Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetically inherited disease primarily affecting the right ventricle (RV) but with a significant potential for involvement of the left ventricle (LV). While the RV is the epicenter of pathological changes, the extent and nature of LV involvement significantly impact the overall prognosis and clinical management of ARVC. This article explores the current understanding of LV involvement in ARVC, focusing on global myocardial strain and characteristic features revealed through various imaging modalities. We will examine the implications of LV involvement for disease progression, risk stratification, and therapeutic strategies.
Project Overview: This review aims to synthesize current knowledge regarding left ventricular (LV) involvement in arrhythmogenic right ventricular cardiomyopathy (ARVC), specifically focusing on global myocardial strain analysis as a non-invasive tool to assess LV function and structure. We will analyze the available literature on the prevalence, characteristics, and prognostic implications of LV involvement in ARVC, highlighting the discrepancies and knowledge gaps that need to be addressed through future research.
Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): A Primer
ARVC, also known as arrhythmogenic right ventricular dysplasia (ARVD), is a primary cardiomyopathy characterized by fibrofatty replacement of the myocardium, primarily in the RV. This replacement leads to structural abnormalities, impaired myocardial function, and a heightened risk of life-threatening arrhythmias, including ventricular tachycardia (VT) and sudden cardiac death (SCD). The disease is typically inherited in an autosomal dominant pattern, with mutations in genes encoding desmosomal proteins being the most common genetic cause. However, a significant proportion of ARVC cases remain genetically undiagnosed.
The clinical presentation of ARVC is highly variable, ranging from asymptomatic individuals with subtle abnormalities on electrocardiogram (ECG) to patients experiencing recurrent VT, syncope, and SCD. The diagnosis relies on a combination of clinical features, ECG findings (e.g., epsilon waves, T-wave inversions), imaging studies (e.g., cardiac magnetic resonance imaging [CMR]), and genetic testing.
Left Ventricular Involvement in Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC): The Silent Player
While ARVC is named for its primary right-sided involvement, a significant proportion of patients also exhibit LV involvement, albeit often subtly. This LV involvement can manifest in various ways, including:
* Structural abnormalities: LV dilation, reduced LV ejection fraction (LVEF), and regional wall motion abnormalities are observed in a subset of ARVC patients. These changes can be insidious and may not be apparent in early stages of the disease.
* Electrophysiological abnormalities: LV involvement can contribute to the complex arrhythmogenic substrate, increasing the risk of VT and SCD. This may be due to fibrofatty infiltration within the LV myocardium, affecting conduction pathways and predisposing to re-entrant arrhythmias.
* Myocardial dysfunction: Impaired LV myocardial strain, reflecting reduced contractile function, is a key indicator of LV involvement. This can be assessed non-invasively using CMR and echocardiography.
LV AREM VRW: Global Myocardial Strain Analysis
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