Left Ventricular Outflow Obstruction Research Articles

Impact of Effective Management Strategies on Patients With the Most Extreme Phenotypic Expression of Hypertrophic Cardiomyopathy

Advances in treatment options for hypertrophic cardiomyopathy (HC) have proven effective in many patients for promoting favorable long-term outcomes. Whether this expectation is similar for patients with the most extreme expression of massive left ventricular (LV) hypertrophy, a particularly aggressive form of the disease is unresolved. Of 1,766 consecutive HC patients presenting to Tufts HC Institute (2004 to 2015), 92 were identified with extreme LV wall thickness (30 to 48 mm), and compared with 1,674 HC patients with less marked hypertrophy (13 to 29 mm). Follow-up assessment was over 5.3 ± 3.4 years. Patients with massive LV hypertrophy (n = 92) had higher sudden death event rates (3.0%/year) than did patients with lesser hypertrophy (0.8%/year; p <0.001). In 16 of the 92 patients (17%), potentially lethal ventricular tachyarrhythmia were successfully aborted by primary prevention implantable cardioverter defibrillator (ICD) therapy at 30 ± 13 years (n = 11), or by resuscitated cardiac arrest with external defibrillation (n = 5) and later by secondary prevention interventions (n = 3); no patient experienced arrhythmic sudden death. Aborted sudden death events (3.0%/year) exceeded HC-related mortality by 7-fold (n = 2; 0.4%/year; p <0.001). European Society of Cardiology risk score would have failed to identify 60% of patients with arrhythmic sudden death events, leaving them exposed to sudden death without ICDs. In addition, 35 patients required surgical myectomy for progressive heart failure due to LV outflow obstruction (improved to NYHA I/II in 30). Eighty-eight (96%) of the 92 patients have survived to age 38 ± 14 years (23% ≥ 50 years). All-cause mortality did not differ from an age and gender-matched general population (p = 0.62). In conclusion, in this referral-based population, patients with the most extreme expression of HC are at increased arrhythmic sudden death risk reliably prevented with prophylactic ICDs. Progressive heart failure secondary to outflow obstruction was reversible with surgical myectomy. Despite extreme phenotypic expression, with contemporary treatment interventions young HC patients have an opportunity to achieve extended survival with good quality of life.

Upregulated Autophagy in Calcific Aortic Valve Stenosis Confers Protection of Valvular Interstitial Cells.

Autophagy serves as a cell survival mechanism which becomes dysregulated under pathological conditions and aging. Aortic valve thickening and calcification causing left ventricular outflow obstruction is known as calcific aortic valve stenosis (CAVS). CAVS is a chronic and progressive disease which increases in incidence and severity with age. Currently, no medical treatment exists for CAVS, and the role of autophagy in the disease remains largely unexplored. To further understand the role of autophagy in the progression of CAVS, we analyzed expression of key autophagy genes in healthy, thickened, and calcified valve tissue from 55 patients, and compared them with nine patients without significant CAVS, undergoing surgery for aortic regurgitation (AR). This revealed a upregulation in autophagy exclusively in the calcified tissue of CAVS patients. This difference in autophagy between CAVS and AR was explored by LC3 lipidation in valvular interstitial cells (VICs), revealing an upregulation in autophagic flux in CAVS patients. Inhibition of autophagy by bafilomycin-A1 led to a decrease in VIC survival. Finally, treatment of VICs with high phosphate led to an increase in autophagic activity. In conclusion, our data suggests that autophagy is upregulated in the calcified tissue of CAVS, serving as a compensatory and pro-survival mechanism.

Left atrial remodeling postseptal myectomy for severe obstructive hypertrophic cardiomyopathy: Analysis by two-dimensional speckle-tracking echocardiography.

