Pubblicazioni recenti - hypertrophic cardiomyopathy
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Benefits of ninerafaxstat in non-obstructive hypertrophic cardiomyopathy.
Nat Rev Cardiol -
Cardiac manifestations in inherited metabolic diseases.
Curr Probl Cardiol2024 Apr;():102587. doi: 10.1016/j.cpcardiol.2024.102587.
Cuenca-Gómez Jose Ángel, Lara-Rojas Carmen María, Bonilla-López Antonio,
Abstract
Inherited metabolic diseases (IMD) are caused by the functional defect of an enzyme, of genetic origin, that provokes a blockage in a specific metabolic pathway. Individually, IMD are considered rare diseases, with an incidence of less than 1/100,000 births. The symptoms are usually multisystemic, but frequently include cardiac manifestations. Of these, the most common are cardiomyopathies, especially hypertrophic cardiomyopathy. In addition, they can cause dilated or restrictive cardiomyopathy and non-compacted cardiomyopathy of the left ventricle. Characteristic signs also include rhythm alterations (atrio-ventricular conduction disturbances, Wolff-Parkinson-White syndrome or ventricular arrhythmias), valvular pathology and ischaemic coronary pathologies. The aim of this study is to present a narrative review of the IMDthat may produce cardiac involvement. We describe both the specific cardiac manifestations of each disease and the systemic symptoms that guide diagnosis.
Copyright © 2024. Published by Elsevier Inc.
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Adult-Onset Dystonia and Hypertrophic Cardiomyopathy in Patient with a De Novo 16q12.2q21 Deletion.
Mov Disord2024 Apr;():. doi: 10.1002/mds.29818.
Qin Shaochen, Li Yifeng, Li Yanjing, Wu Yiwen,
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MYBPC3-c.772G?>?a mutation results in haploinsufficiency and altered myosin cycling kinetics in a patient induced stem cell derived cardiomyocyte model of hypertrophic cardiomyopathy.
J Mol Cell Cardiol2024 Apr;():. doi: S0022-2828(24)00054-3.
Sonette Steczina, Mohran Saffie, Bailey Logan R J, McMillen Timothy S, Kooiker Kristina B, Wood Neil B, Davis Jennifer, Previs Michael J, Olivotto Iacopo, Pioner Josè Manuel, Geeves Michael A, Poggesi Corrado, Regnier Michael,
Abstract
Approximately 40% of hypertrophic cardiomyopathy mutations are linked to the sarcomere protein cardiac myosin binding protein-C (cMyBP-C). These mutations are either classified as missense mutations or truncation mutations. One mutation whose nature has been inconsistently reported in the literature is the MYBPC3-c.772G?>?A mutation. Using patient-derived human induced pluripotent stem cells differentiated to cardiomyocytes (hiPSC-CMs), we have performed a mechanistic study of the structure-function relationship for this MYBPC3-c.772G?>?A mutation versus a mutation corrected, isogenic cell line. Our results confirm that this mutation leads to exon skipping and mRNA truncation that ultimately suggests ~20% less cMyBP-C protein (i.e., haploinsufficiency). This, in turn, results in increased myosin recruitment and accelerated myofibril cycling kinetics. Our mechanistic studies suggest that faster ADP release from myosin is a primary cause of accelerated myofibril cross-bridge cycling due to this mutation. Additionally, the reduction in force generating heads expected from faster ADP release during isometric contractions is outweighed by a cMyBP-C phosphorylation mediated increase in myosin recruitment that leads to a net increase of myofibril force, primarily at sub-maximal calcium activations. These results match well with our previous report on contractile properties from myectomy samples of the patients from whom the hiPSC-CMs were generated, demonstrating that these cell lines are a good model to study this pathological mutation and extends our understanding of the mechanisms of altered contractile properties of this HCM MYBPC3-c.772G?>?A mutation.
Copyright © 2023. Published by Elsevier Ltd.
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Mechanisms of ischaemia-induced arrhythmias in hypertrophic cardiomyopathy: a large-scale computational study.
Cardiovasc Res2024 Apr;():. doi: cvae086.
