Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova

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Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Cardiopathies,acquises

           Dr Daniela Laux

 Cardiopédiatre associée – UE3C –Paris

    Médecin hospitalier temps partiel
  Centre Chirurgical Marie Lannelongue
          Le Plessis Robinson
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Plan,du,cours,

• Maladie(de(Kawasaki(

• Endocardite(infec4euse(

• Myocardite(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Maladie(de(Kawasaki(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Kawasaki – Les points clés

• Maladie(décrite(en(1967(–(seulement(50(ans(de(recul(!(
• Vascularite, systémique, qui( touche( essen4ellement( les,
  artères, de, moyen, calibre, avec( un( tropisme( élec4f( pour( les,
  artères,coronaires,(gravité(de(la(maladie)((
• Les(complica4ons(coronaires(surviennent(dans(15,à,25,%,des(
  cas(chez(les(enfants(non(traités(
• L'administra4on( précoce( d'immunoglobulines( humaines( par(
  voie( intraveineuse( a( transformé( le( pronos4c( en, diminuant,
  par,5,le(risque(d'anévrisme(coronaire(
• Risque, de, mortalité, 0,015, %, (Japon),( surtout( entre( le(
  15N45eme( jour( (thrombocytose( concomiQante( avec( la(
  vascularite)(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Epidémiologie(
• Première,cause,de,cardiopathie,acquise,de,l'enfant,dans,les,
  pays,développés,
(
• Tous,les,âges,pédiatriques,(80,%,des,cas,avant,5,ans)(
• Les(pa4ents(de(moins,de,1,an,ou(de,plus,de,8,ans,sont(rares(
  mais(ont(un(risque(plus(élevé(d'anévrisme(coronaire(

• Formes,de,l'adulte:,première(fois(décrite(en(1977(
• symptômes(majeurs(décrits(iden4ques(
(
• Kawasaki,aOeint,chaque,année:,
   – 265/100.00(enfants(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
CaractérisTques,cliniques:,critères,majeurs,
    La,fièvre,de,plus,de,5,jours,et,au,moins,4,critères,suivants,
    (
    N(La(conjoncTvite(bulbaire(non(purulente(

    N(L’aOeinte,muqueuse(:(la(pharyngite,(la(chéilite,(la(langue(framboisée,(la(stoma4te(

    N(L'exanthème,polymorphe,du,tronc,

    N(L'aOeinte,des,extrémités(:(un(érythème(des(paumes(des(mains(et/ou(des(plantes(
    des(pieds,(l'œdème(palmoNplantaire,(la(desquama4on(palmoNplantaire(secondaire((

    N(L'aOeinte,unilatérale,des,ganglions,cervicaux,(de(plus(de((
    (1.5(cm(de(diamètre((
    (

Critères majeurs définis par l'American Heart Association Committee On Rheumatic Fever, Endocarditis, and Kawasaki Disease
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Faciès(typique(d’un(enfant(avec((
   une(maladie(de(Kawasaki(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
AQeinte(muqueuse(et(desquama4on(palmoNplantaire(
Cardiopathies,acquises - Dr Daniela Laux Cardiopédiatre associée - UE3C -Paris Médecin hospitalier temps partiel - Arcothova
Formes(rares:(forme(psoriasiforme/(BCGite(
AQeinte(cardiovasculaire(

• Pas,d’aOeinte,coronaire,dans,75%,des,cas,!,
• Anomalie,ECG,ou,échocardiographique:,
   – DilataTon,des,artères,coronaires,(20%),
   – Anévrismes(coronaires(
   – Infarctus(
   – Myocardite(avec(possible(insuffisance(ventriculaire(gauche(
     sévère(
   – Péricardite,(épanchement(péricardique(
   – Fuites(valvulaires(par(inflamma4on(des(valves(cardiaques(et(
     par4culièrement(la(valve(mitrale((1(%)(
   – Troubles(conduc4fs(et(troubles(du(rythme(par(inflamma4on(
     du(4ssu(de(conduc4on(
Les(anévrismes(coronaires(
•                                (
   Entre,le,10 ,et,le,25 ,jour,d'évoluTon,
             ème         ème

• 5(%(lorsque(le(traitement(est(fait(précocement((
       (=(,2,,2,5,,10,ou,>,8,mm,
  (

                                                           Newburger JACC 2016
Formes(atypiques(
Tableau,clinique,dominé,par,un,symptôme,inhabituel:(
(
«(convulsions,(œdème(pulmonaire,(diarrhée(sanglante,(ascite,(
obstruc4on(des(voies(aériennes(supérieures,(épiglonte,(
adénopathies(cervicales(compressives(ou(hémolyse(et(
défaillance(mul4Nviscérale,(syndrome(néphro4que,(
hyponatrémie….(»(
(
Formes,de,l'adulte,
•        (Troubles(diges4fs,(aQeinte(hépa4que,(signes(
        (ar4culaires(et(encéphalites(sont(plus(fréquent(
Les(formes(incomplètes(
,

Pa4ents(ayant(eu(une(fièvre(depuis(au,moins,5,jours,et((
au(moins,deux,critères,cliniques,de,Kawasaki,(sans(cause(évidente,((
et(des(critères(biologiques(en(faveur(d'une(inflamma4on(systémique(
(
• Différent,de,la,«,forme,atypique,»,
,
• Manque(un(ou(plusieurs,des,cinq,critères,diagnos4ques(majeurs(
(,
• Plus,fréquentes,chez,les,enfants,les,plus,jeunes,,à,risque,,
,,,,,d'anomalies,coronaires(
         ((
    Diagramme(décisionnel(proposé(par(l'American(Academy((
    of(Paediatrics(pour(aider(à(la(prescrip4on(d’IgG(dans(les(formes(
    incomplètes((

                                                  Newburger JW et al., Circulation. 2004
Critères,cliniques,et,biologiques,supplémentaires,
Cardiovasculaires,:(dilata4on(des(artères(coronaires,(anévrismes(coronaires,(
infarctus,(myocardite(avec(possible(insuffisance(cardiaque(conges4ve,(péricardite,(
épanchement(péricardique,(fuites(valvulaires,(troubles(conduc4fs(et(troubles(du(
rythme,(anévrismes(des(vaisseaux(du(cou,(des(artères(rénales,(spléniques,(
hépa4ques,(pancréa4ques,(génitales,(gangrènes(distales(et(pseudoNRaynaud(
(

DigesTfs,:,diarrhées,(vomissements,(douleurs(abdominales,(hydrocholécyste,(
dysfonc4on(hépa4que(
(

Respiratoires(:(toux(et(rhinorrhée((
(

Neuroaméningés(:(troubles(de(la(conscience(avec(irritabilité,(apathie,(état(grognon,(
hypoacousie(
(

ArTculaires,:(arthrite,(arthralgies(
Autres,:(uvéite,(érythème(au(niveau(de(la(cicatrice(de(BCG,(desquama4on(de(l’aine(
(
Albumine(
secondaire en « doigt de gant »
   Atteinte unilatérale des ganglions cervicaux, de plus de 1,5 cm de diamètre, ferme

