Related Experiment Video

Updated: Oct 8, 2025

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
03:45

Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model

Published on: August 8, 2022

3.8K

EACVI survey on hypertrophic cardiomyopathy

Tomaz Podlesnikar1,2, Nuno Cardim3,4, Nina Ajmone Marsan5

  • 1Department of Cardiac Surgery, University Medical Centre Maribor, Ljubljanska ulica 5, 2000 Maribor, Slovenia.

European Heart Journal. Cardiovascular Imaging
|December 27, 2021
PubMed

Insights

Global survey reveals most centers follow European guidelines for hypertrophic cardiomyopathy (HCM) diagnosis and management. Emphasis on provocation maneuvers and exercise stress echocardiography for LV outflow obstruction is needed, with additional sudden cardiac death (SCD) risk markers potentially updating recommendations.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Clinical Practice

Background:

  • Hypertrophic cardiomyopathy (HCM) management requires standardized assessment and follow-up.
  • Global practices for HCM patient care vary, necessitating evaluation of current approaches.

Purpose of the Study:

  • To assess current global practices for the diagnosis and management of hypertrophic cardiomyopathy (HCM).
  • To evaluate the utilization of imaging modalities and risk stratification tools in HCM care.

Main Methods:

  • A global survey was conducted by the EACVI Scientific Initiatives Committee.
  • Responses from 213 centers across 38 countries detailed patient management, imaging techniques (echocardiography, CMR), and risk assessment for sudden cardiac death (SCD).

Main Results:

  • Most centers (57%) manage HCM patients in general cardiology clinics, while 40% use specialized clinics.
  • Echocardiography is primary, with CMR as a complementary tool. Bedside provocation maneuvers (49%) and exercise stress echocardiography (55%) are used for LV obstruction assessment.
  • The HCM Risk-SCD score is widely used, but additional markers like late gadolinium enhancement and LV apical aneurysm are also employed for ICD decisions. Regular imaging follow-up (99%) is standard.

Conclusions:

  • The majority of centers adhere to European guidelines for HCM diagnosis and management.
  • Enhanced emphasis on provocation maneuvers and exercise stress echocardiography is recommended for diagnosing LV outflow obstruction.
  • The use of additional SCD risk markers suggests a potential need to update current European recommendations.
Abstract

Keywords:
EACVIhypertrophic cardiomyopathymultimodality imagingsudden cardiac deathsurvey

More Related Videos

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix10:21

Scanning Electron Microscopy of Macerated Tissue to Visualize the Extracellular Matrix

Published on: June 14, 2016

10.2K
An Approach to Study Shape-Dependent Transcriptomics at a Single Cell Level06:02

An Approach to Study Shape-Dependent Transcriptomics at a Single Cell Level

Published on: November 2, 2020

5.8K

Related Concept Videos

Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
86
Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
60
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
60
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
115
Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies01:22

Rheumatic Heart Disease II: Clinical Manifestations and Diagnostic Studies

The key clinical manifestations of Rheumatic heart disease (RHD) include several distinct cardiac symptoms.Carditis, a hallmark of acute rheumatic fever, involves inflammation of the heart's endocardium, myocardium, and pericardium. Chronic RHD often results from recurrent episodes of carditis. Its symptoms include the following:Murmurs are caused by valvular damage, especially to the mitral and aortic valves. Mitral stenosis or regurgitation is common, with characteristic heart murmurs...
109
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
87