Coleman James A, Doste Ruben, Ashkir Zakariye, Coppini Raffaele, Sachetto Rafael, Watkins Hugh, Raman Betty, Bueno-Orovio Alfonso,
Abstract
AIMS:
Lethal arrhythmias in hypertrophic cardiomyopathy (HCM) are widely attributed to myocardial ischaemia and fibrosis. How these factors modulate arrhythmic risk remains largely unknown, especially as invasive mapping protocols are not routinely used in these patients. By leveraging multiscale digital-twin technologies, we aim to investigate ischaemic mechanisms of increased arrhythmic risk in HCM.
METHODS AND RESULTS:
Computational models of human HCM cardiomyocytes, tissue and ventricles were used to simulate outcomes of phase 1A acute myocardial ischaemia. Cellular response predictions were validated with patch-clamp studies of human HCM cardiomyocytes (n=12 cells, N=5 patients). Ventricular simulations were informed by typical distributions of subendocardial/transmural ischaemia as analysed in perfusion scans (N=28 patients). S1-S2 pacing protocols were used to quantify arrhythmic risk for scenarios in which regions of septal obstructive hypertrophy were affected by (i) ischaemia, (ii) ischaemia and impaired repolarisation, and (iii) ischaemia, impaired repolarisation, and diffuse fibrosis.HCM cardiomyocytes exhibited enhanced action potential and abnormal effective refractory period shortening to ischaemic insults. Analysis of c.a. 75,000 re-entry induction cases revealed that the abnormal HCM cellular response enabled establishment of arrhythmia at milder ischaemia than otherwise possible in healthy myocardium, due to larger refractoriness gradients that promoted conduction block. Arrhythmias were more easily sustained in transmural than subendocardial ischaemia. Mechanisms of ischaemia-fibrosis interaction were strongly electrophysiology dependent. Fibrosis enabled asymmetric re-entry patterns and break-up into sustained ventricular tachycardia.
CONCLUSIONS:
HCM ventricles exhibited an increased risk to non-sustained and sustained re-entry, largely dominated by an impaired cellular response and deleterious interactions with the diffuse fibrotic substrate.
© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.
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Cardiac adaptation and malformation in twin-twin transfusion syndrome and selective fetal growth restriction: A systematic review.
Prenat Diagn2024 Apr;():. doi: 10.1002/pd.6575.
Noll Anne T R, Gijtenbeek Manon, Verweij E J T Joanne, Lewi Liesbeth, Herling Lotta, Haak Monique C,
Abstract
OBJECTIVES:
This systematic review explores cardiac adaptation in monochorionic (MC) twins with twin-twin transfusion syndrome (TTTS) or selective fetal growth restriction (sFGR) and assesses the risk of congenital heart defects (CHDs).
METHODS:
Adhering to PRISMA guidelines, 63 studies were reviewed (49 on cardiac adaptation, 13 on CHD, one on both). A narrative synthesis of cardiac adaptation patterns was performed. Additionally, a meta-analysis compared the livebirth prevalence of CHD in TTTS and sFGR against uncomplicated MC twins.
RESULTS:
In TTTS recipients, cardiac function may be impaired for diastolic, systolic, as well as global functions, while in donors, cardiac function is generally preserved. In sFGR, large twins may show hypertrophic cardiomyopathy, and small twins may show impaired systolic function. Co-occurrence of TTTS and sFGR magnifies cardiac impact but is often underreported. Meta-analysis for CHD prevalence revealed a relative risk ratio of 3.5 (95% CI: 2.5-4.9) for TTTS and 2.2 (95%CI: 1.3-3.5) for sFGR compared with uncomplicated MC twins.
CONCLUSIONS:
This study highlights the well-documented cardiac adaptation in TTTS, contrasting with limited understanding in sFGR. Elevated CHD risks were observed in both conditions. Enhanced cardiovascular surveillance is warranted in complicated MC twin pregnancies. Future research should explore cardiac adaptation in sFGR and its long-term consequences.
© 2024 The Authors. Prenatal Diagnosis published by John Wiley & Sons Ltd.