                                                                      Formes incomplètes
 upplémentaires
                                                  Fièvre de plus de 5 jours et 2 ou 3 critères cliniques
e, péricardite, état de choc,                                              ou
 ronaire, anévrismes extra-               Fièvre de plus de 7 jours sans cause retrouvée (enfants ≤ 6 mois+++)
(vaisseaux du cou, artères
s, pancréatiques, génitales),
 du culot aortique                                                Faire un bilan biologique
 s, vomissements, douleurs
 e, dysfonction hépatique,
 te/méatite, hydrocèle
                                  CRP < 30 mg/l et VS < 40 mm/h                    CRP ≥ 30 mg/l et/ou VS ≥ 40 mm/h
  ribronchique et interstitiel,
monaires
   conscience avec irritabilité
aralysie faciale, hypoacousie     Réexaminer et contrôler le bilan               Au moins 3 critères biologiques ou plus :
  ies (hyperleucocytose du         biologique si la fièvre persiste
novial)                                                                                    Anémie pour l’âge
 eau de la cicatrice de BCG,       Échocardiographie en cas de                         Plaquettes ≥ 450 000/mm3
                                                                           Non
hlegmon rétropharyngé             desquamation en doigt de gant                            Albumine ≤ 30 g/l
                                             typique                                       ALAT augmentées
                                                                                     Globules blancs ≥ 15 000/mm3
                                                                                    ECBU ≥ 10 globules blancs/champ
 stics différentiels                                                       Oui                       ou
ntérovirus, EBV, rougeole                                   Traiter                     Échocardiographie positive
ine
se staphylococcique
hoc toxique                                                    Échocardiographie positive
actérienne
x médicaments                                           Z-score IVA ou CD ≥ 2,5
vens-Johnson                                            Ou anévrisme coronaire (Z-score ≥ 2,5)
juvénile                                                Ou plus de 3 critères :
rose                                                    - dysfonction ventriculaire gauche
                                                        - fuite mitrale
                                                        - épanchement péricardique
oronaire élevé si score ≥ 5)                            - Z-score IVA ou CD compris entre 2 et 2,5
                                                                                                                     Bajolle et al. 2018 EMC
(1 point)                                                         Description coronaire
s jours de fièvre (2 points)
Evolu4on(naturelle(

(
1°)(La(phase(aiguë((J0NJ10)(:(aOeinte,cardiaque,rare,
(
2°)(La(phase(subaiguë((J10NJ20)(:(diagnosTc,de,complicaTon,
coronaire,
(
3°)(La(phase(de(convalescence((J20NJ70)(:(constataTon,d’anévrysmes,
et,de,sténoses,cicatricielles,en(cas(de(complica4on(coronaire(à(la(
deuxième(phase(
Exemples(d’aQeinte(coronaires(
Dilata4on(anévrysmale(des(artères(coronaires(

  Aorte         IVA =6.9 mm
                                                   Thrombus
                                        Aorte      dans l’IVA

          (A)(IVA=interventriculaire(antérieure(
          (B)(thrombus(dans(l’IVA(
Dysfonc4on(ventriculaire(gauche(sévère(en(
             échographie(

 Etat de choc dans 7% des cas de maladie de Kawasaki

             VG
            dilaté
                                    VG
                                   dilaté
Dilata4on(anévrysmale(des(artères(
      coronaires(au(scanner(

   CD =4.7 mm

                Aorte

                    IVA =6.9 mm
Dilata4on(anévrysmale(de(l’IVA(en(
           chapelet(au(scanner(

Aorte
                            Aorte

    Chapelet anévrysmal
                               Chapelet anévrysmal
Anévrysme(coronaire(avec(sténose(
coronaire(au(cathétérisme(cardiaque(

           Sténose     Anévrysmes et sténoses
          coronaire    multiples des coronaires
AQeinte(diffuse(des(axes(vasculaires(

                                       A                                                         B

                                                                  Foie

                                                        Dilatation
                                                        fusiforme

                 Aorte

(A)(épaississement(pariétal(hyperéchogène(de(l’aorte,(de(l’artère(mésentérique(supérieure(et(du(tronc(
coeliaque(en(échographie((
(B)(dilata4on(fusiforme(de(l’artère(mésentérique(supérieure,(du(tronc(caeliaque(au(scanner(
Le(traitement(de(1ère(inten4on(

Immunoglobulines,intraveineuses:(
• 2g/kg(en(8(à(12(heures,(à(posologie(progressive(
(
En,associaTon(à(de(l’acide,acétylsalicylique,(AAS),:,
• à( fortes( doses( pour( ses( effets( an4Ninflammatoires( et( an4N
  thrombo4ques((30a50,mg/kg/j,en,4,fois),à(la(phase(aiguë((
• puis( à( dose, anTaaggrégante, plaqueOaire, rapidement( 48N72( h(
  après(l’apyrexie((3N5(mg/kg/jour)(

                                    De Graeff et al 2019, Newburger 2016
CE NTRAL ILL USTRATIO N Management of Kawasaki Disease

                                 Diagnosis of Kawasaki disease according to American Heart Association criteria*

                 Anti-inflammatory therapy with intravenous immunoglobulin (IVIG) and ASA (acetylsalicylic acid or aspirin).
                                    Echocardiogram to assess coronary arteries and determine Z score.

      Defervescence with no coronary dilation (Z score
Kawasaki Disease                                                                                                                                         APRIL 12, 2016:1738–49

                                         Recommanda4ons(américaines(
              TABLE 1 Principles in Acute Management of KD

              1. The goal of therapy is to reduce systemic and tissue-level inflammation as rapidly as possible. For this reason, patients should be treated as soon as
                 diagnosis can be confidently established.
              2. All patients within the first 10 days of fever onset should be treated with IVIG. Patients diagnosed after 10 days should receive IVIG treatment if
                 they are still febrile, have markedly elevated inflammatory parameters, or have coronary artery dilation.
              3. Recrudescent fever at least 36 h after the end of IVIG infusion without other explanation is a marker for persistent inflammation and should prompt
                 immediate and aggressive anti-inflammatory therapy
                   a. Antibody-mediated hemolysis has become common in KD patients who have received IVIG retreatment and have type A or B blood; rescue
                      therapies other than IVIG (e.g., infliximab, corticosteroids) should be considered.
              4. Patients with coronary artery dilation (z-score >2.0) should be followed with a repeat echocardiogram at least twice a week until dimensions
                 stabilize; additional anti-inflammatory therapy should be considered.
              5. Patients with giant aneurysms should have frequent echocardiograms in the first 3 months of illness for thrombus surveillance, even after
                 dimensions stabilize.
              6. Infants under 6 months of age are at extremely high risk of aneurysm formation, even with timely therapy. They require echocardiograms every few
                 days until dimensions have stabilized.
              7. Patients with giant CAA (z-score $10) are at highest risk for thrombosis during the first 3 months after fever onset
                   a. Systemic anticoagulation together with an antiplatelet agent should be administered until coronary dimensions improve.
                   b. Low-molecular-weight heparin is easier to regulate than warfarin in infants, as well as in patients of any age, during the acute phase of illness or
                      until hsCRP normalizes.

              CAA ¼ coronary artery aneurysm; hsCRP ¼ high-sensitivity C-reactive protein; IVIG ¼ intravenous immunoglobulin; KD ¼ Kawasaki disease.