Septal myectomy relieves left ventricular outflow obstruction (LVOTO) and is associated with excellent long-term outcomes. LVOTO is associated with diastolic dysfunction and increased left atrial (LA) size. We sought to investigate the changes in LA volumes and function postmyectomy and the association between these changes with clinical outcomes postmyectomy. Sixty-six hypertrophic cardiomyopathy patients undergoing myectomy were retrospectively studied. Preprocedural and 6- to 18-month postmyectomy follow-up transthoracic echocardiographic images were obtained. LA volumes and strain were assessed by two-dimensional speckle-tracking echocardiography. Left atrial volumes, that is, indexed maximal, minimal, and pre-A volumes reduced postmyectomy, yet remained increased compared to controls (105.6±34.5mL vs 84.9±26.7mL, 45.2±25.7mL vs 35.4±22.6mL, 70.1±31.4mL vs 35.4±22.6mL, respectively, P<0.05). The total emptying index did not improve postmyectomy and remained lower than controls (58.6±12.4 vs 59.9±12.8, P=NS) whereas atrial contraction improved, yet did not normalize (active emptying index 36.1±14.9 vs 41.1±16.2, P<0.05). The conduit volume remained reduced postmyectomy (18.6±13.3mL vs 16.6±15.1mL, P=NS). LA strain also did not improve postmyectomy (26.8±7.3 vs 28.5±8.8, P=NS). A multivariable logistic regression identified preprocedural E/e' ratio and indexed maximal LA volume, as independent predictors for LA volume reduction ≥20% postmyectomy. During a mean follow-up of 4.9±2.3 years postmyectomy, 24.2% of the patients developed atrial fibrillation and <5% of patients were severely symptomatic. We found no associations between LA volumes/function and atrial fibrillation or symptoms postmyectomy. Postmyectomy LA volumes decreased, and the contractile function improved. There was no association between LA volumes/function and clinical outcomes postmyectomy. Notably, the LA remained enlarged (though to a lesser degree) with reduced strain and emptying fraction, suggesting possible atrial myopathy.

Multilevel left ventricular outflow obstructive lesions in an adult Nigerian female

Congenital left ventricular outflow obstruction malformations are a group of left-sided stenotic lesions that can occur individually or in various combinations at subvalvular, valvular, and supravalvular levels. We present the case of a 23-year-old female who presented with multilevel obstructions not having been diagnosed in childhood. She subsequently underwent surgical correction.

Guz lewej komory serca u nastolatka

Asymptomatic 17-years-old boy with a suspicion of the heart tumor was admitted to the Cardiology Department for further investigation. Due to the murmur over the heart echocardiography was performed revealing a bright mass near the left ventricular apex. He was in a good physical condition, no symptoms like chest pain, shortness of breath, syncope, palpitations were reported. On physical examination diminished heart sounds and systolic murmur 2-3/6 in the Levin’s scale over the heart were found. Laboratory data including troponin T, CK, CK-MB, NSE, urea acid, marker were within normal limits. On ECG abnormalities of the repolarization were recorded (ST segment depression and T waves inversion in the II, III, aVF leads). Transthoracic echocardiogram revealed a 4.7 x 5.8 cm tumor within the postero-lateral wall of the left ventricle, without left ventricular inflow nor outflow obstruction. Based on cardiac magnetic resonance hemangioma supplied from the right coronary artery was suspected. Cardiac catheterization was performed, the left ventricular mass with supply from vascular network of the right coronary artery was confirmed. No abnormalities of the central nervous system in magnetic resonance were found. Full-body positron emission tomography showed cardiac mass without any evidence of extra-cardiac fluorodeoxyglucose-avid disease. Cardiac hemangioma was recognized. Pharmacological treatment with propranolol was introduced without any impact on the tumor size during 6 months follow-up.