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The feasibility of left ventricular strain and strain rate for evaluating patients with risk factors of sudden cardiac death in hypertrophic cardiomyopathy by Feature-tracking Cardiac Magnetic Resonance.
Am J Cardiol2024 Apr;():. doi: S0002-9149(24)00285-6.
Zhu Xinyu, Tian Yuan, Shi Ying, Lian Jianxiu, Shen Honghu, Li Lulu, Wu Haishan, Liu Pengfei,
Abstract
Sudden cardiac death (SCD) represents the most severe complication of hypertrophic cardiomyopathy (HCM). However, the relationship between strain, strain rate, and risk factors in SCD risk stratification remains elusive. The study aimed to assess the attenuation of strain and strain rate in HCM by feature-tracking cardiac magnetic resonance (FT-CMR). All strain and strain rates were obtained automatically by FT, with manual adjustment of endocardial and epicardial borders. Strain indicators included left ventricular (LV) global longitudinal (GLS), circumferential (GCS), radial strain (GRS), peak diastolic-longitudinal (PD-LSR), circumferential (PD-CSR), and radial strain rate (PD-RSR). Patients were categorized into high and low-risk groups for SCD based on the 2020 American Heart Association/American College (AHA/ACC) HCM Risk-SCD model. The correlation between strain/strain rate and SCD risk factors was assessed via Spearman correlation analysis. Furthermore, a multivariate logistic regression analysis was conducted to explore the factors that influence SCD risk in HCM patients. A total of 105 HCM patients were analyzed in this study, including 38 patients in the high-risk group, and 67 patients in the low-risk group. Compared to the low-risk group, the high-risk group exhibited significantly worse strain and strain rate (p
Copyright © 2024. Published by Elsevier Inc.
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Myosin inhibitor reverses hypertrophic cardiomyopathy in genotypically diverse pediatric iPSC-cardiomyocytes to mirror variant correction.
Cell Rep Med2024 Apr;():101520. doi: 10.1016/j.xcrm.2024.101520.
Kinnear Caroline, Said Abdelrahman, Meng Guoliang, Zhao Yimu, Wang Erika Y, Rafatian Naimeh, Parmar Neha, Wei Wei, Billia Filio, Simmons Craig A, Radisic Milica, Ellis James, Mital Seema,
Abstract
Pathogenic variants in MYH7 and MYBPC3 account for the majority of hypertrophic cardiomyopathy (HCM). Targeted drugs like myosin ATPase inhibitors have not been evaluated in children. We generate patient and variant-corrected iPSC-cardiomyocytes (CMs) from pediatric HCM patients harboring single variants in MYH7 (V606M; R453C), MYBPC3 (G148R) or digenic variants (MYBPC3 P955fs, TNNI3 A157V). We also generate CMs harboring MYBPC3 mono- and biallelic variants using CRISPR editing of a healthy control. Compared with isogenic and healthy controls, variant-positive CMs show sarcomere disorganization, higher contractility, calcium transients, and ATPase activity. However, only MYH7 and biallelic MYBPC3 variant-positive CMs show stronger myosin-actin binding. Targeted myosin ATPase inhibitors show complete rescue of the phenotype in variant-positive CMs and in cardiac Biowires to mirror isogenic controls. The response is superior to verapamil or metoprolol. Myosin inhibitors can be effective in genotypically diverse HCM highlighting the need for myosin inhibitor drug trials in pediatric HCM.
Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.
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Myocardial injury in dogs: a retrospective analysis on etiological, echocardiographic, electrocardiographic, therapeutic, and outcome findings in 102 cases.
J Vet Cardiol2024 Mar;53():36-51. doi: 10.1016/j.jvc.2024.03.004.
Romito G, Palatini L, Sabetti M C, Cipone M,
Abstract
INTRODUCTION:
In dogs, myocardial injury (MI) is a poorly characterized clinical entity; therefore, this study aimed to provide a detailed description of dogs affected by this condition.
