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Related Concept Videos

Dysrhythmias IV: Characteristics of Bradyarrhythmias01:18

Dysrhythmias IV: Characteristics of Bradyarrhythmias

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Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
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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 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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Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

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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...
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Dysrhythmias I: Introduction01:15

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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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Mechanism of Cardiac Arrhythmias01:28

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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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Seckel syndrome presenting with complete heart block.

Mostafa Abohelwa1, Mohamed Elmassry1, Marina Iskandir2

  • 1Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, Texas.

Proceedings (Baylor University. Medical Center)
|May 6, 2021
PubMed
Summary
This summary is machine-generated.

Seckel syndrome, a rare genetic disorder, is linked to cardiac issues. This case highlights complete heart block in a Seckel syndrome patient, suggesting a potential association needing further investigation.

Keywords:
Case reportSeckel syndromecomplete heart block

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Area of Science:

  • Genetics
  • Cardiology
  • Pediatrics

Background:

  • Seckel syndrome is a rare autosomal recessive disorder associated with distinct facial features ('bird-headed dwarfism').
  • Cardiac anomalies are a potential, though infrequently reported, comorbidity in Seckel syndrome.
  • Epilepsy is also a recognized neurological complication in some individuals with Seckel syndrome.

Observation:

  • A 21-year-old woman diagnosed with Seckel syndrome and epilepsy presented with status epilepticus.
  • The patient exhibited hypotension and bradycardia.
  • Electrocardiogram revealed complete heart block, necessitating pacemaker insertion.

Findings:

  • This report details the third documented case of complete heart block in a patient with Seckel syndrome.
  • The cardiac anomaly occurred in the context of status epilepticus and associated hemodynamic instability.
  • Despite initial attempts with a transcutaneous pacer, a permanent pacemaker was ultimately required.

Implications:

  • The recurrent association of complete heart block in Seckel syndrome patients warrants increased clinical vigilance for cardiac evaluation.
  • Further research is needed to elucidate the underlying mechanisms connecting Seckel syndrome and cardiac conduction defects.
  • Early identification and management of cardiac anomalies may improve outcomes for individuals with Seckel syndrome.