                                                                                                                                                       Newburger JACC 2016
               The progression of aneurysm formation in some                                       Children With Kawasaki Disease and Coronary Artery
Surveillance(à(court(terme((
Pour,les,paTents,sans,complicaTon,coronaire:,
• 1(echo(entre(1N2(semaines(et(1(echo(entre(4N6(semaines((Classe(1)(

Pour,les,paTents,avec,Z,score,coronaire,>,2,0,à,la,phase,aigue:,
• 2(echos(par(semaines(jusqu’à(l’arret(de(la(progression(
(

Pour,les,paTents,avec,anévrismes,géants:,
• 2(échos(par(semaine(tant(que(les(lésions(progressent(
• 1(écho/sem(pdt(45(jours(puis(
• 1(écho/mois(pendant(3(mois((Casse(IIA)(

                                                          Newburger JACC 2016
An4agréga4on(et(an4coagula4on(
PaTents,sans,aOeinte,coronaire:,
• Aspirine(pendant(6(semaines((Cl(1)(
(
PaTents,avec,aOeinte,coronaire,d’aggravaTon,rapide:,
• Hospitalisa4on(pour(mise(sous(heparine((An4Xa(0,5N1)(
• Arret(si(stabilisa4on;(Z(score(
Que(faire(en(cas(de(persistance(de(la(fièvre(
          après(une(première(cure(d’IgIV?(
• Résistants:,15(à(20(%(des(cas(
• Associée(à(un(risque(plus(élevé(d'aQeinte(coronaire(

• Deuxième(dose(d’IVG((
• associée(à(un(bolus(de(cor4coides((20N30(mg/kg/jour(IV(
  methylprednisolone)(

• Discuter:,prednisolone(p.o.(plus(prolongé(2N3(semaines(
• Alterna4ve:(infliximab(
• En(cas(d’échec:(ciclosporine(

                                          Newburger(JW(et(al.,(Circula4on.(2004(
                                                   Egami&K&et&al.,&J&Pediatr&2006&
Qui sont les patients à haut risque?
                       Comment        identifier    les   résistants     ?
                       • Score issu de la littérature de patients japonais!!

              Score d’Egami (2006)            Score de Kobayashi (2006)        Score de Sano (2007)

              Age ≤ 6 mois (2 points)         Age ≤ 12 mois (1 point)          Bilirubine totale ≥ 0.9mg/dL (1
                                                                               point)
              ≤ 4 jours de fièvre (1 point)   Traitement dans les 4 premiers CRP ≥ 7mg/dL (1 point)
                                              jours de fièvre (2 points)
              Plaquettes ≤ 300.109/L (1 Plaquettes          ≤   300.109/L   (1 ASAT ≥ 200 U/L (1 point)
              point)                          point)
              CRP ≥ 8mg/dL(1 point)           CRP ≥ 10mg/dL (1 point)
              ALAT > 100 U/L (1 point)        ASAT ≥ 100 U/L (1 point)
                                              ≥ 80% neutrophiles (2 points)
                                              Na+ ≤ 133 mmol/L (2 points)

              Haut risque si ≥ 3 points       Haut risque si ≥ 5 points        Haut risque si ≥ 2 points

              Scores issus de la population japonaise, spécifique mais non sensible
Shohai O et al., Pediatrics 2011; Egami K et al., J Pediatr 2006 ; Chen S, et al. JAMA 2016; Mc Crindle Circ 2017
2. Treatment of KD should include IVIG at a dose of 2 g/kg as a single infusion.                                                                                                          1A                                                                                                       A
           3. In non-Japanese patients, the Kobayashi criteria may indicate risk of IVIG resistance if ‘positive’ (score                                                                             2A                                                                                                       C
                     Original article
             54) but may not reliably exclude IVIG resistance if ‘negative’ (score
Quelle,est,l’histoire,naturelle,des,complicaTons,
                   coronaires,de,la,MK?,

• Dispari4on(complète(dans(plus(de(50%(des(cas(même(
  en(cas(d’anévrysme((sauf(géant)(dans(les(2(ans(

• Occlusion(coronaire;(sténoses(localisées(ou(mul4ples(
  parfois(très(tardives…(

• Gravité(des(lésions(tardives(car(mul4ples(et(chirurgie(
  difficile(
Anévrysme(géants((1%)(
• Mortalité(et(morbidité(+++(
• Survie(à(30(ans:(88N90%(

•   Cardiac(event(free(à(30(ans(:(30%(
•   26%(infarctus(myocardique(           Adapted with permission from Newburger et al. (99).

•   Risque(accru(dans(les(2(ans(après(le(diagnos4c((
•   50%(de(bypass(coronaire(à(30(ans(
Surveillance à long terme
19-1800 ! Maladie de Kawasaki

                                                       Immunoglobulines IV à 2 g/kg sur 12 h
                                     Aspirine à dose anti-inflammatoire 60 mg/kg/j jusqu’à disparition de la fièvre
                                                            Puis relais par AAP 3–5 mg/kg/j

               75 %                    20 %                                         4%                                              1%

                                                                                                                         Anévrisme géant
              Coronaires                                    Petit anévrisme                Anévrisme moyen
                                        Dilatation                                                                    Z-score ≥ 10 ou ≥ 8 mm
               normales                                     2,5 ≤ Z-score < 5               5 ≤ Z-score < 10
                                     2 ≤ Z-score < 2,5                                                                      AAP à vie
              Z-score < 2                                       AAP 1 an                       AAP à vie
                                     AAP 6 semaines                                                                      AVK pour INR 2-3
            AAP 6 semaines                                 Cs à M3, M6, M12                Cs à M3, M6, M12
                                     Cs à 6 semaines                                                               Cs à M1, M2, M3, M6, M9, M12
            Cs à 6 semaines                                Coroscanner à 1 an              Coroscanner à 1 an
                                                                                                                      Coronarographie à 1 an

                                                                                         Même                Si         Normali-           Si
                         Normali-           Si
             Arrêt                                        Normali-         Si               si          persistance      sation       persistance
                          sation       persistance
            aspirine                                       sation     persistance     normalisation      Aspirine       Aspirine     Aspirine à vie
                           Arrêt        Maintien
            Clôture                                         Arrêt      Maintien       Aspirine à vie       à vie          à vie      Maintien AVK
                         aspirine       aspirine
              du                                          aspirine     aspirine         ± double         ± double       ± double     ± double AAP
                        Clôture du       Cs/2–5
            dossier                                      Cs/1–3 ans     Cs/an             AAP              AAP            AAP            ± BB
                         dossier           ans
                                                                                         Cs/an            Cs/an          Cs/an         Cs/6 mois

                                                             Prévention des FDRCV pour tous !

Figure 2. Arbre décisionnel. Prise en charge proposée par le centre de référence Malformations Cardiaques Congénitales Complexes (M3C) Necker. IV : par
voie intraveineuse ; AAP : aspirine à dose antiagrégante plaquettaire ; Cs : consultation ; AVK : antivitamine K ; INR : international normalized ratio ; FDRCV :
facteurs de risques cardiovasculaires.

! Formes incomplètes                                                                 lines est le plus souvent excellente. La recommandation est une
                                                                                     perfusion lente de 12 heures pour éviter tout effet secondaire
   Lorsqu’on a une forme incomplète de maladie de Kawasaki                           (flush, hypertension, malaise,Bajolle     et al.etc.).
                                                                                                                     hypotension,      2018 Les EMC
                                                                                                                                                immuno-
(fièvre d’au moins cinq jours associée à deux ou trois critères cli-                 globulines humaines sont des médicaments dérivés du sang. À
iffness   testing should minimize risks of anesthesia and
 North     ionizing radiation. Children too young to exercise
 in pe-
, reas-                      Devenir(à(long(terme(
of late     T A B L E 2 Principles in the Long-Term Management of Patients With KD
nifesta-
            1. On the basis of available data, patients with no demonstrated coronary artery
always-         dilation by echocardiogram with excellent visualization of all arterial segments
ears of         during the first weeks of illness appear to have normal cardiovascular status in
                early adulthood.
            2. Remodeling (so-called regression) of aneurysms, especially if moderate or large,
                to normal internal lumen diameter is often accompanied by luminal
                myofibroblastic proliferation and abnormal vascular reactivity.
            3. Patients with persistent CAA are at lifelong risk of progressive coronary artery
                stenosis or occlusion and worsening ischemia.
ifelong     4. Patients with CAA documented at any stage require lifelong cardiovascular
 are to         surveillance tailored to disease severity and age.
            5. Testing should minimize exposure to ionizing radiation whenever possible.
cardial
            6. Sedentary life-style should be avoided.
ble 2).
            7. Women with coronary aneurysms can carry pregnancy successfully, but should
de the          have reproductive counseling.
testing     8. Monitoring and counseling regarding traditional CV risk factors is appropriate to
               reduce the likelihood of later atherosclerosis.
d upon                                                                                Newburger JACC 2016

athero-
Endocardite(
Endocardite(infec4euse(
(
Def:(Infec4on/inflamma4on(de(l’endocarde(=(valves(cardiaques(

