Familial Hypertrophic Cardiomyopathy: Late Potentials and Other Prognostic Markers

Ândrea Chaves-Markman1, Manuel Markman2, Marcelo Antônio O Santos-Veloso3

  • 1Cardiology, Rarus - A Rare Disease Service, Recife, BES.

Cureus
|February 12, 2020
PubMed

Insights

This study found no link between late potentials and a family history of sudden death, syncope, or arrhythmias in hypertrophic cardiomyopathy patients. These findings are crucial for understanding hypertrophic cardiomyopathy risks.

Area of Science:

  • Cardiology
  • Genetics
  • Sudden Cardiac Death

Background:

  • Familial hypertrophic cardiomyopathy (HCM) is a genetic disorder and a leading cause of sudden cardiac death (SCD) in young adults, particularly athletes.
  • HCM diagnosis relies on echocardiogram criteria, but risk stratification for adverse events remains critical.

Purpose of the Study:

  • To investigate the association between late potentials and a family history of SCD, syncope, and complex ventricular arrhythmias in patients with HCM.
  • To identify potential predictors of adverse cardiac events in HCM patients.

Main Methods:

  • A case series study involving 22 patients diagnosed with HCM via transthoracic echocardiogram.
  • Exclusion criteria included pacemaker use, right bundle branch block, cardiac transplant, and inability to undergo examinations.
  • Data collected included type of hypertrophy, family history, and presence of late potentials and arrhythmias.

Main Results:

  • Asymmetric septal hypertrophy was the most common type (73%) in the study cohort.
  • A significant proportion of patients had a positive family history: 63% for HCM, 55% for SCD, and 23% for syncope.
  • Late potentials were detected in 23% of patients, and complex ventricular arrhythmias in 14%.

Conclusions:

  • The presence of late potentials was not statistically associated with a family history of SCD, syncope, or complex ventricular arrhythmias in this cohort of HCM patients.
  • Further research is needed to elucidate the prognostic value of late potentials in HCM and their relationship with genetic predisposition and clinical events.

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...
304
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,...
373
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...
252
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...
424
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
158
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...
381