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

Yıl 2019, Cilt: 7 Sayı: 2, 110 - 117, 27.08.2019

Hadi Sasani , Memduh Dursun Ahmet Kaya Bilge

Öz

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.

Anahtar Kelimeler

Arrhythmogenic right ventricle dysplasia, Myocardium, Magnetic resonance imaging

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

Öz

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.

Anahtar Kelimeler

Aritmojenik sağ ventrikül displazisi, Miyokard, Manyetik rezonans görüntüleme

Kaynakça

• Corrado D, Fontaine G, Marcus FI, et al. Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Circulation. 2000;101:E101–E106.
• Kayser HWm, van der Wall EE, Sivananthan MU, Plein S, Bloomer TN, Roos AD. Diagnosis of Arrhythmogenic Right Ventricular Dysplasia: A Review. RadioGraphics. 2002;22:639–50.
• Gemayel C, Pelliccia A, Thompson PD. Arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2001;38:1773–81.
• Corrado D, Basso C, Thiene G. Arrhythmogenic right ventricular cardiomyopathy: diagnosis, prognosis, and treatment. Heart. 2000;83:588–95.
• Peters S, Peters H, Thierfelder L. Risk stratification of sudden cardiac death and malignant ventricular arrhythmias in right ventricular dysplasia- cardiomyopathy. Int J Cardiol. 1999;71:243–50.
• McKenna WJ, Thiene G, Nava A, Fontaliran F, Blomstrom-Lundqvist C, Fontaine G, et al. Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Br Heart J. 1994;71:215–18.
• Cesare E. MRI assessment of right ventricular dysplasia. Eur Radiol (2003); 13:1387–1393.
• Ferrari VA, Scott CH. Arrhythmogenic right ventricular dysplasia. Time for a new look. J Cardiovasc Electrophysiol. 2003;14:483–84.
• Dalal D, Nasir K, Bomma C, Prakasa K, Tandri H, Piccini J, et al. Arrhythmogenic right ventricular dysplasia: a United States experience. Circulation. 2005;112:3823–32.
• Murphy DT, Shine SC, Cradock A, Galvin JM, Keelan ET, Murray JG. Cardiac MRI in Arrhythmogenic Right Ventricular Cardiomyopathy. AJR. 2010;194:299–306.
• Tandri H, Bomma C, Calkins H, Bluemke DA. Magnetic resonance and computed tomography imaging of arrhythmogenic right ventricular dysplasia. J Magn Reson Imaging. 2004;19(6):848-58.
• Marcus F, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, et al. Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/ Dysplasia (ARVC/D). Circulation. 2010;121:1533-41. doi:10.1161/CIRCULATIONAHA.108.840827.
• Calkins H. Arrhythmogenic right ventricular dysplasia. Trans Am Clin Climatol Assoc. 2008;119:273–88.
• Friedrich MG. Magnetic resonance imaging in cardiomyopathies. J Cardiovasc Magn Reson. 2000;2:67–82.
• Burke A, Farb A, Tashko G, Virmani R. Arrhythmogenic right ventricular cardiomyopathy and fatty replacement of the right ventricular myocardium: are they different diseases? Circulation. 1998;97:1571–80.
• Bluemke DA, Krupinski EA, Ovitt T, Gear K, Unger E, Axel L, et al. MR imaging of arrhythmogenic right ventricular cardiomyopathy: morphological findings and interobserver reliability. Cardiology. 2003;99:153–62.
• Bomma C, Dalal D, Tandri H, Prakasa K, Nasir K, Roguin A, et al. Regional differences in systolic and diastolic function in arrhythmogenic right ventricular dysplasia/cardiomyopathy using magnetic resonance imaging. The American journal of cardiology. 2005;95(12):1507-11.
• Boulos M, Lashevsky I, Reisner S, Gepstein L. Electroanatomic mapping of Arrhythmogenic right ventricular dyplasia. J Am Coll Cardiol. 2001;38:2020–27.
• Tandri H, Saranathan M, Rodriguez ER, Martinez C, Bomma C, Nasir K, et al. Noninvasive detection of myocardial fibrosis in arrhythmogenic right ventricular cardiomyopathy using delayed-enhancement magnetic resonance imaging. Journal of the American College of Cardiology. 2005;45(1):98-103.
• Hunold P, Wieneke H, Bruder O, Krueger U, Schlosser T, Erbel R, et al. Late enhancement: a new feature in MRI of arrhythmogenic right ventricular cardiomyopathy? J Cardiovasc Magn Reson. 2005;7(4):649-55.
• McCrohon JA, Moon JC, Prasad SK, McKenna WJ, Lorenz CH, Coats AJ, et al. Differentiation of heart failure related to dilated cardiomyopathy and coronary artery disease using gadolinium- enhanced cardiovascular magnetic resonance. Circulation. 2003;108:54–59.
• Tandri H, Saranathan M, Rodriguez ER, Martinez C, Bomma C, Nasir K, et al. Non-invasive detection of myocardial fibrosis in arrhythmogenic right ventricular dysplasia by delayed-enhancement magnetic resonance imaging – correlation with histopathology (abstract). J Cardiovasc Magn Reson. 2004;6:44–45.
• White RD, Trohman RG, Flamm SD, VanDyke CW, Optican RJ, Sterba R, et al. Right ventricular arrhythmia in the absence of arrhythmogenic dysplasia: MR imaging of myocardial abnormalities. Radiology. 1998;207:743–51.
• Grimm W, List-Hellwig E, Hoffmann J, Menz V, Hahn-Rinn R, Klose KJ, et al: Magnetic resonance imaging and signal-averaged electrocardiography in patients with repetitive monomorphic ventricular tachycardia and otherwise normal electrocardiogram.1997;20:1826–33.
• Abbara S, Migrino RQ, Sosnovik DE, Leichter JA, Brady TJ, Holmvang G. Value of fat suppression in the MRI evaluation of suspected arrhythmogenic right ventricular dysplasia. Am J Roentgenol. 2004;182:587–91.
• Nava A, Bauce B, Basso C, Muriago M, Rampazzo A, Villanova C, et al: Clinical profile and long-term follow-up of 37 families with arrhythmogenic right ventricular cardiomyopathy. Journal of the American College of Cardiology (2000), 36(7):2226-2233.
• Corrado D, Basso C, Thiene G, McKenna WJ, Davies MJ, Fontaliran F, et al: Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. Journal of the American College of Cardiology. 1997;30(6):1512-20.
• Bauce B, Basso C, Rampazzo A, Beffagna G, Daliento L, Frigo G, et al: Clinical profile of four families with arrhythmogenic right ventricular cardiomyopathy caused by dominant desmoplakin mutations. Eur Heart J. 2005;26(16):1666-75.
• Lindstrom L, Nylander E, Larsson H, Wranne B. Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy- a scintigraphic and echocardiographic study. Clin Physiol Funct Imaging. 2005;25(3):171-77.
• Sen-Chowdhry S, Syrris P, Ward D, Asimaki A, Sevdalis E, McKenna WJ. Clinical and genetic characterization of families with arrhythmogenic right ventricular dysplasia/cardiomyopathy provides novel insights into patterns of disease expression. Circulation. 2007;115(13):1710-20.
• Jain A, Shehata ML, Stuber M, Berkowitz SJ, Calkins H, Lima JAC, et al. Prevalence of Left Ventricular Regional Dysfunction in Arrhythmogenic Right Ventricular Dysplasia Circulation: Cardiovascular Imaging. 2010;3:290–97.