EP and the Unusual Cardiomyopathies-Noncompaction: A Disease You Should Share With Your Electrophysiologist (Presenter: David N. Rosenthal, MD) (PDF)

Pdf Summary

LV Noncompaction, also known as LVNC, is a condition that affects the heart. Despite progress in understanding the disease over the past 50 years, there is still confusion surrounding its fundamental identity as a disease. The classification of LVNC varies across different organizations, with some considering it an unclassified cardiomyopathy and others classifying it as a primary genetic cardiomyopathy.<br /><br />The diagnosis of LVNC relies on imaging techniques such as echocardiography (echo) and cardiac magnetic resonance imaging (cMRI). However, there is no consensus on the diagnostic criteria, and different studies have used different criteria to identify LVNC. The clinical presentation of LVNC is often non-specific and dominated by other cardiovascular diseases that may be present, such as congenital heart disease or cardiomyopathy. Common symptoms include heart failure, arrhythmias, and embolic events, although some individuals may remain asymptomatic and are only identified through screening.<br /><br />The prognosis of LVNC varies depending on the population studied. Studies from Australia and Japan have reported variable mortality rates, while a retrospective cohort study showed that the majority of children with isolated LVNC had normal cardiac function. In otherwise healthy adults, LVNC has been detected in a significant percentage of individuals but does not necessarily lead to adverse cardiovascular events.<br /><br />LVNC has also been observed in athletes, suggesting that the severity of trabeculations can vary based on activity levels. Genetic factors play a role in LVNC, with mutations in genes associated with neuromuscular diseases, sarcomere mutations, and channelopathies being implicated. However, the relationship between genetic findings and the course of the disease is still uncertain.<br /><br />It is important to avoid overdiagnosis of LVNC in otherwise healthy individuals, especially athletes. The question of whether LVNC is truly a cardiomyopathy remains open. Managing the associated cardiomyopathy and screening family members is recommended for patients with LVNC.

Meta Tag

Lecture ID 3458

Location Room 203

Presenter David N. Rosenthal, MD

Role Invited Speaker

Session Date and Time May 09, 2019 1:30 PM - 3:00 PM

Session Number S-031

Keywords

LV Noncompaction

LVNC

heart condition

cardiomyopathy

diagnostic criteria

echocardiography

cardiac magnetic resonance imaging

prognosis

genetic factors

overdiagnosis