A passionate endurance cyclist ultimately survives sudden death in right ventricular giant cell myocarditis

A passionate endurance cyclist ultimately survives sudden death in right ventricular giant cell myocarditis
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  Letter to the Editor A passionate endurance cyclist ultimately survives sudden death in rightventricular 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 a r t i c l e i n f o  Article history: Received 8 October 2013Accepted 2 November 2013Available online 16 November 2013 Keywords: Giant cell myocarditisSudden cardiac deathEndurance exerciseVeno-arterial extracorporealmembrane oxygenationExtracorporeal cardiopulmonary resuscitationBiventricular assist device A previously healthy 50-year old male suffered out-of-hospital car-diac arrest (OHCA; ventricular  fi brillation). Return of spontaneouscirculation occurred after 12 min of immediate resuscitation. Upon ad-mission, recurrent ventriculartachyarrhythmias(VT) caused refractorycardiogenic shock, rescued by peripheral veno-arterial extracorporealmembrane oxygenation (VA-ECMO). Urgent echocardiography andchest CT revealed right ventricular (RV) dilation and biventricularhypokinesia (Fig. 1) in the absence of angiographic evidence of pulmo-naryembolismorcoronarydisease.RVendomyocardialbiopsies(EMB)demonstrated extensive in fl ammatory in fi ltrates with multinucleatedgiant-cellsindicatinggiant-cellmyocarditis(GCM;Fig.1).Furthermore,reverse-transcriptase polymerase chain reaction showed presence of low-loadhumanherpesvirus-6(HHV-6;18 copies/ μ  gDNA)andparvo-virus B19 (PVB19; 82 copies/ μ  g DNA), lacking serological indication foractive cardiotropic viruses. Sarcoidosis and ANCA granulomatosis wereexcluded 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 sepsisafter 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 predominantRV failure. After a complicated ICU stay, immunosuppressants (cyclo-sporine 100 mg bid, azathioprine 50 mg qd) were reinitiated onday72, after serialEMBrevealed ongoingGCM and progressive fi brosis(Appendix1).Onday101,hewasweanedfromventilation,followedbyICU and hospital discharge without any neurologic sequelae on days104 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.GCMisarareanddevastatingdiseasewitha1-yearmortalityandrateof transplant of ~70% [1]. A majority (~75%) presents with heart failure,whereasonly~14%exhibitsVTorsuddencardiacdeath(SCD)[1].There-portedmediantimefromonsetofsymptomstopresentationis~3 weeks[1]. However, detailed history taking in this patient revealed  fl u-likesymptomssuchasmalaiseandarthralgia N 12 monthsbeforeSCD.Impor-tantly,ourpatient,apassionate racecyclist ( N 15 years,  N 6000 kmannu-ally), experienced a subtle decline in exercise tolerance during hiscontinued endurance training. Strikingly, cycling power, as registered bya bike computer in the months preceding SCD, showed a steady descentfollowed by a rapid deterioration in the weeks prior to his OHCA (Fig. 2).His long-term, vigorous endurance training may have resulted inoverload-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 todangersignalssuchasvirusinducedinjuryandlocalischemia.Inthecon-textofhis fl u-likesymptomsmonthsbeforeSCD,itisindeedtemptingtospeculate that RV remodeling has set the stage for the adverse immuneresponsetoviralpresence.Therapidfunctionaldeteriorationnoticedbe-fore SCD (Fig. 2) may well be the result of exaggerating RV overloadunder continued exercise and sustained myocardial in fl ammation International Journal of Cardiology 170 (2014) e74 – e75 ⁎  Corresponding author at: Department ofIntensive CareMedicine, University MedicalCenter 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). Contents lists available at ScienceDirect International Journal of Cardiology  journal homepage: 0167-5273/$  –  see front matter © 2013 Elsevier Ireland Ltd. All rights reserved.  [2,3]. This notion is supported by extensive RV dilation and marked  fi -brosis as compared to the LV in the explanted heart (Appendix 1). No-tably, also recurrent VT observed on presentation and to a lesserextent thereafter srcinated from the RV (Appendix 2) and have likelyled to SCD. The incessant RV arrhythmogeneity was intriguing and, in-terestingly,relatedtoareducedexpressionofdesmosomalplakoglobin,associated with RV arrhythmias in athletes and other arrhythmogenicRV cardiomyopathies [4,5] (Appendix 3).OurclinicalobservationsshednewlightonthenaturalcourseofGCMand provide mechanistic insights suggesting that longstanding RV me-chanicaloverload,i.e.,enduranceexercise,mayimpactonmyocardialre-modeling,immunemodulationand phenotypical manifestationof GCM.Supplementary data to this article can be found online at Contributors All authors wrote the report. Written consent to publish wasobtained.  Acknowledgments We pay tribute to the incredible patience, as well as the mental andphysical perseverance of the patient and his family. He contributedsigni fi cantly to the manuscript by providing all his detailed  ‘ cyclingpower ’  data. The professional engagement and passion in dailyhealthcareprovidedbythenursingteamoftheCardiothoracicIntensiveCare Unit in Maastricht, led by Christophe Bout, RN; André Steenhof,RN; and Leon Moonen, RN; and the nursing teams, perfusionists andphysiotherapistsinMaastricht,AachenandRotterdamaregratefullyac-knowledged.WeappreciatethecontributionofBasKietselaer,MD,PhD,DepartmentofCardiology,MaastrichtUniversityMedicalCenter,RobertM.Verdijk,MD,PhD,DepartmentofPathology,ErasmusMedicalCenter,Rotterdam and Egbert Noll, MD, Department of Intensive Care, Univer-sity Hospital RWTH Aachen, Germany. References [1] Cooper Jr LT, ElAmm C. Giant cell myocarditis. Diagnosis and treatment. Herz 2012Sep;37(6):632 – 6.[2] O'Keefe JH, Patil HR, Lavie CJ, et al. Potential adverse cardiovascular effects from ex-cessive endurance exercise. Mayo Clin Proc 2012 Jun;87(6):587 – 95.[3] Ilbäck NG, Fohlman J, Friman G. Exercise in coxsackie B3 myocarditis: effects onheart lymphocyte subpopulations and the in fl ammatory reaction. Am Heart J1989;117(6):1298 – 302.[4] Asimaki A, Tandri H, Duffy ER, et al. Altered desmosomal proteins in granulomatousmyocarditis and potential pathogenic links to arrhythmogenic right ventricular car-diomyopathy. Circ Arrhythm Electrophysiol 2011 Oct;4(5):743 – 52.[5] La Gerche A, Burns AT, Mooney DJ, et al. Exercise-induced right ventricular dys-function and structural remodelling in endurance athletes. Eur Heart J 2012Apr;33(8):998 – 1006. Fig.1.Leftpanel: ChestCT(day5)demonstratingRAandRVdilationwithpreservedLVmorphology.VA-ECMOvenouscannula(blackarrow).Noticetheinterventricularseptaldeviationtowardsthe LV indicating increased RV pressures (white arrow). RA: right atrium, RV: right ventricle, LV: left ventricle, VA-ECMO: veno-arterial extracorporeal membrane oxygenation. Rightpanel: Histology(H&Estaining)ofinitialEBMsdemonstratingfocalin fi ltrationoflymphocytes,histiocytes,eosinophils(whitearrows;x200),andmultinucleatedgiant-cells(blackarrows; x200). Fig.2. Timecourseofcyclingactivityrepresentedbyaverageheart(beats/min)andcyclingpower(Watts)asregisteredbythepatients ’ bikecomputerduringatotalnumberof267train-ing days through the years 2008  –  2011 (see text for details).e75 M.R. Hazebroek et al. / International Journal of Cardiology 170 (2014) e74 – e75
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