Dg:,Echographie(transNthoracique(voire(ETO(

         (N(EI(des(VAV:(sur(le(versant(auriculaire(

         (N(EI(des(valves(sigmoïdes:(sur(le(versant(ventriculaire(

•   Hémocultures:(au(moins(3!!!!!!!!!!!((au(mieux(6)(

•   Pas(d’ATB(à(l’aveugle(

•   Scanner(total(body((cérébral,(thoracique(et(abdominal)(
•   Examen(ophtalmologique,(bandeleQe(urinaire(
•   Recherche(porte(d’entrée:(examen(dentaire,(ORL,(cutané,(diges4f,(
    urinaire,(KTC…(

(
Endocardite:(germes(
Endocardite:(Traitement(médical(
Endocardite:(Traitement(chirurgical(
Endocardite:(préven4on(
Endocardite:(préven4on(
Endocardite:(préven4on(

- Bonne hygiène dentaire quotidienne
- Consultation dentaire tous les 6 mois

               Indispensable pour diminuer le risque d’endocardite
POPULATION CONGENITALE(N(ENFANTS(

34 279 enfants avec CC suivis de 0 à 18 ans
Incidence annualisée = 4.1 / 10 000 pt-année

                                               Rushani et al. Circulation
                                                                    2013
POPULATION CONGENITALE(N(ADULTES(

              Registre CONCOR (14 224 patients>18 ans)
                  Incidence EI : 1.33/1000 pt-years
               Prothèse valvulaire: HR=3.57(2.58–5.36)

                                              Kuijpers et al. Eur Heart Jour
                                                                       2017
INCIDENCES(COMPARATIVES(

Valve Melody : 0.8 – 3% pt-année

Valves/conduits pulmonaire chir : 0.5 - 3% pt-année

TAVI: 0.67 – 2.1% pt-année

Valves Ao/mitrale chir : 0.3 – 1.2% pt-année

Dispositifs electroniques implantables : 1.9/1000,deviceaannée,,

Patients avec CC: 0.4 – 1.33 / 1000 pt-année
                                                                   Miranda et al. Eur Heart Jour 2016
                                                                              Wang et al. JAMA 2007
                                                                       Rushani et al. Circulation 2013
Population générale : 30 -100/ million pt-année                      Habib et al. Eur Heart Jour 2015
                                                                            Dayer et al. Lancet 2015
COMPARER(CE(QUI(EST(COMPARABLE(

                                  50
VALVES(PERCUTANÉES(VS(CHIRURGICALES(

          p=0.1
            3

134 chir et 208 percut (33 Sapien)    195 chir et 93 percut (0 Sapien)         631 chir et 107 percut (0 Sapien)

Incidence IE: 0.5 vs 1.5 %pt/années   Incidence IE: 1.2 vs 3.9 %pt/années      Incidence IE: 0.8 vs 2.7 vs 3% %pt/années

           Lluri et al. CCI 2017          Malekzadeh-Milani et al. JTCS 2014                   Van(Dijck(et(al.(Heart(2014(
SUBSTRAT(VALVULAIRE(
  Author     Year     n       Substrate           EI      EI Annualized    Median
                                             Cumulative     Incidence     Follow-up
                                              incidence    (% pt-year)     (years)

 Albanesi,   2014   12/106     Contegra         11.3                         7.6

Malekzadeh   2014   5/190    Homografts         2.6            1.2           2
                              Contegra

Ramanan      2015   6/115      Freestyle        5.4             -            4.3

  Mery       2016   23/586    Homograft          4              -            7
                              Contegra
                             Porcine valve
  Ugaki      2016   21/298    Contegra           7              -            3.4
                              Homograft

Tous les dispositifs valvulaires sont susceptibles d’être le
                       siège d’une EI
      Avec une incidence variable mais significative                                             Albanesi(et(al.(EJCTS(2014(
                                                                                       Ramanan et al. Ann Thorac Surg
                                                                                                                      2015
                                                                                      Ugaki et al. Ann Thorac Surg 2016(
                                                                                                     Mery(et(al.(JTCS(2016(
SUBSTRAT(VALVULAIRE(

• EI plus fréquente chez les patients avec VJB
• Quelle que soit la techniqued’implantation (i.e. Contegra et Melody)
• Comparés aux homogreffes RR=8.7 and 9.7 pour Melody et Contegra

MalekzadehNMilani(et(al.(JTCS(2014(                                                    (Mery et(al.(JTCS(2016(
Van(Dijck(et(al.(Heart(2014(                                             Ugaki(et(al.(Ann Thorac Surg 2015
SUBSTRAT(VALVULAIRE(
                                      Méta-analyse sur IE chez les patients avec RVP chirurgical ou
                                      percutané
                                      7063 patients
                                      Incidence cumulative globale = 2.5%
                                      VJB vs autres substituts : 5.4% vs 1.2%; p < 0.0001

Sharma et al. JACC Int. 2017
VALVE(SAPIEN(
                                                                                                                                                                         JACC: CARDIOVASCULAR INTERVENTIONS                                                                            VOL. 10, NO. 5, 2017

                                                                                                                                                                         ª 2017 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION                                                    ISSN 1936-8798/$36.00

                                                                                                                                                                         PUBLISHED BY ELSEVIER                                                                  http://dx.doi.org/10.1016/j.jcin.2016.12.012

    512           Hascoet et al.                                                                                   JACC: CARDIOVASCULAR INTERVENTIONS VOL. 10, NO. 5, 2017

                  Endocarditis After PPVI: Melody Versus Sapien                                                           Infective Endocarditis Risk After                  MARCH 13, 2017:510–7

                                  JACC: CARDIOVASCULAR INTERVENTIONS VOL. 10, NO. 5, 2017
                                                                                                                          Percutaneous Pulmonary             Hascoet et al.Valve513
                                  MARCH 13, 2017:510–7                                                                       Endocarditis After PPVI: Melody Versus Sapien
                                                                                                                          Implantation With the Melody
                                                                                         and from phone calls to the patients and to their
     TABLE 1         Patient Demographics, Procedural Data, and Post-Procedural Outcomes                                  and Sapien Valves
                                                                                         cardiologists and general practitioners. For cases of
                                             PPVI With                  PPVI With                                IE, every effort was made to obtain
                                                                                                                                                Sebastieninformation
                                                                                                                                                          Hascoet, MD,a Luciaon
                                                                                                                                                                             Mauri, MD,a Caroline Claude, MD,a Emmanuelle Fournier, MD,a Julie Lourtet, MD,b
                                            Melody Valve               Sapien Valve         Standardized                                                                                         c                      c
                                                                                                                 the Duke criteria, clinical and microbiological
                                                                                                                                                    Jean-Yves Riou, MD, details,
                                                                                                                                                                        Philippe Brenot, MD,                                Jérôme Petit, MDa
                                              (n ¼ 32)                   (n ¼ 47)            Difference