ANIMALS, MATERIALS, AND METHODS:
Dogs diagnosed with MI according to the concentration of cardiac troponin I (cTnI) were retrospectively searched. Signalment, diagnostic, therapeutic, and outcome data were retrieved. Dogs were divided into six echocardiographic (dilated cardiomyopathy phenotype; hypertrophic cardiomyopathy phenotype; hypertrophic cardiomyopathy phenotype with systolic dysfunction; abnormal echogenicity only; endocarditis; and no echocardiographic abnormalities suggestive of MI), four electrocardiographic (abnormalities of impulse formation; abnormalities of impulse conduction; abnormalities of ventricular repolarization; and no electrocardiographic abnormalities suggestive of MI), and nine etiological (infective; inflammatory; neoplastic; metabolic; toxic; nutritional; immune-mediated; traumatic/mechanical; and unknown) categories. Statistical analysis was performed to compare cTnI values among different categories and analyze survival.
RESULTS:
One hundred two dogs were included. The median cTnI value was 3.71 ng/mL (0.2-180 ng/mL). Echocardiographic and electrocardiographic abnormalities were documented in 86 of 102 and 89 of 102 dogs, respectively. Among echocardiographic and electrocardiographic categories, the dilated cardiomyopathy phenotype (n = 52) and abnormalities of impulse formation (n = 67) were overrepresented, respectively. Among dogs in which a suspected etiological trigger was identified (68/102), the infective category was overrepresented (n = 20). Among dogs belonging to different echocardiographic, electrocardiographic, and etiological categories, cTnI did not differ significantly. The median survival time was 603 days; only eight of 102 dogs died due to MI.
CONCLUSIONS:
Dogs with MI often have an identifiable suspected trigger, show various echocardiographic and electrocardiographic abnormalities, and frequently survive to MI-related complications.
Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.
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Circular RNA circZFPM2 regulates cardiomyocyte hypertrophy and survival.
Basic Res Cardiol2024 Apr;():. doi: 10.1007/s00395-024-01048-y.
Neufeldt Dimyana, Schmidt Arne, Mohr Elisa, Lu Dongchao, Chatterjee Shambhabi, Fuchs Maximilian, Xiao Ke, Pan Wen, Cushman Sarah, Jahn Christopher, Juchem Malte, Hunkler Hannah Jill, Cipriano Giuseppe, Jürgens Bjarne, Schmidt Kevin, Groß Sonja, Jung Mira, Hoepfner Jeannine, Weber Natalie, Foo Roger, Pich Andreas, Zweigerdt Robert, Kraft Theresia, Thum Thomas, Bär Christian,
Abstract
Hypertrophic cardiomyopathy (HCM) constitutes the most common genetic cardiac disorder. However, current pharmacotherapeutics are mainly symptomatic and only partially address underlying molecular mechanisms. Circular RNAs (circRNAs) are a recently discovered class of non-coding RNAs and emerged as specific and powerful regulators of cellular functions. By performing global circRNA-specific next generation sequencing in cardiac tissue of patients with hypertrophic cardiomyopathy compared to healthy donors, we identified circZFPM2 (hsa_circ_0003380). CircZFPM2, which derives from the ZFPM2 gene locus, is a highly conserved regulatory circRNA that is strongly induced in HCM tissue. In vitro loss-of-function experiments were performed in neonatal rat cardiomyocytes, human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs), and HCM-patient-derived hiPSC-CMs. A knockdown of circZFPM2 was found to induce cardiomyocyte hypertrophy and compromise mitochondrial respiration, leading to an increased production of reactive oxygen species and apoptosis. In contrast, delivery of recombinant circZFPM2, packaged in lipid-nanoparticles or using AAV-based overexpression, rescued cardiomyocyte hypertrophic gene expression and promoted cell survival. Additionally, HCM-derived cardiac organoids exhibited improved contractility upon CM-specific overexpression of circZFPM2. Multi-Omics analysis further promoted our hypothesis, showing beneficial effects of circZFPM2 on cardiac contractility and mitochondrial function. Collectively, our data highlight that circZFPM2 serves as a promising target for the treatment of cardiac hypertrophy including HCM.
© 2024. The Author(s).
