Muscular Subaortic Stenosis: The Initial Left Ventricular Inflow Tract Pressure as Evidence of Outflow Tract Obstruction

Two types of intraventricular pressure differences within the left ventricle of man are described. The first is encountered in cases of muscular (or fibrous) subaortic stenosis, in which the outflow tract pressure distal to the stenosis (and proximal to the aortic valve) is low, whereas all pressures recorded in the left ventricle proximal to the stenosis, including that just inside the mitral valve (the initial inflow tract pressure) are high.The second type of intraventricular pressure difference may be recorded in patients without muscular subaortic stenosis when a heart catheter is advanced to the left ventricular wall in such a manner that it becomes imbedded or entrapped by cardiac muscle in systole. Such an entrapped catheter records a high intraventricular pressure that is believed to reflect intramyocardial tissue pressure, which normally exceeds intracavitary pressure. In such cases the initial inflow tract pressure is not high and is precisely equal to the outflow tract systolic pressure, i.e. both are recording intracavity pressure. This type of intramyocardial to intracavitary pressure difference may also be encountered in the left ventricle of dogs.The recent suggestion that intraventricular pressure differences in the left ventricle of cases of muscular subaortic stenosis are due to catheter entrapment by cardiac muscle is refuted by using the initial inflow tract pressure as the means of differentiation between the two types of intraventricular pressure differences outlined.     

Varix of the heart causing outflow tract obstruction.

We report a case about a neonate who died of severe subaortic stenosis due to a giant vascular dilation of the left ventricular outflow tract. We emphasize the fatal result of this benign lesion and make differential diagnosis with haemangiomas and valvular blood cysts.     

Sudden unexpected death in children with congenital heart disease.

Of 18,000 children with organic heart disease evaluated at The Hospital for Sick Children, Toronto between 1940 and 1971, 33 died suddenly and unexpectedly between 1 and 21 years of age. Nine had discrete obstruction of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract and five had muscular narrowing of the left ventricular outflow tract. Pulmonary vascular disease caused seven sudden deaths, and arrhythmias (usually due to atrioventricular block) caused seven more. Of the five other children who died suddenly three had transposition of the great arteries, one had a complex cyanotic heart defect and one had an anomalous course of the left coronary artery, which originated from the right sinus of Valsalva. With earlier investigation of aortic stenosis, earlier closure of ventricular septal defect to avoid pulmonary vascular disease, better design of artificial pacemakers and better investigation of patients with angina, many of these deaths will be avoided in the future.     

应用同种带瓣管道重建右室流出道的危险因素分析

目的：评价采用同种带瓣管道行右室流出道重建术的临床效果,探讨影响手术效果及临床预后的因素。方法：回顾2002年11月至2010年11月期间应用同种带瓣管道行右室流出道重建患者的临床资料,分析患者手术前后的一般信息、血流动力学表现与临床预后的关系。结果：行右室流出道重建术后49例痊愈出院,5例死亡,存活率90．7％,死亡率9-3％。手术前后比较右室流出道内径较术前明显增加,右室一左室收缩压比值、右室-肺动脉压差较术前明显降低,三尖瓣反流、肺动脉瓣反流较术前加重,肺动脉瓣狭窄较术前减轻。统计分析表明患者死亡的危险因素有术后右室平均压、术后肺动脉-主动脉收缩压比值、术后二尖瓣反流。术后心胸比、术后肺动脉收缩压、术后肺动脉一主动脉收缩压比值、术后三尖瓣反流可能和术后患者ICU时间延长有关。McGoon指数、术后心胸比、术后肺动脉收缩压、术后右室平均压、术后肺动脉一主动脉收缩压比值、合并动脉导管未闭、术后三尖瓣反流可能和术后患者呼吸机时间延长有关。结论：复杂先天性心脏病患者采用同种带瓣管道重建右室流出道可以取得较满意的临床效果,术后流出道梗阻矫正满意,可以防止肺动脉返流导致的心脏损害。     

Undiagnosed hypertrophic obstructive cardiomyopathy during transcatheter aortic valve replacement: a case report

Background

Transcatheter aortic valve replacement is indicated for severe symptomatic aortic stenosis in patients who have a very high or prohibitive surgical risk as assessed pre-procedurally by the Society of Thoracic Surgery Risk Score, EuroSCORE (II), frailty testing, and other predictors. When combined with another left ventricular outflow tract obstruction, careful consideration must be taken prior to proceeding with transcatheter aortic valve replacement because an additional masked left ventricular outflow tract pathology can lead to challenging hemodynamics in the peri-deployment phase, as reported in this case.

