Surgery for hypertrophic cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetically determined cardiac disease characterised by otherwise unexplained myocardial hypertrophy of the left ventricle, and may result in left ventricular outflow tract obstruction. It is the most common cause of sudden cardiac death in young adults due to arrhythmias. Septal myectomy is a surgical treatment for HCM with moderate to severe outflow tract obstruction, and is indicated for patients with severe symptoms refractory to medical therapy. The surgical approach involves obtaining access to the interventricular septum via transaortic, transapical or transmitral approaches, and excising a portion of the hypertrophied myocardium to relieve the outflow tract obstruction. Large, contemporary series from centres experienced in septal myectomy patients have demonstrated a low early mortality of <2 %, excellent long-term survival that matches the general population, and durable relief of symptoms.     

Mammoth interatrial septal aneurysm in the ICE age

Background

Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon.

Case Presentation

A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest); and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient), but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death.

Conclusion

Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM).     

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.

     

Clinical Use of a New Mitral Disc Valve

A disc valve of new design was used successfully for the replacement of the mitral valve in patients with rheumatic mitral valve disease. This valve would appear to have the following advantages over the mitral ball valve prosthesis:• Lower left atrial pressure after replacement.• Elimination of the hazard of left ventricular outflow tract obstruction with mitral valve replacement.• Decreased incidence of thromboembolization.• Abolition of possibility of ventricular septal irritation.Despite the better outlook for this valve compared with the ball valve for mitral valve substitution, the mitral valve should always be repaired whenever feasible. Repair is possible in the majority of patients.     

Echocardiographic features of pigs with spontaneous hypertrophic cardiomyopathy

Ultrasonography is one of the most common, noninvasive techniques used for cardiovascular diagnosis because it provides reliable information and enhances patient safety. Two-dimensional (2-D) and M-mode echocardiography is conducted to assess the severity and distribution of myocardial hypertrophy. Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease that has variable manifestations because interactions between the many facets of systolic and diastolic dysfunction of the heart are complex. The objective of the study reported here was to characterized clinical HCM in pigs. A commercial Vingmed (CFM-800) 3.25 MHz transducer was used to perform 2-D and M-mode echocardiography. Experimental pigs (about 100 kg in body weight) were anesthetized and positioned in left lateral recumbency. Echocardiographic images (2-D) were acquired in parasternal short-axis and long-axis views. The 2-D images provided M-mode under direct anatomic visualization. The pigs were sacrificed for pathologic study after echocardiographic examination. In typical HCM cases (n = 8), the interventricular septum thickness increased, the left ventricular (LV) end-systolic and end-diastolic dimensions decreased, and the left atrial dimensions and the indexes of systolic function, such as ejection fraction and velocity of fiber shortening, increased. The LV outflow tract narrowed, particularly when gross upper septal hypertrophy was evident. Moreover, systolic cranial motion (SCM) of the septal leaflet of the mitral valve was observed. Doppler evidence of mitral regurgitation often was associated with SCM. The echocardiographic findings from pigs with HCM resembled those from humans. Thus, porcine HCM may serve as a spontaneous animal model for the study of HCM in humans.     

肥厚梗阻性心肌病的外科治疗

目的:总结肥厚室间隔切除术治疗肥厚梗阻性心肌病的手术效果,探讨外科治疗策略。方法:2002年3月至2010年10月,外科手术治疗33例肥厚梗阻性心肌病病人。其中男16例,女17例;年龄13～59岁,平均(42.7±13.6)岁;左室流出道压差(LVOTGP)70～120 mmHg(1 mmHg=0.133Kpa),平均(95.0±22.6)mmHg。其中合并二尖瓣关闭不全24例,主动脉瓣关闭不全7例,升主动脉增宽3例,冠心病2例。手术在全麻低温体外循环下完成,按常规经主动脉切口行室间隔心肌切除术,同期完成二尖瓣置换术(MVR)7例,二尖瓣成形术(MVP)7例,二尖瓣、主动脉瓣成形术(MVP+AVP)5例,二尖瓣、升主动脉成形术3例,二尖瓣、主动脉瓣成形、冠状动脉旁路移植术(MVP+AVP+CABG)2例。分析比较病人术前超声心动图(UCG),术中经食管心脏超声(TEE),以及术后1周、3月、6月、1年超声心动图结果。结果:手术死亡1例(3.0%,1/33例),主要死因为严重低心排综合症以及多脏器功能衰竭。二次开胸止血1例(3.0%,1/33例)。术中经食管心脏超声示所有病人二尖瓣前叶收缩期前向运动现象(SAM征)消失。存活病人手术效果良好,解剖狭窄解除,峰值压差降低,SAM现象基本消失。远期随访生存病人症状消失,生活质量明显改善,心功能I～II级,无远期死亡、并发症或再次手术。结论:外科治疗肥厚梗阻型心肌病具有良好的手术效果。了解病生理过程、术中仔细探察、手术切除彻底是手术成功的关键。     