      Age (yrs)
                                    (73 of19.979;(15.8–28.9)
                                                    92.4%). A single   stent was sufficient
                                                               26.3 (18.9–39.9)    0.58*
                                                                                           in all
                                                                                              medical and surgical strategies, and outcome.
                                                                                                                            TABLE 1 Continued
      Weight (kg)                   Melody56.5
                                            group
                                               ! 13.5patients, 65.8
                                                               whereas
                                                                    ! 17.6 in the Sapien
                                                                                    0.59* group 4
                                                                                                                                                                            ABSTRACT
                                                                                                   STATISTICAL ANALYSIS. Statistical analyses were
      Male (%)                               53.1                         66.0           0.26                                          PPVI With                          PPVI With
                                    patients required 2 to 4             stents. Pre-stenting was not
                                                                                                   performed   using Stata 11.2 software   (StataCorp,
                                                                                                                                      OBJECTIVES                 Col- the risk of infective endocarditis (IE) after percutaneous pulmonary valve
                                                                                                                                                     This study compared
      Genetic syndrome (%)                       18.8                     10.6          -0.23                                         Melody    Valve                   Sapien   Valve             Standardized
                                                                                                                                      implantation (PPVI) with the Sapien and Melody valves.
      History of severe infectiousperformed
                                          9.4in 6 patients               with
                                                                           8.5 valve-in-valve
                                                                                        -0.03 implan-
                                                                                                   lege Station, Texas). Continuous data(n ¼were
                                                                                                                                               32) described (n ¼ 47)                               Difference
              disease (%)
      History of endocarditis (%)
                                    tation. Balloon
                                             6.3
                                                    post-dilation
                                                               2.1
                                                                   was performed
                                                                            -0.20
                                                                                  in 19 as
                                                                                        of meanInfective
                                                                                                  ! SD if normally distributed
                                                                                                         endocarditis during
                                                                                                                                 and asThemedian
                                                                                                                             BACKGROUND
                                                                                                                                25.0
                                                                                                                                          incidence of IE after PPVI is estimated at 3% per year with the Melody valve. The Sapien valve is a
                                                                                                                                                              0.0                         -0.80*
                                                                                     (interquartile range [IQR])                                                       more recently Categorical
                                                                                                                                                                   otherwise.        marketed valve used for PPVI.
      Pacemaker/defibrillator (%)79       patients
                                               6.3 (24.1%) and was
                                                               10.6 more common
                                                                            0.16 in the          follow-up (%)
                                                                                     variables were described as                                                    number  (%).
                                                                                                                                                                       METHODS      Bivariate
                                                                                                                                                                               We retrospectively included consecutive patients who underwent PPVI at a single center between 2008 and
      Congenital heart diseases (%)                                                       Pulmonary valve replacement     25.0                               4.3                                                                      -0.59*
                                    Melody group (46.9% vs. 6.5%, respectively). Pro-
                                                                                   analyses with calculation of standardized       differences
                                                                                                                      2016. IE was diagnosed using the modified DUKE criteria.
          Conotruncal malformation               81.3                      68.1                                             during follow-up (%)
          Ross procedure            cedure duration,
                                            9.4             21.3 time, and irradiation
                                                     fluoroscopy                     were                              performed to compare variables
                                                                                                                         Percutaneous
                                                                                                                                                                  between the
                                                                                                                                                             3.1 PPVI was performed in 79 patients
                                                                                                                                                       RESULTS                                     2.1 (Melody valve, 40.5%;-0.06
                                                                                                                                                                                                                                Sapien valve, 59.5%). Median age was 24.9 years
                                                                                                                two valve types and between patients   (range 18.1 towith
                                                                                                                                                                      34.6). IEversus
                                                                                                                                                                                occurred in 8 patients (10.1%) at a median of 1.8 years (minimum: 1.0; maximum: 5.6) after
          TGA
                                    were higher
                                             3.1
                                                 in the Sapien 0.0
                                                               valve group.                                              Surgical                           21.9
                                                                                                                                                       surgery.                                    2.1
                                                                                                                                                                Causative organisms were methicillin-sensitive   Staphylococcus-0.63*
                                                                                                                                                                                                                                aureus (n ¼ 3), Staphylococcus epidermidis (n ¼ 1),
          PA-IVS/PVS                              3.1                      4.3                                  without IE during follow-up.
          DORV                         Severe 3.1
                                               procedural complications
                                                              6.4       occurred in 2                          pa- Kaplan-Meier
                                                                                                                       Death during curves
                                                                                                                                    follow-up of
                                                                                                                                              (%) the cumulative
                                                                                                                                                             3.1
                                                                                                                                                       Streptococcus  mitis (n ¼ 1),
                                                                                                                                                                             IE inci-
                                                                                                                                                                                     Aerococcus    2.1
                                                                                                                                                                                                viridans (n ¼ 1),             -0.06
                                                                                                                                                                                                                  Corynebacterium  striatum (n ¼ 1), and Haemophilus influenzae
                                                                                                                                                                            (n ¼ 1). All 8 cases occurred after Melody PPVI (25.0% vs. 0.0%). The incidence of IE was 5.7% (95% confidence interval:
      RVOT (%)                      tients (2.5%). One patient died of massive hemo-
                                                                                  dence were plotted using the date of  PPVI
                                                                                                                     2.9%        asperthe
                                                                                                                          to 11.4%)         entry
                                                                                                                                       person-year after Melody PPVI. The Kaplan-Meier cumulative incidence of IE with Melody PPVI was
          Native RVOT                             3.1                      25.5                                            Values are time
                                                                                                                                      median since
                                                                                                                                             [interquartile range]
                                    thorax due  to perforation                       date
                                                                  of a distal pulmonary                                  and   the                    PPVI     as orthe
                                                                                                                                                                     %. Standardized
                                                                                                                                                                     24.0%time        difference
                                                                                                                                                                                  scale.
                                                                                                                                                                           (95% confidence           computed
                                                                                                                                                                                             The12.2%
                                                                                                                                                                                          interval:            as the
                                                                                                                                                                                                        to 43.9%)  afterdifference
                                                                                                                                                                                                                         4 years and in means
                                                                                                                                                                                                                                     30.1% (95%orconfidence interval: 15.8% to 52.5%)
          Bioprosthesis                     9.4              23.4                                                           proportions divided by the SE. *Significant after
                                                                                                                                                                        imbalance.
                                                                                                                                                                             6 years, compared with 0.0% with the Sapien PPVI after 4 years (p < 0.04 by log-rank test). There was a trend
                                                                                                                 right censor was the date of IE, valve      replacement,
          Homograft                 branch     during
                                                25.0       Sapien    valve
                                                                         31.9PPVI      over a Lunderquist                                          toward a higher incidence of IE in the first 20 patients with Melody PPVI (who received prophylactic antibiotics during the
                                                                                                                              DORV ¼ double-outlet right ventricle; PA-IVS ¼ pulmonary atresia with intact ventricular septum; PPVI ¼
          Conduits                               62.5                      19.2
                                                                                       heart transplantation,          death, or follow-up
                                                                                              percutaneous pulmonary valve implantation; procedure only) andcomple-
                                                                                                                                            PVS ¼ pulmonary  in patients who had percutaneous interventions, dental care, or noncardiac surgery after PPVI.
                                                                                                                                                                  valve stenosis; RVOT ¼ right ventricle outflow
      RVOT lesion (%)
                                    guidewire. In the other patient, who had a mechanical
                                                                                       tion. Differences      in  incidence        were      evaluated
                                                                                              tract; TGA ¼ transposition of the great arteries.                  using
                                                                                                                                                                            CONCLUSIONS IE after PPVI may be less common with the Sapien compared with the Melody valve.
          Stenosis                  aortic valve,
                                             84.4 a large femoral
                                                             50.0 hematoma developed
                                                                                  the log-rank test. The Kaplan-Meier   method
                                                                                                                    (J Am               was
                                                                                                                          Coll Cardiol Intv    also © 2017 by the American College of Cardiology Foundation.
                                                                                                                                            2017;10:510–7)
          Regurgitation                          0.0                       35.7                                  used to assess the cumulative incidences of pulmo-
          Mixed                                  15.6                      14.3                                  nary valve replacement and of death, with the date of
      Pre-stenting (%)
     1-stage
Hascoet      PPVIJACC
          et al.  (%) Int. 2017
      No. of stents (%)
          0
                                                 90.6
                                                 87.5
                                                                           93.6
                                                                           87.2
                                                                                                0.11
                                                                                                0.01

