Left ventricular noncompaction

R Malla1, R Sharma, B Rauniyar

  • 1Department of Cardiology, Shahid Gangalal National Heart Center, Bansbari, Kathmandu, Nepal.

Insights

Left Ventricular Noncompaction (LVNC) is a rare genetic heart condition. Early diagnosis via echocardiography is crucial, as prognosis is poor with limited treatment options.

Area of Science:

  • Cardiology
  • Genetics
  • Medical Diagnostics

Background:

  • Left Ventricular Noncompaction (LVNC) is an emerging genetic cardiac disease.
  • Clinical signs include heart failure, arrhythmias, embolic events, and in children, facial dysmorphisms and Wolff-Parkinson-White syndrome.
  • Diagnosis is frequently missed due to lack of awareness.

Purpose of the Study:

  • To highlight the importance of recognizing and diagnosing Left Ventricular Noncompaction (LVNC).
  • To emphasize the role of echocardiography in LVNC diagnosis.
  • To underscore the need for screening relatives of affected individuals.

Main Methods:

  • Echocardiography is the gold standard for diagnosing LVNC.
  • Clinical presentation and familial association are key diagnostic considerations.

Main Results:

  • LVNC presents with heart failure, arrhythmias, and embolic events.
  • Prognosis is poor for patients with reduced systolic function.
  • Echocardiography is essential for accurate diagnosis.

Conclusions:

  • Left Ventricular Noncompaction (LVNC) requires greater clinical awareness.
  • Screening first-degree relatives of LVNC patients is recommended due to its genetic nature.
  • Early echocardiographic diagnosis can potentially improve patient outcomes.

Related Concept Videos

Imbalances in Cardiac Output01:26

Imbalances in Cardiac Output

The heart's primary function is to pump blood throughout the body, maintaining a balance between blood sent out (cardiac output) and blood returning (venous return). If this balance is disrupted, it can result in congestive heart failure (CHF), a severe condition where the heart becomes an inefficient pump, leading to inadequate blood circulation.
CHF can occur due to the failure of either side of the heart. Left-side failure leads to pulmonary congestion—the right side continues to send blood...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

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...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

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...
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

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...
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

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,...