Percutaneous septal ablation for left mid-ventricular obstructive hypertrophic cardiomyopathy: a case report

Background

Mid-ventricular obstructive hypertrophic cardiomyopathy (MVOHC) is a rare type of cardiomyopathy. The diagnosis is based on the hourglass appearance on the left ventriculogram and the presence of pressure gradient between apical and basal chamber of the ventriculum on the hemodynamic assessment.

Case presentation

The present case represents successful percutaneous treatment with septal ablation to patient with MVOHC associated with systolic anterior motion of the mitral valve and obstruction at both the mid-ventricular and outflow levels.

Conclusion

Alcohol septal ablation has been proposed as less invasive alternatives to surgery in patients with MVOHC.     

Finite element analysis of the mitral apparatus: annulus shape effect and chordal force distribution

This study presents a three-dimensional finite element model of the mitral apparatus using a hyperelastic transversely isotropic material model for the leaflets. The objectives of this study are to illustrate the effects of the annulus shape on the chordal force distribution and on the mitral valve response during systole, to investigate the role of the anterior secondary (strut) chordae and to study the influence of thickness of the leaflets on the leaflets stresses. Hence, analyses are conducted with a moving and fixed saddle shaped annulus and with and without anterior secondary chordae. We found that the tension in the secondary chordae represents 31% of the load carried by the papillary muscles. When removing the anterior secondary chordae, the tension in the primary anterior chordae is almost doubled, the displacement of the anterior leaflet toward the left atrium is also increased. The moving annulus configuration with an increasing annulus saddle height does not give significant changes in the chordal force distribution and in the leaflet stress compared to the fixed annulus. The results also show that the maximum principle stresses in the anterior leaflet are carried by the collagen fibers. The stresses calculated in the leaflets are very sensitive to the thickness employed.     

ACS,myocardial bridging,Tako-tsubo syndrome and mitral regurgitation

Isolated systolic compression of the mid portion of the left anterior descending artery (LAD) by a bridge of overlying cardiac muscle is an infrequent but well-recognised angiographic anomaly that is often considered harmless. The long-term prognosis appears to be excellent, but occasional reports of patients with angina pectoris, myocardial infarction and sudden death indicate that this is not always true. The prevalence of the anomaly in the normal population is unknown, but the incidence is low and ischaemic events are rare.Tako-tsubo-like left ventricular dysfunction syndrome (TTS) is characterised by ischaemia, anterior ST-segment elevation, no significant coronary artery disease and reversible ampulla-like left ventricular ballooning in postmenopausal females after emotional or physical stress. Dynamic left ventricular outflow tract (LVOT) obstruction is a rare but potentially fatal complication of acute anterior wall infarction.We present a patient with an acute coronary syndrome (ACS) with ST-segment elevation in the anterior leads, transient TTS and transient LVOT obstruction with systolic anterior motion (SAM) of the mitral valve and severe mitral regurgitation. This is the first report of myocardial bridging associated with TTS, and the first report of TTS associated with dynamic LVOT obstruction with SAM and mitral regurgitation.     

Dynamics of left ventricular wall and mitral valve mechanics--a model study

The relation between global left ventricular pumping characteristics and local cardiac muscle fiber mechanics is represented by a mathematical model of left ventricular mechanics in which the mitral valve papillary muscle system is incorporated. The wall of the left ventricle is simulated by a thick-walled cylinder. Transmural differences in fiber orientation are incorporated by changing the direction of material anisotropy across the wall. The cylinder is free to twist. The upper end of the cylinder is covered by a thin, flexible sheet, representing the base of the left ventricle. The mitral valve is incorporated in this sheet. The tips of the mitral leaflets are connected by chordae tendineae to the papillary muscles which are attached to the bottom of the cylinder. Canine cardiac cycles were simulated for various end-diastolic values of left ventricular volume (25-120 ml, control 60 ml), left atrial pressure (0-2.7 kPa, control 0.22 kPa) and aortic pressure (5-11 kPa, control 11 kPa). In this wide range of preload and afterload mechanical loading of the muscle fibers appeared to be distributed quite evenly (SD: +/- 5% of control value) over all muscular structures of the left ventricle, including the papillary muscles.     