                                        FIGURE 1 Kaplan-Meier Cumulative Incidences of Death and Pulmonary
                                                 9.4                       6.4
                                                                                                           Valve
                                                                                                                 PPVI as the entry date and the time since
                                                                                                  time scale and with the right censor set
                                                                                                                 Replacement and Kaplan-Meier
                                                                                                                                       outflow
                                                                                                                                             geryasforthe
                                                                                                                                               Cumulative
                                                                                                                                               tract
                                                                                                  valve replacement, heart transplantation, death,   (RVOT).
                                                                                                                                                              PPVI as the
                                                                                                                                                                         P       ercutaneous pulmonary valve implantation
                                                                                                                                                       (PPVI) has emerged as an alternative to sur-
                                                                                                                                                                                                                                          certification in 2006 and Food and Drug Administra-
                                                                                                                                                                                                                                          tion approval in 2010 for PPVI. The Sapien valve
                                                                                                                                                            date ofthe right ventricular (Edwards SAPIEN pulmonic transcatheter heart valve,
                                                                                                                                                       reconstructing
                                                                                                                                                               Incidences
                                                                                                                                                              PPVI  was firstofdescribed
                                                                                                                                                                    or          Infective
                                                                                                                                                                                        in Endocarditis
                                                                                                                                                                                            Edwards Lifesciences, Irvine, California) was used
                                                                                                                                                                         2000 (1) and since then many studies have supported              initially for transcatheter aortic valve replacement
          1                                      90.6                      85.1                                  end of follow-up. Differences itsinefficacy
                                                                                                                                                       incidence        were
                                                                                                                                                            (2–13) The Melody valve (Medtronic Inc.,                                      and subsequently licensed for PPVI (Europe, 2010;
VALVE(SAPIEN(

Hascoet et al. JACC Int. 2017
VALVE(SAPIEN(

Edwards SAPIEN XT Transcatheter Heart Valve with the NovaFlex1Deliv-
ery System. Vol. 2016. https://www.accessdata.fda. gov/cdrh_docs/pdf13/
p130009s037d.pdf.
(PREVENTION(N(EDUCATION((

                                                                PROCEDURE
 PRE-IMPLANTATION
                                                                 Salle hybride
    Patient et famille
                                                             Optimisation gradient
Depistage foyer infectieux                                         résiduel
  Bilan dentaire et ORL                                    Antibioprophylaxie per +/-
                                                                post procédure

                                POST-IMPLANTATION
               Education patient, parents, médecin traitant, dentiste
                             Anti-aggrégants au long cours
                                Antibioprophylaxie à vie
FACTEURS(AGGRAVANTS(

                   Portes d’entrée évitables

                       Manque observance

                      Déficiences mentales

                 Education - Prophylaxie EI

Bauer et al. Int Jour Cardio
2017
Buber et al. Circ Intrv 2013
Myocardites,aiguës,
       ,
Généralités(
• Série(autopsique:(iden4fica4on(d’une(myocardite(dans(8,6%(à(
  12%(en(cas(de(mort(subite(
• Evolu4on(vers(la(CMD(possible(et(non(excep4onnelle(
• Physiopathologie(

                                              Kindermann, JACC 2012
Kociol et al
                                               E4ologies(des(myocardites(                                                         Recognition and Management of Fulminant Myocarditis

                                                                                                                                                                                         CLINICAL STATEMENTS
                                                                                                                                                                                            AND GUIDELINES
        Downloaded from http

Kociol et al                   Figure 5. Causes of lymphocytic myocarditis.                                                             Recognition and Management of Fulminant Myocarditis
                               Diagram demonstrating the primary causes and associated subcategories of lymphocytic myocarditis. GCM indicates giant cell myocarditis; IBD, inflammatory
                               bowel disease; RA, rheumatoid arthritis; and SLE, systemic lupus erythematosus. Reprinted from Trachtenberg and Hare.99 Copyright © 2017, American Heart
                               Association, Inc.
Diagnos4c(posi4f(
(

• Clinique,évocatrice:,,
   – douleur(thoracique,(fièvre((30%),(tachycardie((58%),(
      dyspnée((68%)(
   – Choc(cardiogénique((Forme(fulminante,(5N10/1(mill(
      d’habitants/an)(
   – Mort(subite((TDR(ou(TDC)(
• Biologie:, ,       ,     ,Troponine,(BNP(ou(NNproBNP(
•   ECG:,      ,        ,  ,infarctus(du(myocarde(
•   Echocardiographie:,    ,dysfonc4on(modérée(à(sévère(
•   IRM(et/ou(biopsie,endomyocardique,(BEM)(
•   Sérologies,virales( (  (peu(u4les(en(pra4que(clinique(
    (
Critères(de(Dallas(historiques((1986)(

Infiltration lymphocytaire
Signe de nécrose non ischémique

               Magnani JW et al. Circulation 2006   Cooper LT et al. Circulation 2007
Problèmes(des(critères(de(Dallas(
• Myocardite(avec(aQeinte(hétérogène(du(myocarde(N
  >(Biopsies(mul4ples(>(5(
• Geste,invasif:,mortalité(0,5%,(complica4ons(5%:(
  perfora4on(cardiaque,(hémopéricarde,(tamponnade(
• Geste(plus(risqué(chez(le(nourrisson(
• Variabilité(d’interpréta4on(même(entre(experts(
                                       1916       Cooper et al.
                                                  Endomyocardial Biopsy in Cardiovascular Disease

                                       Table 1. Risks Associated With Endomyocardial Biopsy in         failure, includi

• «(Goldstandard(»(mais(discutée(++(
                                       546 Procedures                                                  potentially hear
                                       Overall 33 complications (6%)
                                       Sheath insertion 15 (2.7%)                                      Analysis o
                                         12 (2.0%) arterial puncture during local anesthesia
                                         2 (0.4%) vasovagal reaction                                   EMB Proces
                                         1 (0.2%) prolonged venous oozing after sheath removal
                                                                                                       Samples should
                                                                                                       ventricular sept
                                       Biopsy procedure 18 (3.3%)
                                                                                                       range from 5 to
                                         6 (1.1%) arrhythmia
                                                                                                       and each samp
                                         5 (1.0%) conduction abnormalities                             must be handle
                                         4 (0.7%) possible perforation (pain)                          ferred from the
                                         3 (0.5%) definite perforation (pericardial fluid)             formalin) by u
                                            2 of 3 patients with definite perforation died             (21,22). The f
                                         Data derived from Deckers et al (20).                         prevent contrac
                                                                                                          The clinical
                                       (0.03%). Deckers et al (20) prospectively recorded compli-      samples are rem
                                       cations from 546 consecutive right heart biopsy procedures in   least 4 to 5 s
Indication d’une BEM
                      Kociol et al                                                                   Recognition and Management of Fulminant Myocarditis
CLINICAL STATEMENTS
   AND GUIDELINES

                                                                                                           Figure 3. Indications for endomyocardial
                                                                                                           biopsy (EMB).
                                                                                                           Guideline-based algorithm for whether EMB
                                                                                                           is indicated. COR indicates Class of Recom-
                                                                                                           mendation; LOE, Level of Evidence; and MRI,
                                                                                                           magnetic resonance imaging. *Usually a dilated
                                                                                                           cardiomyopathy. Fulminant myocarditis may
                                                                                                           have normal end-diastolic diameter with mildly
                                                                                                           thickened walls. Exclude ischemic, hemodynam-
                                                                                                           ic (valvular, hypertensive), metabolic, and toxic
                                                                                                           causes of cardiomyopathy as indicated clinically.
                                                                                                           Reprinted from Bozkurt et al.3 Copyright ©
                                                                                                           2016, American Heart Association, Inc.

