scholarly journals CMR derived ventricular arterial coupling in hypertrophic cardiomyopathy

2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
A Kharabish ◽  
M Eid ◽  
W Elmozy ◽  
A Elguindy ◽  
M Yacoub
Keyword(s):  
Clinical Practice ◽  
Hypertrophic Cardiomyopathy ◽  
Systolic Pressure ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Systolic Volume ◽  
Funding Sources ◽  
Before And After ◽  
Surgical Myectomy

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Ventriculoarterial coupling (VAC) is an important surrogate measurement of global cardiovascular efficiency. However, up till recently, this measurement has been rarely used in clinical practice. This could be due to its invasive nature, and the relatively complex methods required for acquisition and interpretation. We here describe a noninvasive method of measuring VA coupling using cardiac magnetic resonance (CMR) in a cohort of hypertrophic cardiomyopathy (HCM). Methods   Cardiac MRI was performed in 61 patients with hypertrophic obstructive cardiomyopathy (HOCM) before and after undergoing extended surgical myectomy. Cardiac MRIs from 15 patients with non-obstructive HCM were also included for comparison. Hypertensive patients were excluded from this study. Vitals signs were recorded during each MRI study. Arterial end-systolic pressure (AESP) was determined non-invasively as previously described (0.9x brachial blood pressure). Left ventricular end-systolic pressure (LVESP) was calculated by adding the echo-derived peak systolic gradient across the left ventricular outflow tract to the arterial end-systolic pressure. Indexed stroke volume (SVI) in the ascending aorta as well as indexed end systolic volume (ESVI) were calculated from CMR. Indexed arterial (Ea) and ventricular (Ev) elastance were calculated as AESP/SVI and LVESP/ESVI. VA coupling was then calculated as Ea/Ev.  Results Compared to the baseline measurements, VAC increased significantly post myectomy (mean VAC pre-myectomy and post-myectomy 0.38 vs. versus 0.62 respectively, p= 0.00001). VAC in non-obstructive HCM patients (mean = 0.64) was statistically similar to that in post-myectomy (p = 0.82) and statistically different from pre-myectomy (p = 0.00016). The mean of LVOTO gradients post- and pre-myectomy were significantly different (p = 0.00001). Significant correlation of the delta-VAC with the delta gradient in the LVOTO was found (r= 0.35, p = 0.005).  Conclusion Non-invasive assessment of VA coupling using CMR is feasible and could be a useful tool in the evaluation of patients with various cardiovascular disorders. Further studies with larger numbers of patients are required to establish the utility of this method in clinical practice.

Abstract 17402: Single Nuclei RNA-sequencing of Human Hypertrophic Cardiomyopathy Myectomy Samples Reveals Common Novel Mechanisms of Pathogenesis and Potential Therapeutic Targets Regardless of Genotype

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Amy Larson ◽  
Hassan Rastegar ◽  
Gordon S Huggins ◽  
Ethan J Rowin ◽  
Martin S Maron ◽  
...  
Keyword(s):  
Gene Expression ◽  
Hypertrophic Cardiomyopathy ◽  
Rna Sequencing ◽  
Alternative Treatment ◽  
Expression Patterns ◽  
Therapeutic Targets ◽  
Treatment Strategies ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Surgical Myectomy

Introduction: Hypertrophic cardiomyopathy (HCM) is a common inherited cardiovascular disease, often resulting in left ventricular outflow tract obstruction, relieved by surgical myectomy. Current treatments are largely palliative and do not target the root causes. Understanding the molecular drivers of the disease could lead to alternative treatment strategies through identification of novel therapeutic targets. Methods: We performed single nuclei RNA-sequencing (snRNA-seq) on thousands of nuclei from 9 patient myectomy samples and septal tissue from 4 unused donor hearts selected randomly without regard to genotype to identify the cell populations and determine the gene expression patterns in individual cells. Each sample was processed individually using Seurat v3 for quality control and normalization. Next, all 13 samples were integrated into a combined dataset for clustering and differential gene expression analysis to identify markers specific to each cluster and to assign cell identities. Results: Our results revealed several clusters of cardiomyocytes with differences in sarcomeric and metabolic gene expression. Several fibroblast populations were also observed. Numerous genes were differentially expressed between the HCM and normal samples. For example, RARRES1 expression was observed in many of the fibroblast populations in the normal samples, but was absent in the HCM samples. RARRES1 is involved in regulating fatty acid metabolism and autophagy, both of which are altered in HCM. Additionally, expression of PLA2G2A was absent in the HCM samples but was present in almost every cell type in the normal controls. PLA2G2A is involved in suppression of RTK mediated hypertrophic signaling, impacts lipid signaling, and has tumor suppressor properties. Thus, both RARRES1 and PLA2G2A may represent novel targets in HCM. Conclusions: This approach reveals novel potential therapeutic targets within common final HCM pathological pathways independent of genotype that have the potential to guide development of alternative treatment strategies. Further analysis of larger datasets using this approach will likely identify even more common pathway targets and identify additional common mechanisms in the pathogenesis of obstructive HCM.