Case presentation

A 56-year-old Caucasian man with multiple comorbidities and severe aortic stenosis underwent transcatheter aortic valve replacement under monitored anesthesia care. During the deployment phase, he developed dyspnea that progressed to pulmonary edema requiring emergent conversion to general anesthesia, orotracheal intubation, acute respiratory distress syndrome-type ventilation, and vasopressor medications. Intraoperative transesophageal echocardiography was performed and hypertrophic obstructive cardiomyopathy with systolic anterior motion of the mitral valve was discovered as an underlying pathology, undetected on preoperative imaging. After treatment with beta blockers, fluid resuscitation, and alpha-1 agonists, he stabilized and was eventually discharged from our hospital without any lasting sequelae.

Conclusions

Patients with aortic stenosis most often develop symmetric hypertrophy; however, a small subset has asymmetric septal hypertrophy leading to left ventricular outflow tract obstruction. In cases of severe aortic stenosis, however, evidence of left ventricular outflow tract obstruction via both symptoms and echocardiographic findings may be minimized due to extremely high afterload on the left ventricle. Diagnosing a left ventricular outflow tract obstruction as the cause of hemodynamic instability during transcatheter aortic valve replacement, in the absence of abnormal findings on echocardiogram preoperatively, requires a high index of clinical suspicion. The management of acute onset left ventricular outflow tract obstruction intraoperatively consists primarily of medical therapy, including rate control, adequate volume resuscitation, and avoidance of inotropes. With persistently elevated gradients, interventional treatments may be considered.

     

改良Nikaidoh手术治疗合并肺动脉瓣狭窄的右室双出口

目的：总结改良Nikaidoh手术治疗右心室双出口（DORV）患者的临床经验,以提高手术疗效。方法：2例先天性心脏病右心室双出口伴肺动脉瓣狭窄行改良Nikaidoh手术,游离主动脉根部及冠状动脉,重建左心室流出道,以带单瓣牛心包片补片重建肺动脉及右心室流出道。结果：术后患者紫绀消失,复查心脏彩超仅有轻度肺动脉瓣关闭不全,未发现左、右心室流出道梗阻,康复出院。结论：采用改良Nikaidoh手术治疗伴肺动脉瓣狭窄的右室双出口,术后可获得良好的血流动力学效果,早期临床结果满意。     

Insights from in-vitro flow visualization into the mechanism of systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy under steady flow conditions.

Hypertrophic obstructive cardiomyopathy is a heart disease characterized by a thickened interventricular septum which narrows the left ventricular outflow tract, and by systolic anterior motion (SAM) of the mitral valve which can contact the septum and create dynamic subaortic obstruction. The most common explanation for SAM has been the Venturi mechanism which postulates that septal hypertrophy, by narrowing the outflow tract, produces high velocities and thus low pressure between the mitral valve and the septum, causing the valve leaflets to move anteriorly. This hypothesis, however, fails to explain why SAM often begins early in systole, when outflow tract velocities are low or negligible or why it may occur in the absence of septal hypertrophy. The goal of this study was therefore to investigate an alternative hypothesis in which structural abnormalities of the papillary muscles act as a primary cause of SAM by altering valve restraint and thereby changing the geometry of the closed mitral apparatus and its relationship to the surrounding flow field. In order to test this hypothesis, an in vitro model of the left ventricle which included an explanted human mitral valve with intact chords and papillary muscle apparatus was constructed. Flow visualization was used to observe the ventricular flow field and the mitral valve geometry. Displacing the papillary muscles anteriorly and closer to each other, as observed clinically in patients with cardiomyopathy and obstruction produced SAM in the absence of septal hypertrophy. Flow could be seen impacting on the upstream (posterior) surface of the leaflets; such flow is capable of producing form drag forces which can initiate and maintain SAM.(ABSTRACT TRUNCATED AT 250 WORDS)     

Characteristics of hypotension elicited by electrical stimulation of the lateralhypothalamusin anaesthetized dogs.

Electrical stimulation of the lateral hypothalamus near the paraventricular nucleus hypothalami, resulted in : hypotension, light bradycardia, decrease of the left ventricular systolic pressure without increase of the left ventricular end-diastolic pressure or left ventricular output, femoral vasodilatation and occasionally renal vasodilatation. The hypotensive reactions were potentiated by baroreceptor deafferentation. They were not blocked by anticholinergic, antihistaminic or antidopaminergic agents.     

