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Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

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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...
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Cardiomyopathy I: Introduction and Classification01:25

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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...
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Cardiomyopathy II: Dilated Cardiomyopathy01:30

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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,...
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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...
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Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

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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...
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Effective preventive measures for coronary artery disease (CAD) focus on controlling modifiable risk factors, including cholesterol abnormalities and lifestyle changes.Cholesterol ManagementFirst, the Mediterranean diet and the American Heart Association advocate for maintaining low-density lipoprotein (LDL) cholesterol levels below 100 mg/dL, with a more stringent recommendation of below 70 mg/dL for individuals at high risk. LDL cholesterol, often termed "bad cholesterol," can lead to the...
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Updated: Aug 26, 2025

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Programmed Ventricular Stimulation as an Additional Primary Prevention Risk Stratification Tool in Arrhythmogenic

Alessio Gasperetti1, Richard T Carrick1, Sarah Costa2

  • 1Department of Medicine, Division of Cardiology, Johns Hopkins Hospital, Baltimore, MD (A.G., R.T.C., C. Tichnell, B.M., H.T., H.C., C.A.J.).

Circulation
|October 7, 2022
PubMed
Summary

Programmed ventricular stimulation (PVS) improves risk prediction for ventricular arrhythmias (VA) in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. PVS adds significant value beyond existing risk calculators, especially for low-to-intermediate risk individuals.

Keywords:
arrhythmogenic right ventricular cardiomyopathydefibrillator, implantableelectrophysiological techniques, cardiacrisk assessmentsudden cardiac death

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Area of Science:

  • Cardiology
  • Electrophysiology
  • Genetics

Background:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic heart condition.
  • Ventricular arrhythmias (VA) are a major risk in ARVC patients.
  • A novel risk calculator for VA in ARVC exists, but its limitations are unknown.

Purpose of the Study:

  • To determine if programmed ventricular stimulation (PVS) offers additional prognostic value for VA risk in ARVC patients.
  • To assess the combined performance of a risk calculator and PVS in predicting VA.
  • To evaluate PVS's role in primary prevention of VA in ARVC.

Main Methods:

  • Retrospective analysis of 288 ARVC patients from 6 international registries.
  • Patients had a definite ARVC diagnosis, no prior sustained VA, and baseline PVS.
  • Assessed calculator-predicted risk and PVS inducibility for sustained VA over 5-year follow-up.

Main Results:

  • PVS identified inducible ventricular tachycardia in 47.6% of patients.
  • Sustained VA occurred in 60.6% of PVS-positive vs. 24.5% of PVS-negative patients (P<0.001).
  • PVS remained an independent predictor of VA, improving risk prediction accuracy (C-statistic 0.75 vs. 0.72).

Conclusions:

  • Programmed ventricular stimulation significantly enhances VA risk stratification in ARVC patients.
  • PVS provides added prognostic value beyond existing risk calculators.
  • PVS is particularly beneficial for refining risk assessment in low-to-intermediate risk ARVC patients.