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Related Concept Videos

Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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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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Disturbances in Heart Rhythm01:29

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Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
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Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

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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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ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias01:25

ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias

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Arrhythmia is a condition characterized by an irregular heart rhythm, with ECG changes that differ based on its origin and nature. The types of arrhythmias discussed below include atrial, junctional, and ventricular arrhythmias.Atrial ArrhythmiasPremature Atrial Complexes (PACs): PACs are early atrial beats caused by stress, caffeine, alcohol, electrolyte imbalances, hypoxia, hyperthyroidism, or certain medications (e.g., bronchodilators and decongestants). The ECG shows early P waves with an...
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Cardiac Action Potential01:30

Cardiac Action Potential

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Cardiac action potentials are essential for proper heart function, enabling the rhythmic contractions needed for adequate blood circulation. Nodal cells and Purkinje fibers, specialized for electrical conduction, generate these action potentials.
The cardiac action potential process involves a series of phases characterized by the movement of ions across the cardiac cell membranes, leading to the depolarization and repolarization of the cardiac myocytes.
Ionic Basis of Cardiac Action Potentials
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Mechanism of Cardiac Arrhythmias01:28

Mechanism of Cardiac Arrhythmias

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Arrhythmias are irregular heart rhythms occurring when the heart's electrical impulses become abnormal. These disturbances can lead to various symptoms, depending on their severity and the underlying cause. Some common factors contributing to arrhythmias include hypoxia, ischemia, electrolyte imbalances, excessive catecholamine exposure, drug toxicity, and muscle overstretching. Arrhythmias can be classified into two main types based on the rate and site of origin of abnormal heart rhythms.
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Related Experiment Video

Updated: Sep 10, 2025

Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples
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Electrical Storm in Patients With Hypertrophic Cardiomyopathy.

Pierre Groussin1, Melvyn Dezecot1, Donovan Decaudin1

  • 1University of Rennes, Centre Hospitalier Universitaire (CHU) Rennes, INSERM, LTSI-UMR 1099, Rennes, France.

JACC. Clinical Electrophysiology
|August 21, 2025
PubMed
Summary

Electrical storm (ES) in hypertrophic cardiomyopathy (HCM) patients often requires antiarrhythmic drugs but frequently leads to ventricular arrhythmia (VA) recurrences. Long-term outcomes are poor, with significant mortality within 18 months.

Keywords:
catheter ablationelectrical stormhypertrophic cardiomyopathyventricular arrhythmia

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Area of Science:

  • Cardiology
  • Electrophysiology
  • Cardiovascular Medicine

Background:

  • Electrical storm (ES) management in hypertrophic cardiomyopathy (HCM) is not well-defined.
  • Patients with HCM are at high risk for ventricular arrhythmias (VAs).

Purpose of the Study:

  • To analyze the acute management of ES in HCM patients.
  • To evaluate in-hospital and long-term outcomes for HCM patients experiencing ES.

Main Methods:

  • Retrospective multicenter study of 23 HCM patients with ES (2017-2023).
  • Data collected on demographics, prior VA history, treatments (antiarrhythmic drugs, radiofrequency catheter ablation), and outcomes.

Main Results:

  • Most VAs were monomorphic ventricular tachycardias; amiodarone and beta-blockers were commonly used.
  • Radiofrequency catheter ablation was performed in 44% of patients, but was not associated with better outcomes.
  • High rates of VA recurrence (30%) and mortality (9% in-hospital, 18% by 18 months) were observed.

Conclusions:

  • ES in HCM patients often requires antiarrhythmic drugs but is associated with frequent VA recurrences.
  • Long-term outcomes for HCM patients with ES are poor, with significant mortality.
  • Further research is needed to optimize radiofrequency ablation outcomes in this population.