改良Nikaidoh 手术治疗合并肺动脉瓣狭窄的右室双出口

目的:总结改良Nikaidoh手术治疗右心室双出口(DORV)患者的临床经验,以提高手术疗效。方法:2例先天性心脏病右心室双出口伴肺动脉瓣狭窄行改良Nikaidoh手术,游离主动脉根部及冠状动脉,重建左心室流出道,以带单瓣牛心包片补片重建肺动脉及右心室流出道。结果:术后患者紫绀消失,复查心脏彩超仅有轻度肺动脉瓣关闭不全,未发现左、右心室流出道梗阻,康复出院。结论:采用改良Nikaidoh手术治疗伴肺动脉瓣狭窄的右室双出口,术后可获得良好的血流动力学效果,早期临床结果满意。     

Systolic pressure gradients between the wall of the left ventricle, the left ventricular chamber, and the aorta during positive inotropic states: implications for left ventricular efficiency

To study systolic pressure gradients developed between the left ventricular wall, its chamber, and the aortic root, in one group of dogs left ventricle ventral wall intramyocardial pressure, left ventricular outflow tract pressure, and aorta pressure were compared with aortic flow as well as left ventricular dimension changes during control conditions as well as during positive intropic states induced by isoproterenol, stellate ganglion stimulation, and noradrenaline. In another group of dogs systolic pressures in the ventral wall of the left ventricle, the main portion of the left ventricular chamber, and the aorta were compared with aortic flow during similar interventions, before and after the administration of phentolamine. Pressure gradients between the wall of the left ventricle and the outflow tract of the left ventricle were minimal during control states, but during the three positive inotropic states were increased significantly. In contrast, pressure gradients between the outflow tract of the left ventricle and the aortic root were insignificant during positive inotropic states; those between the wall and main portion of the chamber were only significantly different during left stellate ganglion stimulation. The data derived from these experiments indicate that useful peak power output of the left ventricle (systolic aortic pressure X flow) is unchanged following isoproterenol infusion, but is increased by stellate ganglion stimulation and noradrenaline. The useful peak power output index (an index of left ventricular efficiency derived by dividing useful peak power output by peak intramyocardial pressure) was reduced more by isoproterenol than the other two interventions.(ABSTRACT TRUNCATED AT 250 WORDS)     

New insights into the assessment of the prosthetic valve performance in the presence of subaortic stenosis through a fluid-structure interaction model

The aim of this study was to contribute to improving the accuracy of clinical assessments of valve performance in situations involving the concomitant presence of a prosthetic valve and subaortic stenosis (SAS). Physiological flow in a two-dimensional model for a bileaflet mechanical heart valve was investigated numerically in terms of the fluid-structure interactions. The fluid dynamics in a model with SAS of the left ventricle outflow tract were compared with those given by a healthy model. The results show that in the model with SAS, one leaflet did not close during the observed systolic phase, whereas the other one showed similar behaviour to that of the leaflet in the healthy model. In addition, the main flow did not occur along the central axis and a deviated jet was set up between leaflets, contrary to what occurred in the model without SAS. Current clinical diagnostic indices, which are mainly based on the central jet flow velocities, are therefore unsuitable for use in this pathological situation and should be used with great caution.     

Morphology of aortic arch obstruction with patent ductus arteriosus

Thirty-one hearts with aortic arch obstruction and patent ductus arteriosus were examined with special reference to associated cardiac anomalies. Six presented with complete interruption of the aortic arch, four with atretic isthmus, twelve with coarctation, and three with tubular hypoplasia. Associated cardiac anomalies were divided into two main groups: (1) septal defect with left-to-right shunt, and (2) left ventricular inflow and/or outflow obstruction. A high incidence (9/19=47.4%) of ventriculo-infundibular malalignment type of ventricular septal defect with subaortic stenosis was observed. Associated cardiac lesions that reduce blood flow in the aortic arch during fetal life may be responsible for poor development of this structure.     

The Hemodynamic Response of the Right Ventricle Following Acute and Chronic Partial Obstruction of the Main Pulmonary Artery in Dogs

In eight adult dogs the main pulmonary artery was constricted to elevate the right ventricular peak systolic pressure to 50% of the peak aortic pressure at rest. The response of the right ventricle was assessed immediately, at 30 minutes and at six months. The right ventricle responded to acute systolic loading by complete compensation. After 30 minutes there was a reduction in the right ventricular outflow tract resistance. The cardiac output, heart rate and aortic pressure were maintained. The right ventricular systolic ejection period, end-diastolic pressure, peak pressure time, mean systolic pressure, right ventricular—main pulmonary artery mean systolic gradient, right ventricular work index, systolic work and outflow tract resistance were all increased.The right ventricle in the dog was shown to have an immediate capacity to compensate for systolic loading and retains this capacity for long periods of time. The ability to increase work is accomplished by adaptations in right ventricular physiology which increase right ventricular mean systolic pressures and prolong the right ventricular ejection period.     