A computational procedure for prediction of structural effects of edge-to-edge repair on mitral valve

Edge-to-edge technique is a surgical procedure for the correction of mitral valve leaflets prolapse by suturing the edge of the prolapsed leaflet to the free edge of the opposing one. Suture presence modifies valve mechanical behavior and orifice flow area in the diastolic phase, when the valve opens and blood flows into the ventricle. In the present work, in order to support identification of potentially critical conditions, a computational procedure is described to evaluate the effects of changing suture length and position in combination with valve size and shape. The procedure is based on finite element method analyses applied to a range of different mitral valves, investigating for each configuration the influence of repair on functional parameters, such as mitral valve orifice area and transvalvular pressure gradient, and on structural parameters, such as stress in the leaflets and stitch tension. This kind of prediction would ideally require a coupled fluid-structural analysis, where the interactions between blood flows and mitral apparatus deformation are simultaneously considered. In the present study, however, an alternative approach is proposed, in which results obtained by purely structural finite element analyses are elaborated and interpreted taking into account the Bernoulli type equations available in literature to describe blood flow through mitral orifice. In this way, the effects of each parameter in terms of orifice flow area, suture loads, and leaflets stresses can be expressed as functions of atrioventricular pressure gradient and then correlated to blood flow rate. Results obtained by using this procedure for different configurations are finally discussed.     

Defect in oxidative phosphorylation in LV papillary muscle mitochondria of patients undergoing mitral valve replacement

Mitochondria play a pivotal role in cellular metabolism, especially in energy production. Myocardial function depends on adenosine triphosphate (ATP) supplied by oxidation of several substrates. In the adult heart, this energy is obtained primarily from fatty acid oxidation through oxidative phosphorylation (OXPHOS). With this in view, we studied OXPHOS, Total-ATPase and cytochrome content in the mitochondria of the left ventricular (LV) papillary muscles in excised mitral valves of patients who underwent mitral valve replacement (MVR). The mitochondrial OXPHOS, cytochrome content and ATPase activity were studied in 70 patients (ranging from 22 to 40 years) operated on for mitral valve disease. Control study includes 25 normal mitral valves removed at necropsy from patients who died of extracardiac causes. In the presence of glutamate and succinate as substrates, the rate of mitochondrial oxygen consumption was significantly lower in LV papillary muscles of pathological mitral valves (P<0.001) by using with and without addition of ADP. The ADP/O ratio indices for glutamate and succinate were not significantly affected. Using glutamate as substrate, respiratory control index was significantly raised (P<0.05) as compared with control. A significant reduction of total cytochrome content and ATPase activity (P<0.001) was noted in LV papillary muscles of patients operated for mitral valve disease. Our results showed that OXPHOS, cytochromes 'a', 'b', 'c+c(1)' and ATP activity are significantly impaired in LV papillary muscles in patients with pathological mitral valve. Cardiac mitochondrial oxygen consumption is a very valuable tool to investigate the regulation of cardiac mitochondrial energy metabolism. There is increasing evidence that mitochondrial diseases, such as mitochondrial cardiomyopathy, valvular disease and some myopathies, can be responsive to treatment with metabolic intermediates such as coenzyme Q(10), thiamine, prednisone, and vitamin therapy.     

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.     

Left ventricular endocardial longitudinal and transverse changes during isovolumic contraction and relaxation: a challenge

Left ventricular (LV) longitudinal and transverse geometric changes during isovolumic contraction and relaxation are still controversial. This confusion is compounded by traditional definitions of these phases of the cardiac cycle. High-resolution sonomicrometry studies might clarify these issues. Crystals were implanted in six sheep at the LV apex, fibrous trigones, lateral and posterior mitral annulus, base of the aortic right coronary sinus, anterior and septal endocardial wall, papillary muscle tips, and edge of the anterior and posterior mitral leaflets. Changes in distances were time related to LV and aortic pressures and to mitral valve opening. At the beginning of isovolumic contraction, while the mitral valve was still open, the LV endocardial transverse diameter started to shorten while the endocardial longitudinal diameter increased. During isovolumic relaxation, while the mitral valve was closed, LV transverse diameter started to increase while the longitudinal diameter continued to decrease. These findings are inconsistent with the classic definitions of the phases of the cardiac cycle.     

