Phenotype-modifying Factors in Hypertrophic Cardiomyopathy

Publication date: Available online 26 May 2018 Source:Revista Española de Cardiología (English Edition) Author(s): Javier Limeres Freire
Source: Revista Espanola de Cardiologia - Category: Cardiology Source Type: research

Related Links:

Publication date: Available online 19 November 2019Source: Pharmacological ResearchAuthor(s): Nebojša Mujović, Dobromir Dobrev, Milan Marinković, Vincenzo Russo, Tatjana S. PotparaAbstractAmiodarone is an iodinated benzofuran derivative, a highly lipophilic drug with unpredictable pharmacokinetics. Although originally classified as a class III agent due to its ability to prolong refractoriness in cardiac regions and prevent/terminate re-entry, amiodarone shows antiarrhythmic properties of all four antiarrhythmic drug classes. Amiodarone is a potent coronary and peripheral vasodilator and can be safely used in pati...
Source: Pharmacological Research - Category: Drugs & Pharmacology Source Type: research
Inherited heart diseases include numerous conditions, from the more prevalent hypertrophic cardiomyopathy (HCM) and familial hypercholesterolaemia (FH), to the comparatively less common inherited arrhythmia syndromes, such as long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT) and Brugada syndrome (BrS). Genetic testing has evolved rapidly over the last decade and is now considered a mainstream component of clinical management of inherited heart diseases. Cardiac manifestations can also be part of wider syndromes, and genetic testing can play a critical role in clarifying the underlying ae...
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Tags: Review Source Type: research
Authors: Du Y, Wang Y, Han X, Feng Z, Ma A Abstract Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiovascular diseases and possesses a high risk for sudden cardiac death. Although mutations in more than 20 genes have been reported to be associated with HCM thus far, the genetic backgrounds of most HCM patients are not fully understood. We performed a genetic analysis in a Chinese family that presented with HCM using next-generation sequencing (NGS). Clinical data, family histories, and blood samples were collected from the proband and family members. Five patients showed typical clinical ...
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
Authors: Miura S, Okada T, Kuroda H, Yamaguchi K, Yoshitomi H, Watanabe N, Endo A, Tanabe K Abstract Hypertrophic cardiomyopathy with left ventricular (LV) mid-cavity obstruction and LV apical aneurysm is associated with high morbidity and mortality rates. However, consensus is lacking on the treatment modality for LV mid-cavity obstruction and LV apical aneurysm. Here, we report a case of reduced LV mid-cavity pressure gradient and symptoms, treated using permanent pacing. The effect of permanent pacing on pressure gradient and symptoms lasted for 4 years. As pacing is relatively non-invasive compared to surgical ...
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
PMID: 31738597 [PubMed - in process]
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
In this issue of the International Journal of Cardiology, Dr. Maurizi and colleagues report on the higher than expected prevalence of cardiac amyloidosis in patients undergoing evaluation for hypertrophic cardiomyopathy (HCM) in a tertiary referral center [1]. This carefully performed analysis highlights the clinical overlap of cardiac amyloidosis in a substantial percentage (a total of 9%) of patients who display a phenotype of hypertrophic cardiomyopathy and emphasizes the need to consider cardiac amyloidosis in any patient with ventricular hypertrophy without obvious inciting factor or known hypertrophic mutation.
Source: International Journal of Cardiology - Category: Cardiology Authors: Tags: Editorial Source Type: research
Condition:   Cardiomyopathy, Hypertrophic Interventions:   Drug: LCZ696;   Drug: Placebo Sponsor:   Novartis Pharmaceuticals Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Hypertrophic cardiomyopathy (HCM) is a common genetic disorder characterized by left ventricular hypertrophy and cardiac hyper-contractility. Mutations in the β-cardiac myosin heavy chain gene (β-MyHC) are a major cause of HCM, but the specific mechanistic changes to myosin function that lead to this disease remain incompletely understood. Predicting the severity of any β-MyHC mutation is hindered by a lack of detailed examinations at the molecular level. Moreover, because HCM can take ≥20 years to develop, the severity of the mutations must be somewhat subtle. We hypothesized that mutations that result i...
Source: Journal of Biological Chemistry - Category: Chemistry Authors: Tags: Molecular Bases of Disease Source Type: research
Resolution of left ventricular outflow tract (LVOT) obstruction predicts symptom relief postmyectomy. Intraoperative measurement of LVOT gradients thus is essential for surgical guidance. We hypothesized that (1) hypertrophic cardiomyopathy patients have lower LVOT gradients when measured intraoperatively with transesophageal echocardiography (TEE) compared with preoperative measurements with transthoracic echocardiography (TTE) and that (2) intraoperative provocative testing can help evaluate the adequacy of surgical resection.
Source: Journal of the American Society of Echocardiography - Category: Cardiology Authors: Source Type: research
ConclusionMachine learning-based texture analysis of late gadolinium-chelate enhancement-positive areas is a promising tool for the classification of hypertrophic cardiomyopathy patients with and without VT.
Source: Diagnostic and Interventional Imaging - Category: Radiology Source Type: research
More News: Cardiology | Cardiomyopathy | Heart | Hypertrophic Cardiomyopathy