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Mitral Regurgitation in Obstructive Hypertrophic Cardiomyopathy: Insight from the VALOR-HCM Study.
JACC Cardiovasc Imaging2024 Apr;():. doi: S1936-878X(24)00109-8.
Cremer Paul C, Geske Jeffrey B, Owens Anjali, Jaber Wael A, Harb Serge C, Saberi Sara, Wang Andrew, Sherrid Mark, Naidu Srihari S, Schaff Hartzell V, Smedira Nicholas G, Wang Qiuqing, Wolski Kathy, Lampl Kathy L, Sehnert Amy J, Nissen Steven E, Desai Milind Y,
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Clinical characteristics and outcomes of alcohol septal ablation in the era of transcatheter valve interventions.
Catheter Cardiovasc Interv2024 Apr;():. doi: 10.1002/ccd.31051.
Engel Gonzalez Pedro, Gregerson Samuel, Mahmood Shazil, Brooks Collin, Villablanca Pedro A, Frisoli Tiberio M, Lee James, Wyman Janet F, Wang Dee Dee, O'Neill William W, O'Neill Brian P,
Abstract
BACKGROUND:
The clinical efficacy and safety of alcohol septal ablation (ASA) for obstructive hypertrophic cardiomyopathy (HCM) have been well-established; however, less is known about outcomes in patients undergoing preemptive ASA before transcatheter mitral valve replacement (TMVR).
AIMS:
The goal of this study is to characterize the procedural characteristics and examine the clinical outcomes of ASA in both HCM and pre-TMVR.
METHODS:
This retrospective study compared procedural characteristics and outcomes in patient who underwent ASA for HCM and TMVR.
RESULTS:
In total, 137 patients were included, 86 in the HCM group and 51 in the TMVR group. The intraventricular septal thickness (mean 1.8 vs. 1.2?cm; p?0.0001) and the pre-ASA LVOT gradient (73.6 vs. 33.8?mmHg; p???0.001) were higher in the HCM group vs the TMVR group. The mean volume of ethanol injected was higher (mean 2.4 vs. 1.7?cc; p?0.0001). The average neo-left ventricular outflow tract area increased significantly after ASA in the patients undergoing TMVR (99.2?±?83.37?mm vs. 196.5?±?114.55?mm; p?=?
CONCLUSIONS:
Preemptive ASA before TMVR was performed in patients with higher degree of clinical comorbidities, and correspondingly is associated with worse short-term clinical outcomes in comparison to ASA for HCM patients. ASA before TMVR enabled percutaneous mitral interventions in a small but significant minority of patients that would have otherwise been excluded. The degree of LVOT and neoLVOT area increase is significant and predictable.
© 2024 Wiley Periodicals LLC.
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Exercise limitation in hypertrophic cardiomyopathy: combined stress echocardiography and cardiopulmonary exercise test.
ESC Heart Fail2024 Apr;():. doi: 10.1002/ehf2.14776.
Erez Yonatan, Ghantous Eihab, Shetrit Aviel, Zamanzadeh Ryan S, Zahler David, Granot Yoav, Sapir Orly Ran, Laufer Perl Michal, Banai Shmuel, Topilsky Yan, Havakuk Ofer,
Abstract
AIMS:
The study aims to investigate exercise-limiting factors in hypertrophic cardiomyopathy (HCM) using combined stress echocardiography and cardiopulmonary exercise test.
METHODS AND RESULTS:
A symptom-limited ramp bicycle exercise test was performed in the semi-supine position on a tilting dedicated ergometer. Echocardiographic images were obtained concurrently with gas exchange measurements along predefined stages of exercise. Oxygen extraction was calculated using the Fick equation at each activity level. Thirty-six HCM patients (mean age 67 ± 6 years, 72% men, 18 obstructive HCM) were compared with age and sex-matched 29 controls. At rest, compared with controls, E/E' ratio (6.26 ± 2.3 vs. 14 ± 2.5, P
CONCLUSIONS:
Both central and peripheral cardiovascular limitations are involved in exercise intolerance in HCM. Diastolic dysfunction seems to be the main driver for this limitation.
© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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Prevalence and Predictors of Readmissions Among Patients with Hypertrophic Cardiomyopathy and Atrial Fibrillation/Flutter.
Am J Cardiol2024 Apr;():. doi: S0002-9149(24)00269-8.
Almani Muhammad Usman, Talha Khawaja Muhammad, Khan Laibah Arhsad, Hameed Ishaque, Asad Zain Ul Abideen, Fudim Marat, Krasuski Richard, Khan Muhammad Shahzeb,
Abstract
Atrial fibrillation/flutter (AF) is the most common dysrhythmia in patients with hypertrophic cardiomyopathy (HCM). Unexplained left ventricular hypertrophy and left ventricular outflow tract (LVOT) obstruction are integral components of HCM pathology which can cause increased left atrial pressure and atrial myopathy contributing to the substrate for AF. Our aim was to determine the impact of AF on hospital readmissions in patients with HCM. We conducted a retrospective analysis using the 2015 - 2019 Nationwide Readmission Database to analyze the effect of AF on 30-day readmission and causes of 30-day readmission in patients with HCM. We also determined the hospital, patient and procedure-specific independent predictors of readmission in patients with HCM and AF. Among 191,235 index HCM hospitalizations, 81,390 (42.6%) had a secondary diagnosis of AF. A total of 16.9% patients with HCM and AF were readmitted within 30 days as compared with 14% of HCM patients without AF. The presence of AF was independently associated with a higher risk of all-cause 30-day readmission (HR 1.21, 95% CI 1.17 - 1.25, p
Copyright © 2024. Published by Elsevier Inc.
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Evaluating the efficacy and safety of mavacamten in hypertrophic cardiomyopathy: A systematic review and meta-analysis focusing on qualitative assessment, biomarkers, and cardiac imaging.
PLoS One2024 ;19(4):e0301704. doi: e0301704.
Vyas Rahul, Panchal Viraj, Jain Shubhika, Sondhi Manush, Singh Mansunderbir, Jaisingh Keerthish, Thotamgari Sahith Reddy, Thakre Anuj, Modi Kalgi,
Abstract
BACKGROUND:
Hypertrophic Cardiomyopathy (HCM) is a complex cardiac condition characterized by hypercontractility of cardiac muscle leading to a dynamic obstruction of left ventricular outlet tract (LVOT). Mavacamten, a first-in-class cardiac myosin inhibitor, is increasingly being studied in randomized controlled trials. In this meta-analysis, we aimed to analyse the efficacy and safety profile of Mavacamten compared to placebo in patients of HCM.
METHOD:
We carried out a comprehensive search in PubMed, Cochrane, and clinicaltrials.gov to analyze the efficacy and safety of mavacamten compared to placebo from 2010 to 2023. To calculate pooled odds ratio (OR) or risk ratio (RR) at 95% confidence interval (CI), the Mantel-Haenszel formula with random effect was used and Generic Inverse Variance method assessed pooled mean difference value at a 95% CI. RevMan was used for analysis. P
RESULTS:
We analyzed five phase 3 RCTs including 609 patients to compare mavacamten with a placebo. New York Heart Association (NYHA) grade improvement and KCCQ score showed the odds ratio as 4.94 and 7.93 with p
CONCLUSION:
Mavacamten influences diverse facets of HCM comprehensively. Notably, our study delved into the drug's impact on the heart's structural and functional aspects, providing insights that complement prior findings. Further large-scale trials are needed to evaluate the safety profile of Mavacamten.
Copyright: © 2024 Vyas et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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Impact of the New Heart Allocation System on the Medium-Term Outcomes in Patients With Hypertrophic Cardiomyopathy.
ASAIO J2024 Apr;():. doi: 10.1097/MAT.0000000000002216.
