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|Number 11-13: Fibro-fatty replacement and other abnormalities in suspected ARVC|
Number 11-13: Fibro-fatty replacement and other abnormalities in suspected ARVC
Case from: Lorenzo Monti, Tiziana Manias, Barbara Nardi, Luca Balzarini.
Institute: Radiology and Cardiology Department, I.R.C.C.S. Istituto Clinico Humanitas, Rozzano (MI) ITALY
Clinical history:An asymptomatic 38 year-old man was referred to our center for further assessement because of his family history. The patient's father was previously diagnosed with right ventricular arrhythmogenic cardiomyopathy, a diagnosis that was confirmed on endomyocardial biopsy and subsequently on the explanted heart following heart transplant for ventricular arrhythmias and dilated cardiomyopathy.
His resting 12-lead ECG however was abnormal, showing non-significant Q waves in the inferior leads, diffuse T wave inversion in the precordial leads, and T-wave flattening in the peripheral leads (see ECG 1).
Movie 1 Movie 2 Movie 3
Figure 1a, b, c
There is suspicion of extensive fibro-fatty replacement of the inferior left and right ventricular wall as shown in Figure 1. The site of fatty infiltration (white arrows) can be appreciated on T1 weighted sequences (Figure 1a) when compared to the T1-weighted images with fat-saturation in the same image position (Figure 1b). This infiltration is further unveiled by presence of an unusual chemical shift artefact in the steady-state free precession cine (SSFP) sequences (Figure 1c, still frame, white arrow).
Figure 3 (a,b,c, d)
Late Gadolinium enhancement images (PSIR sequences) confirm the presence of fibrous replacement which is visible in the left inferior and infero-lateral wall (Figure 2, short-axis view). This is also seen on the long axis views (Figure 3 top panel left = 3-chamber view, and top panel right = 4-chamber view). There is also fibrosis of the right ventricular outflow tract (Figures 3 bottom panel).
In this patient, echocardiography failed to identify the initial bi-ventricular dilatation and dysfunction. The number of structural heart abnormalities observed by CMR in this patient allowed a new diagnosis of arrhythmogenic cardiomyopathy with left ventricular involvement to be made. In our case, the newly-modified 2010 diagnostic criteria for ARVC 1 are met in this patient with 3 major criteria being fulfilled: (1) pathologically confirmed diagnosis of ARVC in a first degree relative; (2) RV dilatation with regional akinesia and (3) T wave inversion in V1 - V3. In addition, our CMR data supports not only right but also left ventricular disease involvement, although the left ventricular involvement could already be suspected from the ECG considering the T wave abnormalities extending to the lateral leads3-5.
1.Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MG, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Pro tonotarios N, Saffitz JE, Sanborn DM, Steinberg JS, Tandri H, Thiene G, Towbin JA, Tsatsopoulou A, Wichter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010 Apr 6;121(13):1533-41.
2. McKenna WJ, Thiene G, Nava A, Fontaliron F, Blomstrom-Lundquist G, Fontaine G, Camerini F, on behalf of the Task Force of the working group myocardial and pericardial disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology. Diagnosis of arrhythmogenic right ventricular dysplasia cardiomyopathy.