Abstract 16627: Predictors of Response to Exercise Training in Patients With Hypertrophic Cardiomyopathy

Introduction: RESET-HCM was a randomized clinical trial of 16 weeks of moderate-intensity exercise training (ExT) in patients with hypertrophic cardiomyopathy (HCM). Compared with usual activity (UA), ExT resulted in a modest but significant 6% improvement in mean peak oxygen consumption (VO 2peak ), the best measure of cardiorespiratory fitness (CRF). Studies of heart failure populations have shown that ≥15% improvement in VO 2peak can be achieved with ExT. Hypothesis: We hypothesize that intervention-, disease-, and patient-specific factors differentially influence VO 2peak response to ExT. Methods: Data from 113 subjects were analyzed. Those who achieved ≥ 15% improvement in VO 2peak in response to ExT were categorized as high responders (HR), while low responders (LR) were those who did not. Baseline characteristics and activity log data were compared between HR and LR. Logistic regression investigated associations between VO 2peak response and these measures. Results: Twenty-two subjects were HR (16/57 [28.1%] in the ExT group vs. 6/56 [10.7%] in the UA group, p=0.02). HR had fewer comorbidities such as hypertension (9.1% vs 26.4%, p=0.03) and diabetes mellitus (0% vs 7.7%, p=0.01). Ejection fraction (EF) was lower among HR than LR (68.6% vs 71%, p=0.02), but other measures of cardiac remodeling, including hypertrophy and fibrosis burden, resting left ventricular outflow obstruction, and diastolic function did not differ. HR had lower baseline VO 2peak (18.4 vs 22.6 mL/kg/min, p=0.01). Although adherence to the frequency element of exercise prescriptions decreased over time in both groups, HR were 25% more adherent (p=0.02), exercising an average of 1 more day per week (3.8 vs 2.8, p=0.02). On multivariate analysis, baseline VO 2peak (OR: 0.79; 95% CI: 0.68-0.92) and EF (OR: 0.85; 95% CI: 0.74-0.98) were independent predictors of ≥15% improvement in VO 2peak after adjustment for age and sex. Higher exercise frequency (OR: 1.37; 95% CI: 0.97-1.95) was also an important factor. Conclusions: Higher exercise frequency, lower CRF at baseline, and lower EF predicted a greater magnitude of response to moderate intensity ExT in HCM patients. Future studies will be necessary to make the response to ExT more uniform across a heterogeneous group of HCM patients.

RF34 PARADOXICAL ARTIFICIAL CORDS TECHNIQUE TO TREAT SAM IN HOCM

Introduction: The Systolic Anterior Motion (SAM) of mitral valve (MV) associated with the asymmetric hypertrophy of left ventricular septum are the two components responsible for left ventricular outflow obstruction (LVOTO) in Hypertrophic Obstructive Cardiomyopathy (HOCM). The transaortic left ventricular septal myomectomy has been proposed as the gold standard surgical procedure for symptomatic patients with severe HOCM since the late 1960 s. Objective: We have previously described one technique to prevent SAM post MV repair, the so-called “paradoxical artificial cords to prevent SAM after MV repair”. The aim of this article is to demonstrate the efficacy of this technique also to treat SAM in HOCM patients, in case of concomitant moderate septal myectomy. Methods: The subjects of the study are 5 patients with both SAM and LVOTO. All of them have been surgically treated, performing moderate transaortic septal myomectomy and correction of SAM by paradoxical artificial cords and ring annuloplasty. Results: The postoperative course of all patients was regular. No one of them developed the usual complications of this surgery (Ventricular Septal Defect [VSD] or Pacemaker [PM] implantation). Transthoracic Echocardiography TTE has demonstrated good result of the early postoperative interventricular gradient. The MV function was preserved and free of SAM. Conclusion: This technique could be considered as a valid treatment for SAM in patients with HOCM and LVOTO, if associated with moderate septal myectomy. The combination of these two surgical procedures is particularly important to avoid complications, especially VSD, in patients with a relatively thin interventricular septum, even in presence of hypertrophy.

