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Related Concept Videos

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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Antiarrhythmic Drugs: Class III Agents as Potassium Channel Blockers01:12

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Class III antiarrhythmic drugs are a group of medications that can prolong action potentials in the heart. They achieve this by blocking potassium channels or enhancing inward currents from sodium channels. However, these drugs have a unique property of "reverse use-dependence," which is most pronounced at slower heart rates and can lead to torsades de pointes—a specific type of arrhythmia. However, it is essential to note that excessive QT interval prolongation—a measure of...
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Dysrhythmias V: Evaluating Dysrhythmias01:30

Dysrhythmias V: Evaluating Dysrhythmias

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Dysrhythmias, also known as arrhythmias, are disturbances in the heart's rhythm that range from benign to life-threatening. A thorough evaluation is crucial for appropriate management and involves a comprehensive medical history, physical examination, and various diagnostic tests.Medical HistorySymptoms: Collect detailed information on palpitations, dizziness, syncope, chest pain, and fatigue. Note their onset, frequency, and triggers.Previous Cardiac Issues: Document any history of heart...
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Dysrhythmias I: Introduction01:15

Dysrhythmias I: Introduction

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Dysrhythmias refers to abnormalities in the heart's rhythm. They result from disruptions in the heart's electrical conduction system, which includes the sinoatrial(SA)node, atrioventricular(AV) node, the bundle of His, bundle branches, and Purkinje fibers.Definition and PathophysiologyDysrhythmias result from disorders of impulse formation, impulse conduction, or both. The heart contains specialized cells in the sinoatrial node, atrioventricular node, and the bundle of His and Purkinje fibers...
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Dysrhythmias II: Classification of Tachyarrhythmias01:28

Dysrhythmias II: Classification of Tachyarrhythmias

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Tachyarrhythmias are a type of dysrhythmia where the heart rate exceeds 100 beats per minute. Here are some common types of tachyarrhythmias:Sinus TachycardiaSinus tachycardia originates from increased impulses from the sinus node, leading to an elevated heart rate. It is often triggered by stress, fever, or exercise.Patients may experience palpitations, a sensation of a racing heart, dizziness, and chest discomfort.Causes and Risk Factors: Common causes include physical exertion, emotional...
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Dysrhythmias VI: Management of Dysrhythmias01:25

Dysrhythmias VI: Management of Dysrhythmias

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Dysrhythmia management involves a multifaceted approach, incorporating pharmacological treatments, medical procedures, surgical interventions, lifestyle modifications, and patient education.Pharmacological ManagementAntiarrhythmic Drugs:Class I (Sodium Channel Blockers): This class includes quinidine and procainamide, which reduce the speed of impulse conduction in the heart, stabilize the cardiac membrane, and control arrhythmias. Quinidine and procainamide are Class IA agents that prolong the...
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Related Experiment Video

Updated: Sep 7, 2025

Determining the Likelihood of Variant Pathogenicity Using Amino Acid-level Signal-to-Noise Analysis of Genetic Variation
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CONGENITAL LONG QT SYNDROME: A SYSTEMATIC REVIEW.

Edvard Galić1, Petar Bešlić1, Paula Kilić1

  • 11School of Medicine, University of Zagreb, Zagreb, Croatia; 2Sveti Duh University Hospital, Zagreb, Croatia; 3Merkur University Hospital, Zagreb, Croatia.

Acta Clinica Croatica
|June 23, 2022
PubMed
Summary

Congenital long QT syndrome (LQTS) is a myocardial repolarization disorder affecting 1:2000 individuals, potentially causing sudden cardiac death. Early diagnosis and beta-blocker treatment are crucial for managing this genetic condition.

Keywords:
Congenital long QT syndromeMonogenic mutationSudden cardiac deathSyncopeVentricular arrhythmia

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Area of Science:

  • Cardiology
  • Genetics
  • Electrophysiology

Background:

  • Congenital long QT syndrome (LQTS) is a genetic disorder of myocardial repolarization.
  • Characterized by a prolonged QT interval on ECG, it predisposes individuals to ventricular arrhythmias and sudden cardiac death.
  • While 17 subtypes exist, LQT1, LQT2, and LQT3 are most prevalent.

Purpose of the Study:

  • To review the current understanding of congenital LQTS, including its prevalence, clinical manifestations, diagnostic approaches, and management strategies.

Main Methods:

  • Review of existing literature on congenital LQTS.
  • Analysis of diagnostic criteria including ECG, family history, Schwartz score, and genetic testing.
  • Evaluation of current treatment modalities.

Main Results:

  • The estimated prevalence of congenital LQTS is approximately 1:2000.
  • Clinical presentation varies, with some patients remaining asymptomatic while others experience syncope, aborted cardiac arrest, or sudden cardiac death.
  • Diagnosis involves ECG, family history, Schwartz score, ambulatory monitoring, stress testing, and genetic testing.
  • Beta-blockers are the first-line treatment for both symptomatic and asymptomatic patients.

Conclusions:

  • Congenital LQTS is a significant genetic cardiac disorder requiring comprehensive diagnostic evaluation.
  • Early identification and management, primarily with beta-blockers, are essential for preventing adverse cardiovascular events.