Differentiating Cardiac Sarcoidosis from Arrhythmogenic Right Ventricular Cardiomyopathy: A Systematic Review
Hakan Hasdemir, Abdalla Abshir1, Tolga Sinan Güvenç2
1Acıbadem Atakent Hospital, Cardiology Clinic, Istanbul, Turkey.
Insights
Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) share symptoms, leading to misdiagnosis. Older patients with conduction defects and low ejection fraction may have CS, especially with PET scan findings. ARVC criteria are not useful for differentiating these conditions.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Electrophysiology
Background:
- Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) present similarly, risking misdiagnosis.
- Accurate differentiation is crucial for appropriate therapeutic decisions.
Purpose of the Study:
- To compare clinical and imaging findings between CS and ARVC.
- To identify features that help distinguish CS from ARVC.
Main Methods:
- Systematic review of comparative studies on CS and ARVC published before 2024.
- Literature search in PubMed and Google Scholar.
- Quality assessment using the National Heart, Lung and Blood Institute checklist and PRISMA guidelines.
Main Results:
- Seven studies were included. CS patients were older and had more comorbidities.
- CS showed longer PR interval and QRS duration, and lower left ventricular ejection fraction.
- Septal involvement on cardiac MRI and 18-fluorodeoxyglucose uptake on PET scans were more common in CS. Many CS patients met ARVC criteria.
Conclusions:
- Atrioventricular and intraventricular conduction defects in older patients with low ejection fraction suggest CS, particularly with positive PET scans.
- Current ARVC Task Force criteria (1994, 2010) are insufficient to differentiate CS from ARVC.
Abstract:
Objective: Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) are distinct disorders with different pathophysiologic pathways, but they share similar clinical presentations that could lead to misdiagnosis and inappropriate therapeutic decisions.
Methods:
We searched PubMed and Google Scholar databases and other relevant literature to retrieve comparative studies including CS and ARVC that were published before 2024. The National Heart, Lung and Blood Institute checklist was used for quality assessment and the review was conducted according to the PRISMA guidelines. Three reviewers determined study eligibility and made quality assessments.
Results:
A total of seven studies were included in the review. Patients with CS were older (five of seven studies) and had more comorbidities (two of two studies). PR interval (four of five studies) and QRS duration (four of four studies) were longer in CS. Most studies reported lower left ventricular ejection fraction in CS (five of six studies), and septal involvement on cardiac MRI was more common in CS (two of three studies). 18-Fluorodeoxyglucose uptake on positron emission tomography (PET) scan was seen in up to 90% of CS patients. 62.5%-100% of patients with CS fulfilled 1994 or 2010 International Task Force criteria for ARVC.
Conclusions:
Available evidence suggests that atrioventricular and intraventricular conduction defects in an older (>40 years) patient with low left ventricular ejection fraction should raise suspicion for CS, especially when other supportive findings, such as 18-fluorodeoxyglucose avidity on PET, were present. Neither 1994 nor 2010 ARVC Task Force criteria should be used to discriminate CS from ARVC.
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