Left ventricular non-compaction (LVNC) is a rare cardiomyopathy that affects the left ventricle, characterized by an abnormally thick sponge-like, trabecular layer and a thinner, compacted myocardial layer. This condition, also known as isolated left ventricular noncompaction (ILVNC), poses challenges in diagnosis and management due to its variability in clinical presentation and potential complications. In this article, we will delve into the various aspects of LVNC, including its pathophysiology, clinical manifestations, diagnosis, and treatment options.
Pathophysiology of LVNC:
The pathophysiology of LVNC involves an arrest in the normal compaction process of the myocardium during embryogenesis, leading to the persistence of trabeculations in the left ventricle. This results in a two-layered myocardium with prominent trabeculations and deep intertrabecular recesses. The exact etiology of LVNC remains unclear, with genetic factors believed to play a significant role in its development. Mutations in genes encoding proteins involved in myocardial development and function, such as MYH7, MYBPC3, and TTN, have been associated with familial forms of LVNC.
Clinical Manifestations of LVNC:
Patients with LVNC can present with a wide range of symptoms, including heart failure, arrhythmias, thromboembolic events, and sudden cardiac death. The clinical presentation of LVNC can vary from asymptomatic individuals to those with severe cardiac dysfunction. Common symptoms of LVNC include dyspnea, fatigue, palpitations, and chest pain. Physical examination may reveal signs of heart failure, such as elevated jugular venous pressure, peripheral edema, and crackles on lung auscultation.
Diagnosis of LVNC:
Diagnosing LVNC can be challenging due to its variable presentation and overlap with other cardiomyopathies. Echocardiography is the primary imaging modality used to diagnose LVNC, with the characteristic finding of excessive trabeculations and deep intertrabecular recesses in the left ventricle. Additional imaging modalities, such as cardiac magnetic resonance imaging (MRI) and computed tomography (CT) angiography, can provide further insights into the morphology and function of the left ventricle. Genetic testing may also be recommended in cases of familial LVNC or when there is a high index of suspicion for an underlying genetic cause.
Treatment of LVNC:
The management of LVNC aims to alleviate symptoms, prevent complications, and improve outcomes. Treatment strategies for LVNC include pharmacological therapy, device therapy, and surgical interventions. Medications such as beta-blockers, angiotensin-converting enzyme (ACE) inhibitors, and diuretics are commonly used to manage heart failure symptoms and reduce the risk of arrhythmias. In patients with a history of thromboembolic events, anticoagulation therapy may be indicated to prevent further events. Device therapy, including implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy (CRT), may be considered in patients at high risk of sudden cardiac death or with significant arrhythmias. In severe cases of LVNC with refractory heart failure, heart transplantation may be considered as a last resort.
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