Abstract 13729: Surgical Myectomy Results in Significantly Improved Long-term Event Rate in Hypertrophic Cardiomyopathy Patients With Severe Left Ventricular Outflow Tract Obstruction

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Milind Y Desai ◽  
Nicholas G Smedira ◽  
Aditya Bhonsale ◽  
Nitesh Ainani ◽  
Maran THamilarasan ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Long Term Outcomes ◽  
Free Survival ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Background: In hypertrophic cardiomyopathy (HCM) with severe left ventricular outflow tract obstruction (LVOTO), surgical myectomy (SM) performed for relief of intractable symptoms is safe and associated with excellent long-term symptom-free survival. In such patients, we sought to determine if SM also results in improvement of long-term outcomes. Methods: We studied 1530 HCM patients with severe LVOTO (50±13 years, 63% men) that were evaluated at our center [excluding <18 years of age, LV ejection fraction (LVEF) <50%, LVOT gradient <30 mm Hg). Clinical, echocardiographic and surgical data were recorded. A composite endpoint of death and/or implantable defibrillator (ICD) discharge was recorded. Results: Hypertension, coronary artery disease (CAD), family history of HCM and syncope were present in 41%, 15%, 17% and 18%, while 73% patients were in Functional Class (FC) ≥ II. Mean LVEF, basal septal thickness, LVOT gradient (resting or provocable) and indexed left atrial dimensions were 62±5%, 2.2±1 cm, 101±39 mm Hg, 2.2±0.4 cm/m2. During 8±6 years of follow-up, 990 (65%) patients underwent SM (of which 65% were isolated SM), while 540 (35%) did not. 94 (6%) patients had alcohol septal ablation (66 in the non myectomy group), while 18% developed atrial fibrillation (AF), and 18% had ICD. There were 169 (11%) events (151 deaths), with 0% 30-day mortality in the SM group. On stepwise multivariable Cox Proportional Hazard analysis, increasing age (Hazard Ratio or HR 1.22 [1.06-1.40]), CAD (HR 1.57 [1.06-2.33]), worsening FC (HR 1.34 [1.05-1.71]) and AF (HR 1.73 [1.23-2.43]) predicted higher events, while SM as a time-dependent covariate (HR 0.58 [0.41-0.81]) was associated with improved event-free survival (all p<0.01). Kaplan-Meier curve showing impact of SM on outcomes is shown in Figure. Conclusion: In HCM patients with severe LVOTO, SM is associated with significant improvement in long-term outcomes when compared to watchful waiting.

scholarly journals Alcohol septal ablation in hypertrophic cardiomyopathy

2018 ◽  
Vol 2018 (3) ◽  
Author(s):  
Juan José Santos Mateo ◽  
Juan R Gimeno
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Interventricular Septum ◽  
Ventricular Outflow Tract ◽  
Clinical Results ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Alcohol septal ablation (ASA) has become an alternative to surgical myectomy in obstructive hypertrophic cardiomyopathy since it was first introduced in 1994 by Sigwart. The procedure alleviates symptoms by producing a limited infarction of the upper interventricular septum, resulting in a decrease in left ventricular outflow tract (LVOT) gradient. The technique has been improved over time and the results are comparable with those of myectomy. Initial concerns about long-term outcomes have been largely resolved. In this review, we discuss indications, technical aspects, clinical results and patient selection to ASA.

scholarly journals Hypertrophic Cardiomyopathy Mimicking Acute Anterior Myocardial Infarction Associated with Sudden Cardiac Death

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Y. Daralammouri ◽  
M. El Garhy ◽  
K. Same ◽  
B. Lauer
Keyword(s):  
Myocardial Infarction ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Valsalva Maneuver ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Anterior Myocardial Infarction ◽  
Left Ventricular Outflow