Mazur Matylda, Carmona Rubio Andres, Eisen Howard J, Bhat Geetha, Dowling Robert,
Abstract
The introduction of the new heart allocation system in the United States in 2018 resulted in an increase in the number of heart transplants (HT) performed among patients with hypertrophic cardiomyopathy (HCM). However, whether that affected medium-term post-HT outcomes in this group of patients remains unknown. We conducted an analysis of the United Network for Organ Sharing Transplant Database, including adults with HCM who underwent heart transplantation between 2015 and 2021. Patients were divided into two equal-duration eras: Era 1 (October 17, 2015, to October 17, 2018) and Era 2 (October 18, 2018, to October 18, 2021). In the studied period, 444 patients with HCM underwent HT: 204 in Era 1 and 240 in Era 2. In Era 2, the waitlist time was shorter, transplant rates were higher, patients were less frequently supported with inotropes but more often with an IABP, ischemic time was longer, and donor-to-recipient distance larger. Pre- and post-transplant functional status was comparable across the two eras, while the pre-HT employment rate was higher in the new system. The 3 year survival was unchanged across eras. In the new allocation system, despite more frequent mechanical circulatory support (MCS) use and increased ischemic time, the medium-term outcomes of patients with HCM remained favorable.
Copyright © ASAIO 2024.
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Intramyocardial calcification in apical hypertrophic cardiomyopathy assessed using multimodality imaging: a case series.
ESC Heart Fail2024 Apr;():. doi: 10.1002/ehf2.14775.
Radano Ilaria, Mabritto Barbara, Luceri Stefania, Bongioanni Sergio, Maiellaro Francesco, Zappia Luca, Lario Chiara, Macera Annalisa, Cirillo Stefano, Pizzuti Alfredo, Citro Rodolfo, Galasso Gennaro, Musumeci Giuseppe,
Abstract
Apical hypertrophic cardiomyopathy (ApHCM) is an HCM variant, affecting frequently males in midlife. It is characterized by apical obliteration and persistent diastolic contraction, often resulting in microvascular ischaemia. We report five cases of ApHCM, with evidence of intramyocardial calcification on echocardiogram. On cardiac magnetic imaging (MRI), a hypointense component at early gadolinium enhancement (EGE) sequences, compatible with calcium, and a deep layer, with hyperintensity at late gadolinium enhancement (LGE) sequences, referable to fibrosis, suggest an endomyocardial fibrosis (EMF) diagnosis. EMF pathologic hallmark is endocardium and myocardium scarring, evolving to dystrophic calcification. It is found only in few ApHCM patients. Our series is the largest one described until now. Analysing patients' history, coexistent inflammatory triggers were evident in all of them, so their co-morbidities could represent a further cause of small vessel disease, in the context of ischaemic microvascular stress due to hypertrophy, leading to fibrosis and dystrophic calcification. This series could demonstrate the relation between apical fibrosis/calcification and microvascular ischaemia due to hypertrophy and inflammatory triggers.
© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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Calorie restriction anti-hypertrophic effects are associated with improved mitochondrial content, blockage of Ca-induced mitochondrial damage, and lower reverse electron transport-mediated oxidative stress.
Free Radic Res2024 Apr;():1-18. doi: 10.1080/10715762.2024.2342962.
Brito Lucas Aline Maria, Bezerra Palacio Plinio, Oliveira Cunha Pedro Lourenzo, Tarso Facundo Heberty,
Abstract
Calorie restriction is a nutritional intervention that reproducibly protects against the maladaptive consequences of cardiovascular diseases. Pathological cardiac hypertrophy leads to cellular growth, dysfunction (with mitochondrial dysregulation), and oxidative stress. The mechanisms behind the cardiovascular protective effects of calorie restriction are still under investigation. In this study, we show that this dietetic intervention prevents cardiac protein elevation, avoids fetal gene reprogramming (atrial natriuretic peptide), and blocks the increase in heart weight per tibia length index (HW/TL) seen in isoproterenol-induced cardiac hypertrophy. Our findings suggest that calorie restriction inhibits cardiac pathological growth while also lowering mitochondrial reverse electron transport-induced hydrogen peroxide formation and improving mitochondrial content. Calorie restriction also attenuated the opening of the Ca-induced mitochondrial permeability transition pore. We also found that calorie restriction blocked the negative correlation of antioxidant enzymes (superoxide dimutase and glutatione peroxidase activity) and HW/TL, leading to the maintenance of protein sulphydryls and glutathione levels. Given the nature of isoproterenol-induced cardiac hypertrophy, we investigated whether calorie restriction could alter cardiac beta-adrenergic sensitivity. Using isolated rat hearts in a Langendorff system, we found that calorie restricted hearts have preserved beta-adrenergic signaling. In contrast, hypertrophic hearts (treated for seven days with isoproterenol) were insensitive to beta-adrenergic activation using isoproterenol (50?nM). Despite protecting against cardiac hypertrophy, calorie restriction did not alter the lack of responsiveness to isoproterenol in isolated hearts harvested from isoproterenol-treated rats. These results suggest (through a series of mitochondrial, oxidative stress, and cardiac hemodynamic studies) that calorie restriction possesses beneficial effects against hypertrophic cardiomyopathy.
