Impact of the UNOS Donor Heart Allocation System in Adult Heart Transplant Recipients with Hypertrophic Cardiomyopathy in the United States
Purpose: The latest donor heart allocation system in the United States favors transplant for patients receiving temporary mechanical circulatory support (MCS). However, such a system could be disadvantageous to patients with hypertrophic cardiomyopathy (HCM) awaiting heart transplant (HT). We sought to examine the trends and outcomes in HT in patients with HCM. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , N. Patel, I. Konstantinidis, A. Pillai, K. Singh, W. Baker, A. Jaiswal Source Type: research

Impact of the New 2018 US Donor Heart Allocation System on the Long-Term Outcomes in Patients with Hypertrophic Cardiomyopathy
Purpose: The introduction of the new heart allocation system in the US in October 2018 resulted in an increase in the number of orthotopic heart transplants (OHT) performed among patients with hypertrophic cardiomyopathy (HCM). However, whether that affected long-term (>1 year) post-transplant outcomes in these patients remains unknown. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , A. Carmona Rubio, E. Popjes, G. Bhat, R. Dowling, H. Eisen Source Type: research

Perception vs. Reality: Reported and Objective Measures of Physical Activity in Children with Hypertrophic Cardiomyopathy (HCM)
Purpose: Physical activity (PA) is a pillar of cardiovascular health. However, those with hypertrophic cardiomyopathy (HCM) are often counselled to limit PA due to potential risk of sudden cardiac death (SCD). No clear data exists on how active youth with HCM are and what they perceive as barriers to increasing PA. We therefore aimed to quantify the level of PA in this population and assess the barriers to a heart healthy lifestyle. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , A. Jeewa, S. Lemaire-Paquette, T. Pidborochynski, M. Khoury, C. Cunningham, S. Dhillon, N. Alami Laroussi, L. Vaujois, F. Dallaire, K. Armstrong, D. Schantz, W. Mawad, T. Bradley, J. Conway Source Type: research

Left Ventricle Assist Device as a Treatment for Burned Out Hypertrophic Obstructive Cardiomyopathy
Introduction: LVAD is a promising treatment for severe burned-out hypertrophic obstructive cardiomyopathy(HOCM)with refractory HF (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: R. Sadraldin, , A. Alkhaldi, M. Bin Hudhud Source Type: research

Hypertrophic Cardiomyopathy and Rare Genetic Mutations
Introduction: Hypertrophic cardiomyopathy (HCM) is a genetic disorder of cardiomyocytes, whose prevalence is approximately 1:500 in the general adult population, with a wide spectrum of clinical and morphological presentation. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , G. Campos, S. Mangini, S. Moraes, F. Bacal Source Type: research

Impact of Neighborhood Factors on Exercise Capacity in Children with Hypertrophic Cardiomyopathy
Purpose: Interventions to improve exercise capacity in children with hypertrophic cardiomyopathy (HCM) may be a critical way to improve cardiovascular risk and quality of life. However, there is limited data on the environmental determinants of exercise capacity in children with HCM. Understanding this potential link is a vital step in designing effective and equitable interventions (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , L. Wang, A.L. Roberts, H. Stanley, J. Rossano, M. O'Connor, K. Lin, C. Wittlieb-Weber, H. Ahmed, J. Edwards, V. Tam, J.B. Edelson Source Type: research

Hypertrophic Cardiomyopathy Patients Have More Adverse Hemodynamic Profiles on Pre-Transplant Cardiopulmonary Exercise Testing
Purpose: Non-invasive cardiopulmonary exercise testing (CPET) is useful for predicting transplant-free survival in patients with advanced heart failure (HF). We sought to investigate the differences in CPET parameters in patients with hypertrophic cardiomyopathy (HCM) compared to other etiologies of HF. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , C. Lee, A. Fernandez Valledor, R. Goldsmith, G. Sayer, N. Uriel Source Type: research

Mavacamten Use for Obstructive Hypertrophic Cardiomyopathy and Effect on Beta-Blocker Therapy in an Academic Institution
Purpose: Mavacamten is the first and only cardiac myosin inhibitor approved for the management of obstructive hypertrophic cardiomyopathy (oHCM). While it has been shown to decrease left ventricular outflow tract (LVOT) obstruction in landmark trials, studies on real-world use of mavacamten are still ongoing. First-line therapy includes β-blocker and calcium channel blockers (CCB), with disopyramide as an option for refractory patients. In the EXPLORER-HCM trial, 76% of patients (n = 94) on mavacamten were also on beta-blocker, with only 17% (n= 16) requiring adjustments to the dose of β-blocker therapy. (Source: The Jou...
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , C.K. Cornelio, R. Wu, A.C. Miranda Source Type: research

Genotype Analysis of LVOT Gradient Measurements in Patients Receiving Mavacamten for Obstructive Hypertrophic Cardiomyopathy?
Purpose: Hypertrophic cardiomyopathy (HCM) is the most common genetically inherited cardiac condition encountered. Recent utilization of cardiac myosin inhibitors has altered the management of obstructive symptoms. In this retrospective analysis, we compare the response to therapy in patients with obstructive HCM being treated with mavacamten based on genetic positivity. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , C.K. Cornelio, A.C. Miranda, R. Wu Source Type: research