OC82 TRANSAORTIC MITRAL VALVE REPAIR FOR OBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY: THE POLICLINICO DI MONZA EXPERIENCE

Background and Aim: Abnormalities of mitral valve (MV) apparatus are known to contribute to left ventricular (LV) outflow obstruction in hypertrophic cardiomyopathy (HCM). Purpose of this study is to report our experience with MV repair in patients with obstructive HCM undergoing surgical septal myectomy. Methods: From July 2013 to March 2018, 316 consecutive patients with obstructive HCM underwent surgical septal myectomy. Of these patients, 298 (94.3%) had associated MV repair and only 18 (5.7%) had MV replacement due to severe leaflets calcification and intrinsic MV disease. Age ranged from 15 to 85 years, mean 54 ± 15. Each patient had an outflow gradient ≥50 mmHg at rest or with physiologic provocation and disabling symptoms unresponsive to medical therapy. Results: Transaortic papillary muscle mobilization and cutting of fibrotic and retracted secondary MV chordae was performed, in association with septal myectomy, in all study patients. Plication of a redundant anterior MV leaflet was performed in 119 (37%) patients, and of the posterior leaflet in 29 (9%). Two patients died during hospitalization (0.6%), 8 patients (2.8%) had a residual postoperative resting gradient ≥30 mmHg, 6 (1.9%) patients had persistent important functional limitation (NYHA III), and 9 (2.8%) residual moderate-to-severe MV regurgitation, at the first postoperative evaluation. Conclusions: In our large HCM study cohort, transaortic extensive and systematic repair of the MV apparatus, combined with septal myectomy, was associated with a particularly low perioperative mortality, and abolition of resting LV outflow obstruction, MV regurgitation and heart failure symptoms in the great majority of patients.

Left ventricular outflow obstruction predicts increase in systolic pressure gradients and blood residence time after transcatheter mitral valve replacement

Left ventricular outflow tract (LVOT) obstruction is a relatively common consequence of transcatheter mitral valve replacement (TMVR). Although LVOT obstruction is associated with heart failure and adverse remodelling, its effects upon left ventricular hemodynamics remain poorly characterised. This study uses validated computational models to identify the LVOT obstruction degree that causes significant changes in ventricular hemodynamics after TMVR. Seven TMVR patients underwent personalised flow simulations based on pre-procedural imaging data. Different virtual valve configurations were simulated in each case, for a total of 32 simulations, and the resulting obstruction degree was correlated with pressure gradients and flow residence times. These simulations identified a threshold LVOT obstruction degree of 35%, beyond which significant deterioration of systolic function was observed. The mean increase from baseline (pre-TMVR) in the peak systolic pressure gradient rose from 5.7% to 30.1% above this threshold value. The average blood volume staying inside the ventricle for more than two cycles also increased from 4.4% to 57.5% for obstruction degrees above 35%, while the flow entering and leaving the ventricle within one cycle decreased by 13.9%. These results demonstrate the unique ability of modelling to predict the hemodynamic consequences of TMVR and to assist in the clinical decision-making process.

Analytical validation of platelet microparticle quantification in cats.

Cardiogenic embolism (CE) in cats is a devastating condition primarily associated with hypertrophic cardiomyopathy (HCM). Hypercoagulability may pose a risk for thrombus formation; however, no single test can predict CE development. Platelet microparticles (PMPs) released from platelet membranes are associated with thrombosis in humans. The aims were to validate flow cytometric PMP quantification in cats analytically and, in a pilot study, evaluate the procoagulant annexin V (AnV) positive PMP concentration in healthy cats and cats with asymptomatic HCM. With CD61 as a platelet marker, CD61+ AnV+ PMPs (0.3-1.0μm) were quantified in citrated whole blood (WB) and platelet-poor plasma (PPP) using flow cytometry. Analyses were performed in 6 healthy cats and 5 cats with asymptomatic HCM. The coefficient of variation (CV) for duplicate (intra-assay) and parallel (inter-assay) analyses were calculated. PMP concentrations were quantified with acceptable intra-assay CV for WB (CD61+ /AnV- ; 2.4%, 0.2%-8.4% (median, range), CD61+ /AnV+ ; 3.8%, 0.1%-12.5%) and PPP (CD61+ /AnV- ; 5.0%, 0.7%-12.8%, CD61+ /AnV+ ; 7.4%, 0.5%-15.3%), and acceptable inter-assay CV for WB in 10/11 cats (CD61+ /AnV- ; 6.2%, 1.4%-13.3%, CD61+ /AnV+ ; 6.4%, 0.7%-17.2%), but unacceptable for PPP (CD61+ /AnV- ; 15.6%, 5.8%-42.7%, CD61+ /AnV+ ; 27.8%, 8.4%-77.1%). For WB PMP concentrations, the pilot data demonstrated no differences between healthy cats and cats with asymptomatic HCM (4/5 with left ventricular outflow obstruction) for either the CD61+ /AnV- or the CD61+ /AnV+ PMPs. Only WB PMP concentrations could be quantified reliably in cats in a clinical setting. PMP concentrations did not differ between healthy and asymptomatic HCM cats in this pilot study.