Biventricular / left ventricular pacing in hypertrophic obstructive cardiomyopathy: an overview

Hypertrophic cardiomyopathy (HCM) is an autosomal dominant inherited genetic disease characterized by compensatory pathological left ventricle (LV) hypertrophy due to sarcomere dysfunction. In an important proportion of patients with HCM, the site and extent of cardiac hypertrophy results in severe obstruction to LV outflow tract (LVOT), contributing to disabling symptoms and increasing the risk of sudden cardiac death (SCD). In patients with progressive and/or refractory symptoms despite optimal pharmacological treatment, invasive therapies that diminish or abolish LVOT obstruction relieve heart failure-related symptoms, improve quality of life and could be associated with long-term survival similar to that observed in the general population. The gold standard in this respect is surgical septal myectomy, which might be supplementary associated with a reduction in SCD. Percutaneous techniques, particularly alcohol septal ablation (ASA) and more recently radiofrequency (RF) septal ablation, can achieve LVOT gradient reduction and symptomatic benefit in a large proportion of HOCM patients at the cost of a supposedly limited septal myocardial necrosis and a 10-20% risk of chronic atrioventricular block. After an initial period of enthusiasm, standard DDD pacing failed to show in randomized trials significant LVOT gradient reductions and objective improvement in exercise capacity. However, case reports and recent small pilot studies suggested that atrial synchronous LV or biventricular (biV) pacing significantly reduce LVOT obstruction and improve symptoms (acutely as well as long-term) in a large proportion of severely symptomatic HOCM patients not suitable to other gradient reduction therapies. Moreover, biV/LV pacing in HOCM seems to be associated with significant LV reverse remodelling.     

External approach to in vivo force measurement on mitral valve traction suture

BackgroundForce measurements on the mitral valve apparatus have been reported from in vivo and in vitro studies. Recent reparative techniques for ischemic mitral valve insufficiency call for papillary muscle relocation. This study describes a device to measure forces generated on traction sutures utilized for this purpose.MethodsThe transducer design was based on a modified caliper with strain gauges attached. Finite element computer simulation was employed to optimize the signal output. The system was designed to facilitate investigation of the effects of shortening GoreTex traction suture that was extended from near the fibrous trigones of the mitral valve through the papillary muscles. The suture was exteriorized out through the left ventricle in a porcine setup (n=11) and attached to the dedicated device for simultaneous papillary muscle relocation and traction suture force measurement.ResultsThe transducer demonstrated excellent signal strength, linearity, and durability. Peak force was seen at the onset of the systolic isovolumic contraction (p<0.001). Initial results indicated that this external approach can document force magnitudes comparable to previous internally measured forces in the mitral valve apparatus.ConclusionsIt has been proven feasible to measure forces in the mitral valve papillary muscle relocation sutures with an external device. The results from using this equipment will provide insight into the biomechanical requirements of relocation traction sutures and other devices utilized for papillary muscle relocation.     

In vitro assessment of a combined radiofrequency ablation and cryo-anchoring catheter for treatment of mitral valve prolapse

Percutaneous approaches to mitral valve repair are an attractive alternative to surgical repair or replacement. Radiofrequency ablation has the potential to approximate surgical leaflet resection by using resistive heating to reduce leaflet size, and cryogenic temperatures on a percutaneous catheter can potentially be used to reversibly adhere to moving mitral valve leaflets for reliable application of radiofrequency energy. We tested a combined cryo-anchoring and radiofrequency ablation catheter using excised porcine mitral valves placed in a left heart flow loop capable of reproducing physiologic pressure and flow waveforms. Transmitral flow and pressure were monitored during the cryo-anchoring procedure and compared to baseline flow conditions, and the extent of radiofrequency energy delivery to the mitral valve was assessed post-treatment. Long term durability of radiofrequency ablation treatment was assessed using statically treated leaflets placed in a stretch bioreactor for four weeks. Transmitral flow and pressure waveforms were largely unaltered during cryo-anchoring. Parameter fitting to mechanical data from leaflets treated with radiofrequency ablation and cryo-anchoring revealed significant mechanical differences from untreated leaflets, demonstrating successful ablation of mitral valves in a hemodynamic environment. Picrosirius red staining showed clear differences in morphology and collagen birefringence between treated and untreated leaflets. The durability study indicated that statically treated leaflets did not significantly change size or mechanics over four weeks. A cryo-anchoring and radiofrequency ablation catheter can adhere to and ablate mitral valve leaflets in a physiologic hemodynamic environment, providing a possible percutaneous alternative to surgical leaflet resection of mitral valve tissue.     

Unusual findings of asymmetrical septal hypertrophy associated with calcification of the interventricular septum: Case report

A patient with aortic regurgitation, stenosis, and calcification of the septum is reported. Results of echocardiography revealed asymmetrical septal hypertrophy without other features of idiopathic hypertrophic subaortic stenosis. There was no subaortic obstruction evident on cardiac catheterization and angiography. This case serves to emphasize that calcification of the interventricular septum is another possible cause of asymmetrical septal hypertrophy.