Pedia_Nr-4_2010_Art-3 Urgente Cardio-Vasculare La Sugar

8/6/2019 Pedia_Nr-4_2010_Art-3 Urgente Cardio-Vasculare La Sugar

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REVISTA ROMNDE PEDIATRIE VOLUMUL LIX, NR. 4, AN 2010 249

Adresa de coresponden:Dr. Georgiana Russu, Spitalul Clinic de Urgene pentru Copii Sf. Maria, Str. Vasile Lupu, Nr. 62, Iai

URGENE CARDIOVASCULARELA COPILUL 0-1 AN

Dr. A.G. Dimitriu1, Dr. Georgiana Russu21Universitatea de Medicin i Farmacie Gr. T. Popa, Iai2Spitalul Clinic de Urgene pentru Copii Sf. Maria, Iai

REZUMAT

Urgenele cardiovasculare survin adeseori la nou-nscut i sugar cu o inciden i severitate superioaracelora prezente la alte vrste, fiind datorate n primul rnd anomaliilor cardiace congenitale, dar i unorafeciuni cardiace dobndite. La nou-nscut, malformaiile congenitale de cord cu manifestri de hipoxemierefractari/sau de insuficien cardio-circulatorie acuti manifestrile cardiace induse de suferina hipoxicperinatal pot avea un prognostic rezervat, ceea ce implic un diagnostic ct mai precoce i o terapie

adecvat prompt. Probleme dificile de diagnostic i tratament pot aprea i n insuficiena cardiaccongestiv, miocardit, tulburrile de ritm cardiac. Asemenea dificulti n managementul urgenelor cardiaceale primului an de via sunt amplificate cnd survin la un sugar la care afectarea cardiac nu era precizatanterior, situaie frecvent ntlnit n practic.

Cuvinte cheie: nou-nscut, sugar, urgene cardiace, malformaii congenitale de cord,disritmii neonatale

REFERATE GENERALE

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Copiii cu diferite afeciuni cardiovasculare pot fiinternai n serviciile de urgen chiar de la natere,din primele zile de via sau i mai trziu n cursul

primului an, ca urmare a unei afec iuni diagnosticaten perioada neonatal sau a unei afeciuni cardiacecare, dei congenital, nu a fost nc diagnosticat.Primul an de via se caracterizeaz prin incidenai severitatea mult crescut a urgenelor survenite lanivelul diverselor sisteme i organe, comparativ cualte vrste. Modificrile hemodinamice care au loc

postnatal, cu trecerea la circulaia de tip adult, audurat variabil, n general pn spre vrsta de 3 luni,mai ales n prezena unor anomalii structurale cardiacecongenitalei pot favoriza manifestri severe neonatale

de tipul detresei neonatale de origine cardiac.Manifestrile clinice de suferincardiovascularpot fi remarcate chiar de la prima prezentare sau potsurveni ca o complicaie a unei boli diagnosticateanterior (1).

DETRESE VITALE NEONATALE

DE ETIOLOGIE CARDIAC

n momentul naterii i ulterior n primele luni postnatal survin o serie de modificri fiziologice

cardiovasculare: dispariia circulaiei placentare; cre-

terea debitului sanguin pulmonar prin scderearezistenelor vasculare pulmonare (ameliorareaoxigenrii sngelui); nchiderea canalului arterial;ocluzia foramen ovale; maturarea vascular pul-monar. n cazul existenei unor malformaii con-genitale cardiace (MCC), pot surveni perturbrihemo-dinamice majore cu manifestri clinice dese-ori severe (2).

Cardiopatii congenitale cu manifestriA.

severe neonatale

Hipoxemia refractar izolat. Diagnosticul1.diferenial include alte cauze de cianoz laaceast vrst (3): hipoventilaie de originenervos central, boli respiratorii, methemo-

globinemie, aport insufi

cient de O2, altecauze: hipoglicemie, policitemie.Sindromul de insuficien cardiocirculatorie.2.Diagnosticul diferenial cuprinde: cord in-demn sau malformat (tipul malformaiei) (4),afectare pericardic, prezena i severitateahipertensiunii arteriale pulmonare (HTAP).Sindromul de hipertensiune arterial pul-B.

monar persistent (HTAPP) la nou-nscut

Disritmii cardiace severeC.