Transesophageal Echocardiography for Ross-Konno Procedure

AbstractRoss procedure involves replacing diseased aortic valve with pulmonary autograft and placing a pulmonary/aortic homograft between the right ventricle and pulmonary artery. In case of small aortic annulus in pediatric patients or in patients with multilevel left ventricular outflow obstruction, aortic annulus enlargement might be required concurrently. Konno type of aortic ventriculoplasty is most commonly done. The American Heart Association/American College of Cardiology (AHA/ACC) has classified transesophageal echocardiography (TEE) as class I indication for congenital heart disease

Anaesthetic management of a patient with hypertrophic obstructive cardiomyopathy undergoing laparoscopic cholesytectomy

Hypertrophic cardiomyopathy is a genetic disorder characterized by asymmetric left ventricular hypertrophy with or without left ventricular outflow tract obstruction. From asymptomatic state to sudden death comprise its varied clinical range. Owing to complex patho-physiology and very high risk of cardiac events, its peri-operative management demands meticulous maintenance of hemodynamic parameters. Prevention and prompt correction of factors such as hypovolemia, tachycardia, and increased contractility that can exacerbate the left ventricular outflow obstruction, arrhythmias and myocardial ischemia are essential for improving patient outcome. Laparoscopic surgeries in these patients can further amplify anaesthetic concerns and add to the challenges. A 58 year female was scheduled for surgery having suffered frequent complications of cholelithiasis. As there is paucity of reports evaluating laparoscopic surgery, we report this patient diagnosed with hypertrophic obstructive cardiomyopathy, who received general anaesthesia to undergo laparoscopic cholecystectomy successfully without suffering complications during surgery and hospital stay.

Clinical Spectrum and Management Implications of Left Ventricular Outflow Obstruction With Mild Ventricular Septal Thickness in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HC) has historically been characterized as a disease of substantial left ventricular hypertrophy, often associated with dynamic left ventricular outflow tract obstruction. However, we have recently encountered patients with subaortic obstruction and only minimal basal septal thickness, raising important management implications. Thereby, we sought to characterize the natural history and treatment strategies for this under-recognized subgroup of HC patients with dynamic obstruction. Of 1,591 consecutive patients with a HC diagnosis, 113 (7%) were identified with outflow obstruction due to elongated mitral valve leaflets producing systolic anterior motion and septal contact typical of HC (gradient, 84 ± 29 mm Hg at rest or with exercise), with maximal ventricular septal thickness ≤15 mm, including 14 patients with only 10 to 12 mm. In addition to the mechanism of outflow obstruction, other evidence supporting a HC diagnosis prominently included: positive HC family history and/or pathogenic sarcomere mutation, arrhythmic sudden death event, typical histopathology of septal muscle, and characteristic mitral valve and papillary muscle anomalies. Over 3.8 ± 3.5 years, 41 patients (36%) developed severe heart failure including 36 who have undergone myectomy associated with reconstruction of the outflow tract and mitral valve apparatus, resulting in relief of gradient without iatrogenic ventricular septal defect or mitral valve replacement. Postoperatively, all 36 patients have survived with symptom relief to New York Heart Association classes I/II. In conclusion, these observations expand the HC clinical profile and phenotype to include an under-appreciated subgroup in which disease expression includes outflow obstruction due primarily to an elongated mitral valve, associated with only minimal (or normal) ventricular septal thickness. Such HC patients can develop marked functional limitation amenable to an operative strategy that effectively relieved symptoms due to outflow obstruction, but without mitral valve replacement.

