Validation of ACC/AHA and ESC Sudden Cardiac Death Risk Guidelines in Diverse Hypertrophic Cardiomyopathy Cohort:

Murillo Oliveira Antunes1,2, Fabio Fernandes1, Edmundo Arteaga-Fernandez1

  • 1Instituto do Coração, Faculdade de Medicina Universidade de São Paulo, São Paulo, Brazil.

Global Heart
|December 23, 2024
PubMed

Insights

Current sudden cardiac death (SCD) risk guidelines from ACC/AHA and ESC show limited predictive power in Brazilian hypertrophic cardiomyopathy (HCM) patients. Further research is needed to improve SCD prevention strategies for this population.

Area of Science:

  • Cardiology
  • Genetics
  • Public Health

Background:

  • Sudden cardiac death (SCD) is a significant risk in hypertrophic cardiomyopathy (HCM) patients.
  • Existing risk stratification guidelines from the American College of Cardiology/American Heart Association (ACC/AHA) and the European Society of Cardiology (ESC) differ.
  • The comparative effectiveness of these guidelines in diverse populations is not well-established.

Purpose of the Study:

  • To evaluate the performance of the 2020 ACC/AHA and 2014 ESC guidelines for SCD risk stratification.
  • To assess these guidelines in a Brazilian cohort of patients with HCM.

Main Methods:

  • Retrospective cohort study of 187 Brazilian HCM patients.
  • Primary outcome: SCD, aborted cardiac arrest, ventricular fibrillation (VF), sustained ventricular tachycardia (SVT), or appropriate implantable cardioverter-defibrillator (ICD) therapy.
  • Risk prediction models assessed using the C-index and Area Under the Curve (AUC).

Main Results:

  • The 2020 ACC/AHA guidelines identified 56% as high-risk, versus 29% by the 2014 ESC guidelines.
  • The primary outcome occurred in 12% (ACC/AHA high-risk) and 13% (ESC high-risk) of patients.
  • Both guidelines demonstrated low discriminatory power (AUC 0.634 for ACC/AHA, 0.581 for ESC) in this cohort.

Conclusions:

  • The 2020 ACC/AHA and 2014 ESC guidelines have limitations for predicting SCD events in Brazilian HCM patients.
  • Current guidelines may not be optimal for defining implantable cardioverter-defibrillator (ICD) indications in this population.
  • Further research is required to refine risk stratification and optimize SCD prevention strategies for Brazilian HCM patients.
Abstract

Related Concept Videos

Acute Coronary Syndrome III: Diagnostic Studies01:30

Acute Coronary Syndrome III: Diagnostic Studies

Diagnosing acute coronary syndrome or ACS begins with a thorough patient history. Notable symptoms include central, crushing chest pain radiating to the left arm, neck, jaw, or back, along with shortness of breath, sweating (diaphoresis), nausea, vomiting, dizziness, and palpitations.It is crucial to note any history of cardiac illnesses and assess risk factors, including age, gender, smoking, hypertension, diabetes, hyperlipidemia, and a sedentary lifestyle.During physical examination, vital...
500
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...
828
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,...
798
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...
810
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...
957
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...
761