Related Experiment Video
Updated: Jan 21, 2026

16:49
Multifocal Electroretinograms
Published on: December 4, 2011
18.8K
SCN5A R814W-Associated Multifocal Ventricular Ectopy and Dilated Cardiomyopathy: A Treatable Channelopathy
Marta Catarina Bernardo1, Isabel Martins Moreira1, José Pedro Guimarães1
1Cardiology Department, Local Health Unit of Trás-os-Montes e Alto-Douro, Vila Real, Portugal.
Journal of Cardiovascular Electrophysiology
|January 20, 2026
Summary
Multifocal ectopic Purkinje-related premature contractions (MEPPC), a syndrome linked to SCN5A variants, can cause heart failure. This case highlights quinidine
Area of Science:
- Cardiology
- Genetics
- Electrophysiology
Background:
- Multifocal ectopic Purkinje-related premature contractions (MEPPC) is a genetic syndrome.
- It is characterized by SCN5A gain-of-function variants, multifocal ventricular ectopy, and dilated cardiomyopathy (DCM).
Related Concept Videos
Cardiomyopathy II: Dilated Cardiomyopathy
489
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,...
489
Cardiomyopathy IV: Restrictive Cardiomyopathy
481
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...
481
Cardiomyopathy III: Hypertrophic Cardiomyopathy
418
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...
418
Cardiomyopathy I: Introduction and Classification
517
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...
517
Cardiomyopathy VI: Nursing Management
318
Assessment: Nursing management of patients with cardiomyopathy begins with a thorough assessment of the patient's history, including a family history of cardiomyopathy or sudden cardiac death, personal history of heart disease, hypertension, diabetes, and any alcohol consumption or drug use.During the physical examination, assess vital signs, look for signs of heart failure (such as edema, jugular venous distention, and cyanosis), auscultate for abnormal heart sounds (like murmurs and gallops),...
318
Cardiomyopathy V: Interprofessional Care
353
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...
353

