load, LV ejection fraction (EF), the presence of diastolic dysfunction, and other valvular or systemic disorders. Results: A total of 164 patients had severe AS and 51 had moderate AS. In patients with severe AS, the prevalence of increased vascular load was 42%; LV systolic and diastolic dysfunction was present in 27% and 42%; other valve diseases in 23%; and comorbid disorders in 82%. In the moderate AS group, abnormal vascular load was found in 52%; LV systolic and diastolic dysfunction was prevalent in 26% and 31%; other valve diseases in 17%; and comorbid disorders in 78% patients. More than half the patients in both groups had symptoms. In both severe and moderate AS groups, the prevalence of increased vascular load and systolic dysfunction was higher in the symptomatic group. Conclusion: Considerable number of patients with DCAS have abnormal vascular load, abnormal LV function, and significant coexisting disorders. These could influence the total pathophysiologic burden on the heart and symptom expression. Thus, DCAS should not be considered just as valvular stenosis, but a syndrome of DCAS because of the diagnostic, prognostic, and therapeutic implications of various factors associated with it.     

The Global Burden of Aortic Stenosis

Degenerative, calcific valvular aortic stenosis (AS), caused by an active process of atherosclerosis, calcification and ossification, is the most common cause of AS in industrialized nations. The prevalence of calcific AS is age-dependent, and thus is expected to increase due to demographic aging of the global population. It is well recognized that severe AS carries a poor prognosis if left untreated. Despite this recognition, many patients are inappropriately denied surgery because of perceived risk. This article will examine the etiology, prevalence, and current trends in the treatment of degenerative AS focusing on indications for surgical aortic valve replacement.     

重度主动脉瓣狭窄瓣膜置换术后左室舒张功能的超声评价

目的应用常规超声心动图及斑点追踪二维应变技术研究EF正常的单纯重度主动脉瓣狭窄患者,评价瓣膜置换术后左室重构与左室舒张功能改善的情况并进行相关性分析。方法20例单纯重度主动脉瓣狭窄患者于手术前、术后平均7．5个月行经胸超声心动图检查。比较术前与术后左室舒张末期容积指数（LVEDVI）、左室心肌质量指数（LVMI）、室壁相对厚度（RWT）、左室充盈压（E／E’m）、等容舒张期左室整体应变率（SRIVRT）、舒张早期左室整体应变率（SRE）及其变化的相关性。结果主动脉瓣置换术后LVEDVI、LVMI、LVMI／LVEDVI均较术前减低（P均〈0．05）,SRIVRT、SRE均较术前升高（P均〈0．05）;RWT、E／E’m较术前改变不显著（P均〉0.05）。SRIVRT与SRE正相关（r=0．403,P〈0．05）,术前的SRIVRT与E／Em’及LVMI／LVEDVI呈负相关（P均〈0．05）。结论EF正常的单纯主动脉瓣狭窄患者在主动脉瓣置换术后左室重构的主要变化为左室容量减小,左室壁心肌质量下降,虽然左室充盈压无明显下降,但SRIVRT和SRE在术后有所升高,可作为补充指标更敏感地反映左室整体舒张功能的变化。