                                                                                  despite normalization of cardiac enzymes and biomark-
                      EMB, CORONARY ANGIOGRAPHY,                                  ers.78 EMB can be considered the primary diagnostic
                      AND INVASIVE
                            Circ 2020
                                      HEMODYNAMICS                                strategy76,79 when     magnetic resonance imaging is not
                      Kociol et al                                                               Recognition and Management of Fulminant Myocarditis
                      In the setting of cardiogenic shock, right-sided heart      possible (eg, shock, presence of metal devices) if expe-
                      catheterization and coronary angiography are essential to   rienced operators and cardiac pathologists are readily
                      guide management strategies. The decision to perform        available. According to guidelines, however, indications
  S
Lake(Louise(Criteria:((IRM((

Trois(séquences(IRM(contribu4ves:(
• 1.(Œdème(en(T2(
• 2.(rehaussement(précoce(du(myocarde(
• 3.(rehaussement(tardif(du(myocarde(

• Diagnos4c(posi4f(si(>(2(critères(:(
   – Hypersignal(T2(
   – Ra4o(Signal(myocarde(/(muscle(périph(augmenté(après(injec4on(de(
     Gadolinium(
   – Hypersignal(en(rehaussement(tardif(

• Refaire(IRM(à(1N2(semaines(si:(
   – 0(critère(mais(symptômes(trop(récents,(forte(suspicion(clinique(
   – 1(seul(critère(présent(

                                              Friedrich MG et al. JACC 2009
Traitement(en(fonc4on(de(la(forme(clinique(
,

• Myocardite,segmentaire,focale:,Repos(
• Myocardite,aiguë,diffuse,chez,l’enfant(
    – Surveillance(+/N(assistance(circulatoire((HNF)(
    – Traitement(d’aQaque:((
       • Immunomodulateurs,(immunosuppresseurs,(An4N
         inflammatoire,(immunoadsorp4on(
• Myocardite,fulminante,
    – PEC(du(choc(cardiogénique(
    – (Traitement(spécifique(en(fonc4on(du(type(histologique)(

• Myocardite,chronique,acTve(
    – Discuter(immunosupresseurs(
Traitement(
ce(qui(est(admis(…(
Traitement(de(l’insuffisance(cardiaque(
• Selon,les,guidelines,
• Selon,la,classe,foncTonnelle,NYHA,
   – IEC(
   – Diuré4ques(
   – BNbloquant(
   – ARA(II(
   (

• Formes,sévères:,Prise,en,charge,en,réanimaTon,
   – Traitement,«,agressif,»,
   – Assistance,circulatoire,(>(60(à(80(%(survivants(et(
     récupéra4on(ad(integrum(possible)(
   – Drogues,Inotropes,posiTves,et,héparine!,
                                          Kindermann, et al., JACC 2012
                                          Amabile et al. Heart 2006
Traitement(de(la(myocardite(fulminante(
                    Kociol et al                                                                                               Recognition and Management of Fulminant Myocarditis

                                                                                                                                                                                          CLINICAL STATEMENTS
                                                                                                                                                                                             AND GUIDELINES
Downloaded from h

                                                                                                                                                                              Circ 2020
                    Figure 4. General approach to initial stabilization of patients in cardiogenic shock.
                    ACS indicates acute coronary syndrome; CABG, coronary artery bypass grafting procedure; ECMO, extracorporeal membrane oxygenation; IABP, intra-aortic bal-
                    loon pump; MCS, mechanical circulatory support; PCI, percutaneous coronary intervention; and VAD, ventricular assist device. Reprinted from van Diepen et al.86
Traitement(
ce(qui(est(discuté(…(
          (
  Immunoglobulines(
  An4(inflammatoires(
      An4viraux(
 Immunosuppresseurs(
          (
Immunosuppressive Treatment for
                  Myocarditis in the Pediatric
                                     He et al.                                                                                                             Immunosuppressive Treatment for Myocarditis in the Pediatric

                  Population: A Meta-Analysis
                                     TABLE 1 | Characteristics of studies included in the meta-analysis.

                                     Study                N       Age                 Study       IMSA                IMSA dosage, time of            Follow-up             Observed             Inclusion criteria
                                                                                      methodology                     IMSA start                                            variables
                  Bing He* , Xiaoou Li and Dan Li
                                     Camargo et al.       50      5 months−15         PNCT             P, CyA         P & A: 2.5 mg/kg/d, 1           8.4±1.2 months        LVEDD, LVEF,         Active myocarditis based
                  Department (9)of Pediatrics, Renmin
                                                years Hospital of Wuhan University,     Wuhan,
                                                                         week; 2.0 mg/kg/d, 3  China                                                                        PWP, CI, HR          on EMB findings
                                                                                                                      weeks; 1.5 mg/kg/d, 4
                                                                                                                      weeks
                                                                                                                      Cy: 1.5 mg/kg/d, 1 week;
                  The use of immunosuppressants in the treatment of myocarditis in children remains                   1.0 mg/kg/d, 7 weeks;
                                                                                                                      0.5 mg/kg/d, 1 week
                  controversial.
                           Aziz et al. (6)
                                              The
                                                68
                                                         aim of RCTthis meta-analysis
                                                        3.7 ± 2.9                           P               2 mg/kg/d, 1 month
                                                                                                                                     is to  15.1±9.2
                                                                                                                                                      summarize    LVEDD, LVESD,
                                                                                                                                                                                    the            current empirical
                                                                                                                                                                                      Duration of symptoms
                  evidence for immunosuppressive        years                                  treatment for myocarditis                    months                 LVEF in the for
studies of
                                       Department  arePediatrics,
                                                       included inRenmin
                                                                   the present study,ofofWuhan
                                                                          Hospital        which University,
                                                                                                only one Wuhan,
                                                                                                            treatmentChina
                                                                                                                      in the short term may significantly improve LVEF,
                                           is an RCT study; this might result in a lack of statistical          reduce LVEDD, and reduce the risk of death and heart transplant
                                           power to detect a significant difference in the treatment effect.    in pediatric population with myocarditis. Although this meta-
                                           Moreover, we were able to ascertain publication bias in only         analysis reported beneficial outcomes with immunosuppressive
                                       The four
                                             use    ofstudies,
                                                         immunosuppressants
                                                                which means only four of sixin     thedata  treatment
                                                                                                                therapy, theof    myocarditis           in children         remains
                             Immunosuppressive Treatment for
                                                of six
                                       controversial.        The aimDueofto this
                                           findings of this meta-analysis.
                                                                                                studies’
                                           could be merged, which may have impacted the analysis of the
                                                                                           meta-analysis
                                                                                   the included studies’ lack
                                                                                                                              results have to be interpreted cautiously because
                                                                                                                one RCT was included in this meta-analysis; more large-scale
                                                                                                                   is to summarize the current empirical
                                                                                                                RCTs are required in the future.
                                                                                                                                                                                only