Hypertrophic cardiomyopathy is the most common genetic disease of the heart. We report a rare case of hypertrophic obstructive cardiomyopathy mimicking an acute anterior myocardial infarction associated with sudden cardiac death. The patient presented with acute ST elevation myocardial infarction and significant elevation of cardiac enzymes. Cardiac catheterization showed some atherosclerotic coronary artery disease, without significant stenosis. Echocardiography showed left ventricular hypertrophy with a left ventricular outflow tract obstruction; the pressure gradient at rest was 20 mmHg and became severe with the Valsalva maneuver (100 mmHg). There was no family history of sudden cardiac death. Six days later, the patient suffered a syncope on his way to magnetic resonance imaging. He was successfully resuscitated by ventricular fibrillation.

scholarly journals Alcohol Septal Ablation for the Treatment of Hypertrophic Obstructive Cardiomyopathy

2011 ◽  
Vol 9 (2) ◽  
pp. 108 ◽  
Author(s):  
Constantinos O’Mahony ◽  
Saidi A Mohiddin ◽  
Charles Knight ◽  
◽  
◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Ventricular Hypertrophy ◽  
Left Ventricular Outflow Tract ◽  
Comprehensive Evaluation ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Alcohol Septal Ablation ◽  
Refractory Symptoms ◽  
Left Ventricular Outflow

Hypertrophic cardiomyopathy (HCM) is an inherited myocardial disorder characterised by left ventricular hypertrophy. A subgroup of patients develops limiting symptoms in association with left ventricular outflow tract obstruction (LVOTO). Current international guidelines recommend that symptomatic patients are initially treated by alleviating exacerbating factors and negatively inotropic medication. Drug-refractory symptoms require a comprehensive evaluation of the mechanism of LVOTO and review by a multidisciplinary team to consider the relative merits of myectomy, alcohol septal ablation (ASA) and pacing. This article provides a brief overview of HCM and the pathophysiology of LVOTO, and reviews the use of ASA in patients with drug-refractory symptoms secondary to LVOTO.

scholarly journals The results of alcoholic septal ablation in the treatment of hypertrophic cardiomyopathy

2020 ◽  
pp. 44-49
Author(s):  
B.M. Todurov ◽  
◽  
G.I. Kovtun ◽  
A.V. Khokhlov ◽  
O.V. Pantazi ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow Tract ◽  
Young Patients ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Alcohol Septal Ablation ◽  
Left Ventricular Outflow

Hypertrophic obstructive cardiomyopathy іs a relatively common condition and one of the most common causes of sudden cardiac death in young age. One of the options for the surgical treatment of this pathology is septal myoectomy, which has been the gold standard for decades. However, despite this, surgical treatment is intended for young patients with a low risk of postoperative complications, while patients with concomitant diseases and a higher surgical risk require alternative treatment. Today, alcohol septal ablation is considered an effective, minimally invasive method for treating hypertrophic obstructive cardiomyopathy in patients with a left ventricular outflow tract gradient ≥ 50 mm Hg. The article presents the experience of using alcohol septal ablation in 57 patients with obstruction of the left ventricular outflow tract. Key words: alcoholic septal ablation, hypertrophic cardiomyopathy, left ventricular outflow tract obstruction.

scholarly journals Outcomes in Guideline‐Based Class I Indication Versus Earlier Referral for Surgical Myectomy in Hypertrophic Obstructive Cardiomyopathy

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Alaa Alashi ◽  
Nicholas G. Smedira ◽  
Kevin Hodges ◽  
Zoran B. Popovic ◽  
Maran Thamilarasan ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Obstructive Coronary Artery Disease ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Class I ◽  
Term Survival ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Left Ventricular Outflow ◽  
Artery Disease ◽  
Surgical Myectomy