Obstructive Hypertrophic Cardiomyopathy with Concomitant Mitral Regurgitation Treated with a Septal Myectomy and MV Repair: A Case Report

Abstract Case presentation Hypertrophic cardiomyopathy (HCM) is the most common and very heterogeneous genetic cardiac disease with a different clinical presentation and prognosis. The overall prevalence of the disease is estimated between 0.05-0.2% of the population. Left ventricular outflow obstruction at rest is present in about 20% of patients. Most of the patients have a normal life expectancy, however high risk patients might develop heart failure, atrial fibrillation, ventricular arrhythmias and sudden cardiac death. We present the case of 47-year-old Caucasian man who was hospitalized at our clinic with a history of chest pain and shortness of breath on physical activity in the last six months, which caused significant limitations of his life quality. Hypertrophic obstructive cardiomyopathy was diagnosed in 2011, when the patient was put on therapy with beta blocker. Transthoracic echocardiography revealed normal systolic function, presence of systolic anterior mitral valve motion (SAM) with moderate mitral regurgitation (MR). There was a significant concentric left ventricular hypertrophy predominantly located in the ventricular septum. The intraventricular gradient at rest was 77.8 mmHg. MRI of the heart confirmed significant LV hypertrophy with regions of fibrosis at the septum. The patient shortness of breath worsened progressively in the last month (NYHA III) despite optimized medical treatment with maximal beta blocker dose. Surgical approach with septal myectomy was performed with mitral valve repair. There were no operative complications, with excellent postoperative recovery and complete symptoms resolution. Control Doppler echocardiograms revealed LVOT rest gradient reduction to 34 mmHg. The good operative results were still present 9 months after the intervention. Our case confirmed that septal myectomy with MV repair is an excellent treatment approach in young patient with obstructive hypertrophic cardiomyopathy and mitral valve involvement refractory to medical treatment.

Dynamic Left Ventricular Outflow Obstruction and Systolic Anterior Motion of the Mitral Valve Complicating Surgical Aortic Valve Replacement

Dynamic Left Ventricular Outflow Obstruction and Systolic Anterior Motion of the Mitral Valve Complicating Surgical Aortic Valve Replacement

Perioperative and Anesthetic Considerations in Interrupted Aortic Arch.

Interrupted aortic arch (IAA) is defined as the loss of luminal continuity between the ascending and descending aorta and is classified based on the anatomic level of interruption. IAA is associated with a number of intracardiac anomalies with the most common being patent ductus arteriosus, ventricular septal defect, and left ventricular outflow obstruction. There is also a strong association between type B interruption and 22q11 deletion syndrome. The perioperative management of the neonate with IAA begins in the intensive care unit with optimization of end-organ perfusion and function. Survival depends on the prompt initiation of prostaglandin E1 in order to maintain ductal patency, careful management of the patient's ratio of pulmonary to systemic blood flow (Qp:Qs), and a thorough understanding of the physiologic implications of the surgical plan, type of interruption, and associated syndromes and anomalies. This review will focus on the anatomy, physiology, and perioperative anesthetic management considerations specific to the management of IAA.

A review of the Nunn modified single patch technique for atrioventricular septal defect repair.

Atrioventricular septal defect (AVSD) is a common congenital cardiac surgical problem. Over the years, younger and smaller infants are having operations for this condition before irreversible cardio pulmonary changes occur. Traditionally a single or two patch techniques have been used to repair this defect. However, in the past two decades an innovative method of modified single patch technique popularized by Dr. Graham Nunn has gained worldwide popularity. This review discusses the origin, surgical principles, technique and outcomes of this method, popularly known as Nunn or Australian technique. Research comparing the modified single patch technique to classic single and double patch techniques has shown good preservation of atrioventricular valve function, no residual ventricular septal defect (VSDs), low incidence of left ventricular outflow obstruction, preserved conduction, easy reproducibility, and improved perioperative and long-term mortality.