Genetic Signatures in Heart Transplant and LVAD Patients: Unraveling Their Association with Hypertrophic and Dilated Cardiomyopathy Subtypes
This study evaluates genetic testing outcomes in a cohort of 40 HTx patients and 20 LVAD patients for bridge to transplantation, with a focus on hypertrophic cardiomyopathy (HCMP) and dilated cardiomyopathy (DCMP) subgroups. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: K. Kim Source Type: research

Exception Requests and Approval Rates After Implementation of the National Heart Review Board for Pediatrics
Purpose: The OPTN Guidance for pediatric heart transplant exception requests addresses dilated cardiomyopathy (DCM), hypertrophic or restrictive cardiomyopathy (HCM/RCM), single ventricle congenital heart disease (1V CHD), and cardiac allograft vasculopathy (CAV). The purpose of our study was to evaluate the use and approval rates of exception requests after the implementation of the National Heart Review Board (NHRB) for Pediatrics. (Source: The Journal of Heart and Lung Transplantation)
Source: The Journal of Heart and Lung Transplantation - April 1, 2024 Category: Transplant Surgery Authors: , D. Bearl, L. Wright Source Type: research

A novel gene-trap line reveals the dynamic patterns and essential roles of cysteine and glycine-rich protein 3 in zebrafish heart development and regeneration
In this study, we characterized a novel zebrafish gene-trap line, gSAIzGFFM218A, which harbors an insertion in the csrp3 genomic locus, heterozygous fish served as a csrp3 expression reporter line and homozygous fish served as a csrp3 mutant line. We discovered that csrp3 is specifically expressed in larval ventricular cardiomyocytes (CMs) and that csrp3 deficiency leads to excessive trabeculation, a common feature of CSRP3-related HCM and DCM. We further revealed that csrp3 expression increased in response to different cardiac injuries and was regulated by several signaling pathways vital for heart regeneration. Csrp3 def...
Source: Cellular and Molecular Life Sciences : CMLS - March 31, 2024 Category: Cytology Authors: Shuzhang Liang Yating Zhou Yue Chang Jiayi Li Min Zhang Peng Gao Qi Li Hong Yu Koichi Kawakami Jinmin Ma Ruilin Zhang Source Type: research

Evaluating convolutional neural network-enhanced electrocardiography for hypertrophic cardiomyopathy detection in a specialized cardiovascular setting
AbstractThe efficacy of convolutional neural network (CNN)-enhanced electrocardiography (ECG) in detecting hypertrophic cardiomyopathy (HCM) and dilated HCM (dHCM) remains uncertain in real-world applications. This retrospective study analyzed data from 19,170 patients (including 140 HCM or dHCM) in the Shinken Database (2010 –2017). We evaluated the sensitivity, positive predictive rate (PPR), and F1 score of CNN-enhanced ECG in a ‘‘basic diagnosis’’ model (total disease label) and a ‘‘comprehensive diagnosis’’ model (including disease subtypes). Using all-lead ECG in the"basic diagnosis" model...
Source: Heart and Vessels - March 30, 2024 Category: Cardiology Source Type: research

Investigation of mutation spectrum amongst patients with familial primary cardiomyopathy using targeted NGS in Indian population
J Appl Genet. 2024 Mar 29. doi: 10.1007/s13353-024-00855-2. Online ahead of print.ABSTRACTGenetic cardiomyopathies (CM) are disorders that affect morphology and function of cardiac muscle. Significant number of genes have been implicated in causing the phenotype. It is one of the leading genetic causes of death in young. We performed a study to understand the genetic variants in primary cardiomyopathies in an Indian cohort. Study comprised of 22 probands (13 with family history) representing hypertrophic (n = 10), dilated (n = 7), restrictive (n = 2) and arrhythmogenic ventricular(n = 3) cardiomyopathies. Genomic DNA was t...
Source: J Appl Genet - March 29, 2024 Category: Genetics & Stem Cells Authors: Saroja Mysore Krishnaswamy Gautham Arunachal Kumar Gautam Singh Viji Samuel Thomson Paul George Sudha Rao Sumita Danda Source Type: research

Investigation of mutation spectrum amongst patients with familial primary cardiomyopathy using targeted NGS in Indian population
J Appl Genet. 2024 Mar 29. doi: 10.1007/s13353-024-00855-2. Online ahead of print.ABSTRACTGenetic cardiomyopathies (CM) are disorders that affect morphology and function of cardiac muscle. Significant number of genes have been implicated in causing the phenotype. It is one of the leading genetic causes of death in young. We performed a study to understand the genetic variants in primary cardiomyopathies in an Indian cohort. Study comprised of 22 probands (13 with family history) representing hypertrophic (n = 10), dilated (n = 7), restrictive (n = 2) and arrhythmogenic ventricular(n = 3) cardiomyopathies. Genomic DNA was t...
Source: J Appl Genet - March 29, 2024 Category: Genetics & Stem Cells Authors: Saroja Mysore Krishnaswamy Gautham Arunachal Kumar Gautam Singh Viji Samuel Thomson Paul George Sudha Rao Sumita Danda Source Type: research