The Role of Magnetic Resonance Imaging ın Diagnosis of Arrhythmogenic Right Ventricle Dysplasia

Year 2019, Volume: 7 Issue: 2, 110 - 117, 27.08.2019

Hadi Sasani Memduh Dursun Ahmet Kaya Bilge

Abstract

Objectives

Arrhythmogenic right ventricle dysplasia and cardiomyopathy (ARVD/C) occurs due to fibrofatty tissue infiltration in the ventricle myocardium. It is characterized with structural and functional abnormalities, mostly involving the right ventricle and the anterolateral part or the septum. Although the etiology has not been completely understood, it is responsible for the sudden death in early adolescents and athletics. The objective of this article is to demonstrate the accuracy of ARVD/C findings in clinically confirmed ARVD/C cases by cardiac magnetic resonance imaging (MRI).  

Material-Methods

Cardiac MRI findings of clinically diagnosed 23 ARVD/C patients (18 male, 5 female; mean age: 38.2; SD: ± 13) were evaluated retrospectively.  In four chamber (4CH) cine images at both end-diastolic and end-systolic phases of cardiac cycle, the endocardial diameters of both atria and ventricles were measured.

Results

Findings were found as following: right ventricular dilation at end-diastolic phase (> 42 mm: 78.2%),  right atrial dilation (> 41 mm: 78.2%),  myocardial fatty replacement (74%) and left ventricular dilation (> 42 mm: 74%), ventricle wall motion disorders on Cine MRI (43.5%), decrease in myocardial contractions (30.4%), post-contrast enhancement due to fibrosis (21.7%), right ventricle outflow tract dilation (21.7%), trabeculation (17.4%). Tricuspid insufficiency was 4.3% and Ebstain’s anomaly was found as 4.3%. 

Conclusion

MRI is useful for the diagnosis of ARVD/C. In the current study, the most frequent MRI findings  were right ventricular and atrial dilation, myocardial fatty infiltration and left ventricular dilation respectively. The presence of fibrosis is significant for the diagnosis of ARVD/C. The left ventricle involvement is rare and may occur in ARVD/C, therefore, the fatty replacement areas of left ventricle should be evaluated carefully.

Keywords

Arrhythmogenic right ventricle dysplasia, Myocardium, Magnetic resonance imaging

The Role of Magnetic Resonance Imaging in Diagnosis of Arrhythmogenic Right Ventricle Dysplasia

Abstract

Giriş

Aritmojenik sağ ventrikül displazisi ve kardiyomiyopati (ARVD/C)  ventrikül miyokardındaki fibröz ve yağ dokusunun infiltrasyonuna bağlı olarak ortaya çıkar. Çoğunlukla sağ ventrikül ve anterolateral kısmı veya septumu içeren yapısal ve fonksiyonel anormallikler ile karakterizedir.  Etiyolojisi tam olarak anlaşılmamış olmasına rağmen, erken ergenlik döneminde ve atletlerdeki ani ölümlerden sorumludur. 

Bu çalışmanın amacı, klinik olarak teyit edilmiş ARVD/C olgularında kardiyak manyetik rezonans görüntüleme (MRG) bulguları ile doğruluğunu göstermektir.

Gereç ve Yöntem

Bu çalışmada, 23 (18 Erkek, 5 Kadın; yaş ortalaması 38.2 SD: ± 13) ARVD tanılı hastanın kardiyak MRG bulguları retrospektif olarak değerlendirildi. Kardiyak siklusun diyastol sonu ve sistol sonu fazlarda dört boşluk (4CH) görüntülerde , atrium ve ventriküllerin endokardiyal çapları ölçüldü. 

Bulgular

Elde edilen sonuçlar sırasıyla end-diyastolik fazda sağ ventrikül dilatasyonu (42 mm˂ %78.2) ve sağ atrium dilatasyonu (41 mm˂ %78.2) olup, bunu takiben miyokardda yağ infiltrasyonu (%74), sol ventrikül dilatasyonu (42 mm˂ %74), Cine MRG incelemede paradoks hareket ve duvar hareket bozuklukları (%43.5), miyokard kontraksiyonunda azlama (%30.4), post-kontrast fibrozis lehine tutulum (%21.7), RVOT dilatasyonu (%21.7), trabekülasyon artışı (%17.4) ve eşlik eden diğer anomaliler (triküspit kapak yetersizliği: %4.3 ve ebstain anomalisi: %4.3) şeklinde bulundu.

Sonuç

Kardiyak MRG, ARVD/C tanı için vazgeçilmez bir görüntüleme modalitesidir. Bu çalışmada en sık MRG bulguları sırasıyla sağ ventrikül ve atriyal dilatasyon, miyokard yağ infiltrasyonu ve sol ventrikül dilatasyonu olarak bulundu. Fibrozis varlığı ARVD/C tanısı için önemlidir. Sol ventrikül tutulumu nadir olup, detaylı bir şekilde değerlendirlimelidir.

Keywords

Aritmojenik sağ ventrikül displazisi, Miyokard, Manyetik rezonans görüntüleme

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