Letter to the Editor A passionate endurance cyclist ultimately survives sudden death in right ventricular giant cell myocarditis Mark R. Hazebroek a , Pieter van Paassen b , Patrick W. Weerwind c , Leslie T. Cooper Jr. d , Nir Uriel e , Kadir Caliskan f , Bart de Vries g , Jos G. Maessen c , Rüdiger Autschbach h , Stephane Heymans a , Dirk W. Donker a,i, ⁎ a Department of Cardiology, Maastricht University Medical Center, Maastricht, The Netherlands b Department of Internal Medicine, Maastricht University Medical Center, Maastricht, The Netherlands c Department of Cardiothoracic Surgery, Maastricht University Medical Center, Maastricht, The Netherlands d Gonda Vascular Center, Mayo Clinic, Rochester, MN, USA e Mechanical Circulatory Support Research Center for Advanced Cardiac Care, Columbia University, New York, NY, USA f Department of Cardiology, Thoraxcenter, Erasmus Medical Center, Rotterdam, The Netherlands g Department of Pathology, Maastricht University Medical Center, Maastricht, The Netherlands h Department of Thoracic and Cardiovascular Surgery, University Hospital RWTH Aachen, Aachen, Germany i Department of Intensive Care, Maastricht University Medical Center, Maastricht, The Netherlands article info Article history: Received 8 October 2013 Accepted 2 November 2013 Available online 16 November 2013 Keywords: Giant cell myocarditis Sudden cardiac death Endurance exercise Veno-arterial extracorporeal membrane oxygenation Extracorporeal cardiopulmonary resuscitation Biventricular assist device A previously healthy 50-year old male suffered out-of-hospital car- diac arrest (OHCA; ventricular fibrillation). Return of spontaneous circulation occurred after 12 min of immediate resuscitation. Upon ad- mission, recurrent ventricular tachyarrhythmias (VT) caused refractory cardiogenic shock, rescued by peripheral veno-arterial extracorporeal membrane oxygenation (VA-ECMO). Urgent echocardiography and chest CT revealed right ventricular (RV) dilation and biventricular hypokinesia (Fig. 1) in the absence of angiographic evidence of pulmo- nary embolism or coronary disease. RV endomyocardial biopsies (EMB) demonstrated extensive inflammatory infiltrates with multinucleated giant-cells indicating giant-cell myocarditis (GCM; Fig. 1). Furthermore, reverse-transcriptase polymerase chain reaction showed presence of low-load human herpes virus-6 (HHV-6; 18 copies/μg DNA) and parvo- virus B19 (PVB19; 82 copies/μg DNA), lacking serological indication for active cardiotropic viruses. Sarcoidosis and ANCA granulomatosis were excluded by serologic analysis and chest CT. The initiated immunosup- pressive therapy (methylprednisolone 1000 mg iv q.d. and cyclophos- phamide 200 mg iv q.d.) was discontinued due to intercurrent sepsis after 5 days. On day 11, clinical signs of neurologic recovery were evi- dent and VA-ECMO was converted to a biventricular assist device (BiVAD; Berlin Heart Excor®) due to persistent VT and predominant RV failure. After a complicated ICU stay, immunosuppressants (cyclo- sporine 100 mg bid, azathioprine 50 mg qd) were reinitiated on day 72, after serial EMB revealed ongoing GCM and progressive fibrosis (Appendix 1). On day 101, he was weaned from ventilation, followed by ICU and hospital discharge without any neurologic sequelae on days 104 and 210, respectively. Finally, after 8 months of BiVAD support, heart transplant was performed on day 265 and follow-up was un- eventful beyond 1 year including return to former occupation. GCM is a rare and devastating disease with a 1-year mortality and rate of transplant of ~ 70% [1]. A majority (~75%) presents with heart failure, whereas only ~14% exhibits VT or sudden cardiac death (SCD) [1]. The re- ported median time from onset of symptoms to presentation is ~ 3 weeks [1]. However, detailed history taking in this patient revealed flu-like symptoms such as malaise and arthralgia N 12 months before SCD. Impor- tantly, our patient, a passionate race cyclist (N 15 years, N 6000 km annu- ally), experienced a subtle decline in exercise tolerance during his continued endurance training. Strikingly, cycling power, as registered by a bike computer in the months preceding SCD, showed a steady descent followed by a rapid deterioration in the weeks prior to his OHCA (Fig. 2). His long-term, vigorous endurance training may have resulted in overload-induced RV cardiomyopathy, as well-described [2]. Hypotheti- cally, this remodeling process may have modulazted the immune re- sponse and caused enhanced susceptibility and sustained response to danger signals such as virus induced injury and local ischemia. In the con- text of his flu-like symptoms months before SCD, it is indeed tempting to speculate that RV remodeling has set the stage for the adverse immune response to viral presence. The rapid functional deterioration noticed be- fore SCD (Fig. 2) may well be the result of exaggerating RV overload under continued exercise and sustained myocardial inflammation International Journal of Cardiology 170 (2014) e74–e75 ⁎ Corresponding author at: Department of Intensive Care Medicine, University Medical Center Utrecht, Mail Stop F.06.149, PO Box 85500, 3508 GA Utrecht, The Netherlands. Tel.: +31 88 7561126; fax: +31 88 7561160. E-mail address: d.w.donker@umcutrecht.nl (D.W. Donker). Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard 0167-5273/$ – see front matter © 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ijcard.2013.11.028