BEGOÑA IGUAL MUÑOZ MD, Abstract Co-Author: Nothing to Disclose

Esther Zorio MD,PhD, Abstract Co-Author: Nothing to Disclose

Alicia Maceira MD,PhD, Abstract Co-Author: Nothing to Disclose

Jordi Estornell MD,PhD, Abstract Co-Author: Nothing to Disclose

Fernando Más-Estellés MD, Abstract Co-Author: Nothing to Disclose

Almudena Lucas-Perez MD, Presenter: Nothing to Disclose

Iciar Puchades-Román MD, Abstract Co-Author: Nothing to Disclose

Juan Carlos Martinez Martinez, Abstract Co-Author: Nothing to Disclose

Anastasio Quesada MD, MD, Abstract Co-Author: Nothing to Disclose

Maria Pilar Lopez-Lereu MD, Abstract Co-Author: Nothing to Disclose

Recently, the spectrum of arrythmogenic miocardiopathy (AC) has been expanded with newly described biventricular (BVAC) and left-dominant (LDAC) forms. Cardiac magnetic resonance (CMR) with its superb tisular discrimination abilities, and high reproducibility for ventricular volume calculation,offers potentially relevant information for their diagnosis. We aim to analyze the diagnostic performance of different CMR parameters in the diagnosis of AC.

Patients included in this study come from a prospective protocol evaluating cases of familiar cardiac sudden death ( SD), with diagnosis of AC in the index case. All patients underwent the following tests: EKG, cicloergometry, Holter , echocardiography, CMR, and genetic study specific for AC. Diagnosis was based on Task Force criteria in patients without the mutation while in carriers, the presence of findings suggestive of AC in at least two diagnostic tests of different categories was considered as diagnostic. The diagnostic value ( Sensitivity-Sens / Specificity-Spe)  of the following CMR parameters was evaluated: 1- presence of late gadolinium enhancement (LGE) in left ventricle (LV) and right ventricle (RV); 2- LV ejection fraction (LVEF) ≤ 55% and RV EF≤ 45% , and 3- biventricular dilatation (LVEDVi ≥98ml/m2 and RVEDi ≥100ml/m2 in  male or 90ml/m2 in  female)

The group comprised 59 patients (p): 49% male. There were 5 index cases (3 resuscitated, and 2 with cardiogenic syncope and no SD), and 54 first-degree relatives. 32 p(54%) are carriers  of some desmoplakin  mutation and 5 of them also had another mutation or  polymorphism. AC was diagnosed in 10p (17%), 6 (60%) with LDAC and 4(40%) with BVAC . The diagnostic value of LGE was, in the LV ( Sens 100%, Spe 94% ), and in the RV ( Sens:20%, Spe:97% ); for LVEF (Sens:60%, Spe:100%), for RVEF (Sens:30%; Spe:97%), and for RV dilatation ( Sens:10 %, Spe: 94%). No patient showed LV dilatation

1- In this clinical setting ,with high prevalence of left and biventricular forms of AC, left ventricular delayed gadolinium enhancement is the parameter with the highest diagnostic performance. 2- The other parameters are very specific , though poorly sensitive for diagnosis.

Late gadolinium enhancement in left ventricle is the CMR  parameter with best diagnostic performance in left and biventricular forms of AC.

MUÑOZ, B, Zorio, E, Maceira, A, Estornell, J, Más-Estellés, F, Lucas-Perez, A, Puchades-Román, I, Martinez, J, Quesada, A, Lopez-Lereu, M, Cardiac Magnetic Resonance in Arrhythmogenic Cardiomyopathy: What Are the Parameters with Better Diagnostic Performance?.  Radiological Society of North America 2011 Scientific Assembly and Annual Meeting, November 26 - December 2, 2011 ,Chicago IL. http://archive.rsna.org/2011/11001749.html