Background In patients with obstructive hypertrophic cardiomyopathy, surgical myectomy (SM) is indicated for severe symptoms. We sought to compare long‐term outcomes of patients with obstructive hypertrophic cardiomyopathy where SM was based on guideline‐recommended Class I indication (Functional Class or FC ≥3 or angina/exertional syncope despite maximal medical therapy) versus earlier (FC 2 and/or impaired exercise capacity on exercise echocardiography with severe obstruction). Methods and Results We studied 2268 consecutive patients (excluding <18 years, ≥ moderate aortic stenosis and subaortic membrane, 56±14 years, 55% men), who underwent SM at our center between June 2002 and March 2018. Clinical data, including left ventricular outflow tract gradient, were recorded. Death and/or appropriate internal defibrillator discharge were primary composite end points. One thousand three hundred eighteen (58%) patients met Class I indication and 950 (42%) underwent earlier surgery; 222 (10%) had a history of obstructive coronary artery disease. Basal septal thickness, and resting and maximal left ventricular outflow tract gradient were 2.0±0.3 cm, 61±44 mm Hg, and 100±31 mm Hg, respectively. At 6.2±4 years after SM, 248 (11%) had composite events (13 [0.6%] in‐hospital deaths). Age (hazard ratio [HR], 1.61; 95% CI, 1.26–1.91), obstructive coronary artery disease (HR, 1.46; 95% CI, 1.06–1.91), and Class I versus earlier SM (HR, 1.61; 95% CI, 1.14–2.12) were associated with higher primary composite events (all P <0.001). Earlier surgery had better longer‐term survival (similar to age‐sex‐matched normal population) versus surgery for Class I indication (76 [8%] versus 193 [15%], P <0.001). Conclusions In patients with obstructive hypertrophic cardiomyopathy, earlier versus surgery for Class I indication had a better long‐term survival, similar to the age‐sex‐matched US population.

scholarly journals Image-Based Computational Hemodynamics Analysis of Systolic Obstruction in Hypertrophic Cardiomyopathy

2022 ◽  
Vol 12 ◽  
Author(s):  
Ivan Fumagalli ◽  
Piermario Vitullo ◽  
Christian Vergara ◽  
Marco Fedele ◽  
Antonio F. Corno ◽  
...  
Keyword(s):  
Blood Flow ◽  
Hypertrophic Cardiomyopathy ◽  
Pathological Condition ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Patient Specific ◽  
Cine Mri ◽  
Septal Myectomy ◽  
Blood Flow Dynamics

Hypertrophic Cardiomyopathy (HCM) is a pathological condition characterized by an abnormal thickening of the myocardium. When affecting the medio-basal portion of the septum, it is named Hypertrophic Obstructive Cardiomyopathy (HOCM) because it induces a flow obstruction in the left ventricular outflow tract. In any type of HCM, the myocardial function can become compromised, possibly resulting in cardiac death. In this study, we investigated with computational analysis the hemodynamics of patients with different types of HCM. The aim was quantifying the effects of this pathology on the intraventricular blood flow and pressure gradients, and providing information potentially useful to guide the indication and the modality of the surgical treatment (septal myectomy). We employed an image-based computational approach, integrating fluid dynamics simulations with geometric and functional data, reconstructed from standard cardiac cine-MRI acquisitions. We showed that with our approach we can better understand the patho-physiological behavior of intraventricular blood flow dynamics due to the abnormal morphological and functional aspect of the left ventricle. The main results of our investigation are: (a) a detailed patient-specific analysis of the blood velocity, pressure and stress distribution associated to HCM; (b) a computation-based classification of patients affected by HCM that can complement the current clinical guidelines for the diagnosis and treatment of HOCM.

Hypertrophic obstructive cardiomyopathy: review of surgical treatment

2017 ◽  
Vol 25 (9) ◽  
pp. 594-607 ◽  
Author(s):  
Jonathan Price ◽  
Nicholas Clarke ◽  
Aslan Turer ◽  
Eduard Quintana ◽  
Carlos Mestres ◽  
...  
Keyword(s):  
Left Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Outflow Tract ◽  
Percutaneous Ablation ◽  
Pharmacologic Therapy ◽  
Ventricular Outflow Tract Obstruction ◽  
Left Ventricular Outflow ◽  
Surgical Myectomy

Hypertrophic cardiomyopathy ranks among the most common congenital cardiac diseases, affecting up to 1 in 200 of the general population. When it causes left ventricular outflow tract obstruction, treatment is guided to reduce symptoms and the risk of sudden cardiac death. Pharmacologic therapy is the first-line treatment, but when it fails, surgical myectomy or percutaneous ablation of the hypertrophic myocardium are the standard therapies to eliminate subaortic obstruction. Both surgical myectomy and percutaneous ablation are proven safe and effective treatments; however, myectomy is the gold standard with a significantly lower complication rate and more complete and lasting reduction of left ventricular outflow tract obstruction.

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