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Machine learning-driven diagnostic signature provides new insights in clinical management of hypertrophic cardiomyopathy.
ESC Heart Fail2024 Apr;():. doi: 10.1002/ehf2.14762.
Liu Shutong, Yuan Peiyu, Zheng Youyang, Guo Chunguang, Ren Yuqing, Weng Siyuan, Zhang Yuyuan, Liu Long, Xing Zhe, Wang Libo, Han Xinwei,
Abstract
AIMS:
In an era of evolving diagnostic possibilities, existing diagnostic systems are not fully sufficient to promptly recognize patients with early-stage hypertrophic cardiomyopathy (HCM) without symptomatic and instrumental features. Considering the sudden death of HCM, developing a novel diagnostic model to clarify the patients with early-stage HCM and the immunological characteristics can avoid misdiagnosis and attenuate disease progression.
METHODS AND RESULTS:
Three hundred eighty-five samples from four independent cohorts were systematically retrieved. The weighted gene co-expression network analysis, differential expression analysis (|log2(foldchange)| > 0.5 and adjusted P
CONCLUSIONS:
Our study revealed a number of hub genes and novel pathways to provide potential targets for the treatment of HCM. A stable model was developed, providing an efficient tool for the diagnosis of HCM.
© 2024 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.
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Comparing long-term outcomes of septal myectomy and mitral valve replacement in hypertrophic cardiomyopathy patients: A retrospective cohort study in Iran.
Health Sci Rep2024 Apr;7(4):e2045. doi: e2045.
Ayati Aryan, Khoshfetrat Mehran, Davoodi Saeed, Ahmadi Tafti Seyed Hossein, Arefizadeh Reza,
Abstract
BACKGROUND:
Hypertrophic cardiomyopathy (HCM) affects millions of individuals worldwide. In severe cases, it can cause life-threatening conditions such as left ventricular outflow tract (LVOT) obstruction, mitral regurgitation (MR), and sudden cardiac death, making surgical treatment necessary. This study aimed to report the long-term outcomes of HCM patients undergoing septal myectomy or mitral valve replacement (MVR) and compare the results between different types of surgeries.
METHODS:
This was a retrospective cohort study on HCM patients who underwent surgical treatment in an Iranian referral center between 2005 and 2021. Patients were divided into three groups according to the type of surgery received: septal myectomy, MVR, or a combination of both surgeries. Patient characteristics, surgical and echocardiographic features, and in-hospital and long-term outcomes were reported and compared between the three groups.
RESULTS:
A total of 102 patients with an average age of 53.3?±?16.9 were included. Twenty-six patients had septal myectomy, 23 had MVR, and 53 had combined septal myectomy and MVR surgery. All surgeries were associated with a significant reduction in interventricular septum thickness and LVOT gradients. After a median of 6.8-year follow-up time, patients with an isolated septal myectomy had significantly lower mortality and major adverse cardiac and cerebrovascular events rates than the other groups.
CONCLUSION:
Isolated septal myectomy showed better long-term survival rates and can correct HCM-related MR, while MVR should be preserved only for intrinsic valve defects. More extensive studies are needed to confirm these findings and achieve a comprehensive guideline on surgical treatment of HCM.
© 2024 The Authors. Health Science Reports published by Wiley Periodicals LLC.
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