Effect of Spironolactone on Myocardial Fibrosis and Other Clinical Variables in Patients with Hypertrophic Cardiomyopathy

BackgroundMyocardial fibrosis has proved to be an important marker and determinant in the pathogenesis of hypertrophic cardiomyopathy. In particular, scar formation, if substantial, can promote ventricular tachyarrhythmias or progressive heart failure in the absence of left ventricular outflow obstruction. Therefore, an intervention to mitigate myocardial fibrosis would be potentially advantageous to hypertrophic cardiomyopathy patients. MethodsEligible hypertrophic cardiomyopathy patients were randomized 1:1 in a prospective double-blind fashion to spironolactone 50 mg or placebo to be taken over a 12-month period. The primary endpoint was the effect of mineralocorticoid receptor blockade on serum markers of collagen synthesis and degradation. A number of other functional and morphologic variables and biomarkers comprised secondary exploratory measures. ResultsFifty-three hypertrophic cardiomyopathypatients (41 ± 13 years old; 72% men) were randomized; demographic and clinical variable were well matched at baseline. Absolute change between baseline and 12 months did not differ between hypertrophic cardiomyopathy patients treated with spironolactone and those receiving placebo with respect to serum markers of collagen synthesis or degradation, fibrosis by late gadolinium enhancement on cardiac magnetic resonance imaging, or other clinical variables, including objective measure of functional capacity (peak VO2), New York Heart Association functional class, left ventricular wall thickness, mass and volume, and left atrial size, as well as assessment of diastolic function (P = .4-1.0). ConclusionsThese findings do not support the use of spironolactone in hypertrophic cardiomyopathy to improve left ventricular remodeling by mitigating myocardial fibrosis or altering clinical course.

Optimal reconstruction of left ventricular outflow tract obstruction before surgical myectomy in a case with hypertrophic obstructive cardiomyopathy

We present optimally reconstructed three-dimensional computed tomography images of left ventricular outflow obstruction, comprehensive left ventriculography, and comparable intraoperative transesophageal echocardiography, as well as serial operative pictures, to facilitate the understanding of live-heart anatomy of hypertrophic obstructive cardiomyopathy. As shown in this case, detailed morphological analysis around the left ventricular outflow tract using preoperative computed tomography would be feasible and useful. The present case highlights the importance of obtaining complete three-dimensional information present in the acquired computed tomography dataset because computed tomography is not entirely noninvasive or free of radiation exposure and contrast material.

Minimally invasive trans-mitral septal myectomy for diffuse-type hypertrophic obstructive cardiomyopathy.

Despite excellent long-term results reported for a trans-aortic septal myectomy for hypertrophic obstructive cardiomyopathy (HOCM), surgery for patients with diffuse hypertrophy is very challenging. In addition, a left ventricular outflow obstruction is often aggravated by an abnormal mitral valve and subvalvular apparatus. We performed video-assisted minimally invasive trans-mitral septal myectomy procedures in 3 patients with diffuse-type HOCM, who were highly symptomatic despite maximal medical therapy. Each had at least moderate mitral regurgitation (MR) due to systolic anterior motion (SAM). Using a right mini-thoracotomy, the anterior mitral leaflet was detached, through which an extended septal myectomy could easily be performed. Abnormal bridging chordae between the septum and papillary muscle (PM) were divided, then anterior mitral leaflet continuity was restored with direct closure or augmentation using a glutaraldehyde-treated autologous pericardium. A PM reorientation procedure was performed in 1 case in which both PMs were approximated and sutured onto the posterior ventricular wall. The postoperative course was uneventful in all patients, with marked improvement of symptoms in each. The peak ventricular outflow gradient decreased from 134 ± 40 to 23 ± 5mmHg with significantly diminished SAM, especially in the patient who underwent the PM reorientation procedure. During a mean follow-up period of 42 ± 14 months, no MR has been detected in any case. We believe that a minimally invasive trans-mitral septal myectomy is preferable for HOCM-patients with diffuse hypertrophy and mitral valve abnormality. Aggressive PM reorientation may also be useful for those with an abnormal PM orientation.