                             Myocarditis in the Pediatric
                                       evidence      for follow-up
                                           of long-term    immunosuppressive                 treatment
                                                                     (only two studies had median    follow- for myocarditis in the pediatric population.
                                           up > 1 year), their inferences can only be applied to short-         AUTHOR CONTRIBUTIONS
                                       We term
                                           searched         PubMed,          MEDLINE,          and Embase            for articles to identify studies analyzing
                             Population: A Meta-Analysis
                                                 outcomes. In  this meta-analysis,

                                                   didn’t report
                                                                                   we couldn’t provide
                                           data of viral genome and histologic type, because the included
                                       the studies
                                            efficiency        of these
                                                                    immunosuppressive
                                                                         data even if this information
                                                                                                         exact

                                                                                                      treatment
                                                                                                          was
                                                                                                                BH is responsible for the provision of the overall idea and writing
                                                                                                                articles. in   the data
                                                                                                                          XL collects  pediatric
                                                                                                                                          and modifiespopulation.            Pooled
                                                                                                                                                         the paper. DL is responsible
                                           important to the therapy and prognosis. RCTs in the future           for statistical analysis.
                             Bing estimates wereLigenerated
                                                   and Dan using                                    fixed- or random-effect models. Heterogeneity within
                                 • Groupe d’enfants avec immunosuppresseurs
                                  He* , Xiaoou              Li
                                studies
                          Department        was assessed
                                      of Pediatrics,          Renmin using    Hospital    Cochran’s
                                                                                             of Wuhan University,Q and I2Wuhan,   statistics.  ChinaFunnel plots and Begg’s rank

                          The were
                                   amélioration significative:
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                                                                                                                                                                              M, Kuhl   U, et al.risk (RR):

 ticle
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                                    (2004)      2.69,
                                            90:1167–71.      8.35]. No significant heterogeneity
                                                        doi: 10.1136/hrt.2003.026641                             disease: a scientificacross
                                                                                                                                       statement fromthe        Population: A Meta-Analysi
                                                                                                                 The role of endomyocardial biopsy in the management of cardiovascular
                                                                                                                                                               studies
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                                                                                                                                                                       Heart Association,
   Pediatric Cardiology,
                      There   was no evidence of publication bias when assessed by Begg’s test.
 apondence:
   section of the journal  Keywords: immunosuppressive treatment, myocarditis, pediatric, cardiac function, meta-analysis                                       Bing He*, Xiaoou Li and Dan Li
      Bing in
  Frontiers He          Conclusions:
               Pediatrics                                There may
                                               be a possible   benefit, in the
                              Frontiers in Pediatrics | www.frontiersin.org
                                                         8                               short
                                                                              November 2019  | Volume 7 |term,
                                                                                                         Article 430    to the
                                                                          Department of Pediatrics, Renmin Hospital of Wuhan University, Wuhan, China
 1@sina.com
                   addition of immunosuppressive therapy in the management of myocarditis in the
 ceived: 22 May 2019
Traitement(de(la(myocardite(fulminante(
                      Kociol et al                                                                                                  Recognition and Management of Fulminant Myocarditis

                      Table 7.   Major Myocarditis Subtypes Resulting in a Fulminant Presentation
CLINICAL STATEMENTS

                        Subtype                               H&E Findings                           Clinical Manifestations                                 Treatment
   AND GUIDELINES

                        Fulminant lymphocytic     Extensive dense lymphocytic infiltrate      Acute heart failure rapidly leading          Treatment is primarily supportive; circulatory
                        myocarditis               with associated myonecrosis. May have       to cardiogenic shock, conduction             support as needed to prevent MOSF. Some
                                                  occasional isolated multinucleated giant    abnormalities, or ventricular                evidence that in the absence of cardiotropic
                                                  cells or eosinophils.                       arrhythmias/SCD. Chest pain.                 viral genome by PCR, steroids may be helpful
                        GCM                       Extensive mixed inflammatory infiltrate     Acute heart failure caused by systolic       Treatments consists of multimodality therapy
                                                  characterized by the presence of several    dysfunction, myocardial restriction,         and should be implemented after a tissue
                                                  multinucleated giant cells (usually         or both. Conduction abnormalities,           diagnosis has been confirmed. Usual therapy
                                                  present after 1–2 wk), eosinophils,         including CHB and EMD; ventricular           includes a combination of a high-dose
                                                  monocytes, and macrophages in the           arrhythmias, including sustained VT/VF       steroids, a calcineurin inhibitor (such as
                                                  absence of noncaseating granulomas.         and SCD. Tends to comigrate with other       cyclosporine), and an antimetabolite such
                                                  Edema and extensive myonecrosis often       autoimmune diseases.                         as azathioprine. Cytolytic therapy (purified
                                                  present.                                                                                 rabbit-derived polyclonal IgG directed at
                                                                                                                                           human thymocytes) used for suppression of
                                                                                                                                           life-threatening GCM has been reported.
                        Acute NEM                 Extensive inflammatory infiltration of      Acute heart failure/cardiogenic              Identify potential precipitant, especially if a
                                                  the myocardium with mononuclear cells       shock. May present with a restrictive        drug hypersensitivity (Table 5). High-dose
                                                  and eosinophils. Associated myonecrosis     cardiomyopathy. Prothrombotic                steroids. Anticoagulation. Often presents
                                                  or fibrosis. On EM, may see eosinophil      intracardiac state. Peripheral               with ST-segment elevations and chest
                                                  degranulation and deposition of major       eosinophilia may or may not be               pain mimicking an ST-segment–elevation
                                                  basic protein.                              present. Recent viral infection or new       myocardial infarction. Rapid angiography,
                                                                                              medication.                                  EMB with subsequent circulatory support,
                                                                                                                                           and initiation of high-dose intravenous
                                                                                                                                           corticosteroids can be lifesaving.
                        ICI myocarditis           Newly identified lymphocytic myocarditis    Acute heart failure, cardiogenic shock,      Treatment includes immediate cessation
                                                  resulting from the introduction of          and atrial fibrillation developing           of therapy, high-dose corticosteroids (1 g
                                                  novel chemotherapeutic agents that          soon after ICI therapy is started and        solumedrol intravenously daily for 3 d and
                                                  unleash inhibited antitumor T cells,        generally more severe with combination       then 2 mg/kg prednisone daily to start,
                                                  which also may infiltrate and attack        ICI therapy. Typically occurs early in       followed by a slow wean) and initiation of
                                                  the myocardium. Histopathology              treatment and has a fulminant course.        an angiotensin receptor blocker or sacubitril/
                                                  consistent with lymphocytic infiltrate                                                   valsartan. May initially need MCS.
                                                  and myocardial necrosis.

                        CHB indicates complete heart block; EM, electron microscopy; EMB, endomyocardial biopsy; EMD, electromechanical dissociation; GCM, giant cell myocarditis;
                                                                                                                                                                                Circ 2020
         Downloaded

                      H&E, hematoxylin and eosin; ICI, immune checkpoint inhibitor; IgG, immunoglobulin G; MCS, mechanical circulatory support; MOSF, multiorgan system failure;
                      NEM, necrotizing eosinophilic myocarditis; PCR, polymerase chain reaction; SCD, sudden cardiac death; and VT/VF, ventricular tachyarrhythmia/ventricular fibrillation.
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