应用同种带瓣管道重建右室流出道的危险因素分析

目的:评价采用同种带瓣管道行右室流出道重建术的临床效果,探讨影响手术效果及临床预后的因素。方法:回顾2002年11月至2010年11月期间应用同种带瓣管道行右室流出道重建患者的临床资料,分析患者手术前后的一般信息、血流动力学表现与临床预后的关系。结果:行右室流出道重建术后49例痊愈出院,5例死亡,存活率90.7%,死亡率9.3%。手术前后比较右室流出道内径较术前明显增加,右室-左室收缩压比值、右室-肺动脉压差较术前明显降低,三尖瓣反流、肺动脉瓣反流较术前加重,肺动脉瓣狭窄较术前减轻。统计分析表明患者死亡的危险因素有术后右室平均压、术后肺动脉-主动脉收缩压比值、术后二尖瓣反流。术后心胸比、术后肺动脉收缩压、术后肺动脉-主动脉收缩压比值、术后三尖瓣反流可能和术后患者ICU时间延长有关。McGoon指数、术后心胸比、术后肺动脉收缩压、术后右室平均压、术后肺动脉-主动脉收缩压比值、合并动脉导管未闭、术后三尖瓣反流可能和术后患者呼吸机时间延长有关。结论:复杂先天性心脏病患者采用同种带瓣管道重建右室流出道可以取得较满意的临床效果,术后流出道梗阻矫正满意,可以防止肺动脉返流导致的心脏损害。     

Coronary flow reserve in hypertrophic cardiomyopathy: relation with microvascular dysfunction and pathophysiological characteristics

Background. The decrease in coronary flow reserve (CFR) in hypertrophic cardiomyopathy (HCM) predisposes to myocardial ischaemia, systolic dysfunction and cardiac death. In this study we investigate to which extent haemodynamic, echocardiographic, and histological parameters contribute to the reduction of CFR. Methods. In ten HCM patients (mean age 44±14 years) and eight heart transplant (HTX) patients (mean age 51±6 years) CFR was calculated in the left anterior descending coronary artery. In all subjects haemodynamic, echocardiographic and histological parameters were assessed. The relationship between these variables and CFR was determined using linear regression analysis. Results. CFR was reduced in HCM compared with HTX patients (1.6±0.7 vs. 2.7±0.8, p<0.01). An increase in septal thickness (p<0.005), indexed left ventricular (LV) mass (p<0.005), LV end-diastolic pressure (p<0.001), LV outflow tract gradient (p<0.05) and a decrease in arteriolar lumen size (p<0.05) were all related to a reduction in CFR. Conclusion: In HCM patients haemodynamic (LV end-diastolic pressure, LV outflow tract gradient), echocardiographic (indexed LV mass) and histological (% luminal area of the arterioles) changes are responsible for a decrease in CFR. (Neth Heart J 2007;15:209-15.)     

Coexistence of atrioventricular nodal reentrant tachycardia and idiopathic left ventricular outflow-tract tachycardia

Double tachycardia is a relatively rare condition. We describe a 21 year old woman with history of frequent palpitations. In one of these episodes, she had wide complex tachycardia with right bundle branch and inferior axis morphology. A typical atrioventricular nodal tachycardia was induced during electrophysiologic study, aimed at induction of clinically documented tachycardia. Initially no ventricular tachycardia was inducible. After successful ablation of slow pathway, a wide complex tachycardia was induced by programmed stimulation from right ventricular outflow tract. Mapping localized the focus of tachycardia in left ventricular outflow tract and successfully ablated via retrograde aortic approach. During 7 month's follow-up, she has been symptom free with no recurrence. This work describes successful ablation of rare combination of typical atrioventricular nodal tachycardia and left ventricular outflow tract tachycardia in the same patient during one session.     

