Endomyocardial biopsy: safety and prognostic utility in paediatric and adult myocarditis in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry
Alida L.P. Caforio, Juan Pablo Kaski, Juan R. Gimeno, Perry Elliott, Cécile Laroche, Luigi Tavazzi, Michał Tendera, Michael Fu, Simone Sala, Petar Seferović, Tiina Heliö, Leonardo Calò, О. В. Благова, Ahmad Amin, Ingrid Kindermann, Gianfranco Sinagra, Andrea Frustaci, D Bonnet, Philippe Charron, Aldo P Maggioni, CMY Registry Investigators, Roberto Ferrari, Alvaro Alonso, Jeroen J. Bax, Carina Blomström‐Lundqvist, Stephan Gielen, Patrizio Lancellotti, Aldo P. Maggioni, Nikos Maniadakis, Fausto J. Pinto, Frank Ruschitzka, Luigi Tavazzi, Panos Vardas, F Weidinger, Uwe Zeymer, Alec Vahanian, A Budaj, N Dagres, Nicolas Danchin, Victoria Delgado, J Emberson, Örjan Friberg, Chris P Gale, Gr. Heyndrickx, Bernard Iung, Stefan James, A. Pieter Kappetein, Aldo P. Maggioni, Nikos Maniadakis, Klaudia Vivien Nagy, G Parati, A-S Petronio, Mikko Pietilä, Eva Prescott, Frank Ruschitzka, Frans Van de Werf, F Weidinger, U Zeymer, Chris P Gale, B. Beleslin, A Budaj, Ovidiu Chioncel, N Dagres, Nicolas Danchin, J Emberson, David Erlinge, Michael Glikson, Alastair Gray, M Kayikcioglu, Aldo P. Maggioni, K V Nagy, А. О. Недошивин, A-P Petronio, Jolien W. Roos‐Hesselink, Lars Wallentin, U Zeymer, B A Popescu, David Adlam, Alida L.P. Caforio, Davide Capodanno, Marc R. Dweck, David Erlinge, Michael Glikson, Jörg Hausleiter, Bernard Iung, M Kayikcioglu, Peter Ludman, Lars H. Lund, Aldo P. Maggioni, С.Т. Мацкеплишвили, Benjamin Meder, K V Nagy, А. О. Недошивин, Danilo Neglia, Agnès Pasquet, Jolien W. Roos‐Hesselink, Fernando J. Rossello, Sameh Shaheen, Aleksandra Torbica, Alida L.P. Caforio
Abstract
BACKGROUND AND AIMS: Contemporary multicentre data on clinical and diagnostic spectrum and outcome in myocarditis are limited. Study aims were to describe baseline features, 1-year follow-up, and baseline predictors of outcome in clinically suspected or biopsy-proven myocarditis (2013 European Society of Cardiology criteria) in adult and paediatric patients from the EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry. METHODS: Five hundred eighty-one (68.0% male) patients, 493 adults, median age 38 (27-52) years, and 88 children, aged 8 (3-13) years, were divided into 3 groups: Group 1 (n = 233), clinically suspected myocarditis with abnormal cardiac magnetic resonance; Group 2 (n = 222), biopsy-proven myocarditis; and Group 3 (n = 126) clinically suspected myocarditis with normal or inconclusive or no cardiac magnetic resonance. Baseline features were analysed overall, in adults vs. children, and among groups. One-year outcome events included death/heart transplantation, ventricular assist device (VAD) or implantable cardioverter defibrillator (ICD) implantation, and hospitalization for cardiac causes. RESULTS: Endomyocardial biopsy, mainly right ventricular, had a similarly low complication rate in children and adults (4.7% vs. 4.9%, P = NS), with no procedure-related death. A classical myocarditis pattern on cardiac magnetic resonance was found in 31.3% of children and in 57.9% of adults with biopsy-proven myocarditis (P < .001). At 1-year follow-up, 11/410 patients (2.7%) died, 7 (1.7%) received a heart transplant, 3 underwent VAD (0.7%), and 16 (3.9%) underwent ICD implantation. Independent predictors at diagnosis of death or heart transplantation or hospitalization or VAD implantation or ICD implantation at 1-year follow-up were lower left ventricular ejection fraction and the need for immunosuppressants for new myocarditis diagnosis refractory to non-aetiology-driven therapy. CONCLUSIONS: Endomyocardial biopsy was safe, and cardiac magnetic resonance using Lake Louise criteria was less sensitive, particularly in children. Virus-negative lymphocytic myocarditis was predominant both in children and adults, and use of immunosuppressive treatments was low. Lower left ventricular ejection fraction and the need for immunosuppressants at diagnosis were independent predictors of unfavourable outcome events at 1 year.