心脏收缩时间间期与冠状动脉狭窄程度或范围的相关性研究

心脏收缩时间间期(STI)通过单笔心电图机叠加记录ECG、心音图和颈动脉图后测得。对83例患者进行了STI均值与选择性冠状动脉造影(CAG)及左室造影的相关研究。患者除陈旧性心肌梗塞及室壁瘤外,根据冠状动脉(CA)狭窄程度而分组。STI随CA狭窄程度及范围的增加而显著异常,提示心功能随心肌缺血程度加重而进行性恶化。左室射血分数(LVEF)的降低也支持这一点。CA狭窄程度、范围与射血前时间/左室射血时间(PEP/LVET)呈正相关。在决定左室功能的异常方面,PEP/LVET和LVEF的临床意义完全相同。提示STI在评价冠心病心功能上是有用的。排除影响STI的某些因素后,PEP/LVET≥0.38可作为判断冠心病左心功能减退的标志。但轻度CA狭窄患者的STI可正常,这可能与静息时尚有足够的CA贮备力有关。     

The effect of regular physical activity on the left ventricle systolic function in patients with chronic coronary artery disease

The purpose of this study was to assess the influence of aerobic training on the left ventricular (LV) systolic function. Thirty patients with stable coronary artery disease, who had participated in the conducted 3-month physical training, were retrospectively divided into 2 cohorts. While patients in the cohort I (n=14) had continued training individually for 12 months, patients in the cohort II (n=16) had stopped training after finishing the conducted program. Rest and stress dobutamine/atropine echocardiography was performed in all patients before the training program and 1 year later. The peak systolic velocities of mitral annulus (Sa) were assessed by tissue Doppler imaging for individual LV walls. In addition, to determine global LV systolic longitudinal function, the four-site mean systolic velocity was calculated (Sa glob). According to the blood supply, left ventricular walls were divided into 5 groups: A- walls supplied by nonstenotic artery; B- walls supplied by coronary artery with stenosis ≤50 %; C- walls supplied by coronary artery with stenosis 51-70 %; D- walls with stenosis of supplying artery 71-99 %; and E- walls with totally occluded supplying artery. In global systolic function, the follow-up values of Sa glob in cohort I were improved by 0.23±0.36 as compared with baseline values at rest, and by 1.26±0.65 cm/s at the maximal load, while the values of Sa glob in cohort II were diminished by 0.53±0.22 (p=NS), and by 1.25±0.45 cm/s (p<0.05), respectively. Concerning the resting regional function, the only significant difference between cohorts in follow-up changes was found in walls E: 0.37±0.60 versus -1.76±0.40 cm/s (p<0.05). At the maximal load, the significant difference was found only in walls A (0.16±0.84 versus -2.67±0.87 cm/s; p<0.05). Patients with regular 12-month physical activity improved their global left ventricle systolic function mainly due to improvement of contractility in walls supplied by a totally occluded coronary artery.     

Normalisation of the ECG in a patient with right bundle branch block by cardiac pacing

Right ventricular apical pacing (RVA) appears to have potential deleterious effects on myocardial systolic and diastolic left ventricular function, especially in patients with intact AV conduction. Therefore, new pacing sites in the right ventricle are being explored to overcome these detrimental effects. Alternative pacing sites in the right ventricle are the right ventricular outflow tract (RVOT) and the right ventricular septum (RVS). In this case report, we demonstrate an exceptional form of ventricular fusion, namely normalisation of the QRS complex in a patient with pre-existing right bundle branch block by RVS pacing. To our knowledge, this is the first report in the literature where right ventricular pacing could restore a complete RBBB to a normal QRS complex by stimulating distally from the anatomical position of the RBBB, due to fusion between artificial right ventricular stimulation and intrinsic conduction over the left bundle of the specific His-Purkinje system.     

Conditional deletion of focal adhesion kinase leads to defects in ventricular septation and outflow tract alignment

To examine a role for focal adhesion kinase (FAK) in cardiac morphogenesis, we generated a line of mice with a conditional deletion of FAK in nkx2-5-expressing cells (herein termed FAKnk mice). FAKnk mice died shortly after birth, likely resulting from a profound subaortic ventricular septal defect and associated malalignment of the outflow tract. Additional less penetrant phenotypes included persistent truncus arteriosus and thickened valve leaflets. Thus, conditional inactivation of FAK in nkx2-5-expressing cells leads to the most common congenital heart defect that is also a subset of abnormalities associated with tetralogy of Fallot and the DiGeorge syndrome. No significant differences in proliferation or apoptosis between control and FAKnk hearts were observed. However, decreased myocardialization was observed for the conal ridges of the proximal outflow tract in FAKnk hearts. Interestingly, chemotaxis was significantly attenuated in isolated FAK-null cardiomyocytes in comparison to genetic controls, and these effects were concomitant with reduced tyrosine phosphorylation of Crk-associated substrate (CAS). Thus, it is possible that ventricular septation and appropriate outflow tract alignment is dependent, at least in part, upon FAK-dependent CAS activation and subsequent induction of polarized myocyte movement into the conal ridges. Future studies will be necessary to determine the precise contributions of the additional nkx2-5-derived lineages to the phenotypes observed.