Miocardopatii neonatale de etiologie ische-D.

mic, miocardite


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REVISTA ROMNDE PEDIATRIE VOLUMUL LIX, NR. 4, AN 2010250

Manifestrile clinice care orienteazdiagnosticulctre o MCC la nou-nscut sau n primele luni devia sunt: dificulti de alimentaie (copilul pri-mete prea lent alimentaia, sau n cantitate insu-ficient, prezint cianozi transpiraii i/sau polip-nee n timpul alimentaiei, disfagie, tuse, vrsturi;

tahipnee superficial constant (inclusiv n somn);stridor; sindrom de detres respiratorie; tahicardiesau bradicardie, alte tulburri de ritm; accese decianoz sau tent cianotic generalizat; paloare,tegumente umede; extremiti reci, perfuzie cuta-nat diminuat; tulburri de comportament: apatiesau instabilitate.

Cardiopatii congenitale cu manifestri se-A.

vere neonatale

1. Hipoxemia refractar izolat. Semnul clinicmajor al acestei entiti este cianoza izolat, fr

detres respiratorie sau insuficien respiratorie,insensibil la testul de hiperoxie. Uneori copilul arestare general aparent foarte bun, iar auscultaiacordului nu deceleaz sufluri patologice. n general,deteriorarea strii generale survine rapid n absenaunei terapii medicale i mai ales chirurgicale pre-coce (4,7). Aspectul radiologic al cordului i circu-laiei pulmonare orienteaz diagnosticul (Tabelul 1).

Alte cauze de cianoz la nou-nscut: metabolice(hipoglicemie, hipocalcemie); boala hemolitic anou-nscutului, neurologice (asfixia, hemoragiaintracranian, meningite), tulburri neuromusculare(boala Werdnig-Hoffman); hipotermie, sepsis, me-themoglobinemie, depresie respiratorie secundarmedicaiei materne (narcotice, sulfat de magneziu),obstrucia cilor respiratorii superioare (atrezie coa-nal, stenoz traheal, gu, sindrom Pierre Robin),

policitemie, sindrom de hipervscozitate sanguin,oc neonatal, insuficien cardiac congestiv seve-r; defecte congenitale: hernie diafragmatic, plmn

hipoplastic, emfizem lobar congenital, malformaiechistic adenomatoas pulmonar.

Tratament: atrioseptostomie Rashkind (transpo-ziia de vase mari), meninerea deschis a canaluluiarterial (prin perfuzare de prostaglandin E1 ncardiopatiile ductodependente: atrezie tricuspidian

cu sept interventricular intact, atrezie tricuspidiancu defect septal ventriculari atrezie pulmonar.atrezie pulmonar (sau stenoz pulmonarsever) cu sept interventricular intact;sindromul cordului stng hipoplazic (atreziemitrali atrezie de aort cu ventricul stngvirtual) transpoziie de vase mari cu sept in-terventricular intact; cateterism interven-ional-valvuloplastie pulmonar n stenoze

pulmonare severe (5).2. Sindromul de insuficien cardiocirculatorie

determin, n general, agravare rapid a strii ge-nerale i chiar evoluie spre deces. Tabloul clinicconst n manifestri respiratorii edem pulmonaracut i/sau acidoz: tahipnee postprandial sau per-manent, detres respiratorie important, care de-termin epuizarea nou-nscutului, hepatomegalie,tahicardie, asurzirea zgomotelor cardiace, zgomotde galop, deseori semne de colaps periferic tent

palid cenu ie, extremiti reci, cianotice/marmorate,puls periferic tahicardic, filiform sau neperceptibil,alungirea timpului de recolorare capilar, hipoten-siune arterial, oligoanurie, cianoz (cel mai adesearedus, se amelioreaz prin administrarea de oxi-gen) (7). 90% dintre cazurile de insuficien car-diac la copii apar n primul an de via, etiologia

principalfiind reprezentat de MCC (Tabelul 2).n afara simptomatologiei cardiace, exist di-

ferite semne i simptome care orienteaz diag-nosticul la nou-nscutul i sugarul cu insuficiencardiac (7,8) (Tabelul 3).

TABELUL 1.Elemente de orientare n hipoxemia refractar la nou-nscut (1,2,4,7)

Aspectul circulaiei pulmonare Aspectul cordului Cardiopatia congenital

Vascularizaie pulmonarcrescut sau normal

Cord de volum iniial normal, aspectovoid, n timp cardiomegalie

TMV cu sept inerventricular intact

Staz venoas pulmonar Cord mic ntoarcere venoas pulmonar anormal totalblocat

Vascularizaie pulmonarsrac

Cord normal Atrezie pulmonar +DSVCord n sabot Tetralogia FallotCord n sabot Atrezie tricuspid (EKG: axa QRS=-30,

suprancrcare VS)Cardiopatii complexe cu atrezie pulmonar

Cardiomegalie moderat

(ICT=0,60-0,65)

Atrezie sau stenoz sever pulmonar cu SIV

intact (EKG: axaQRS=+60-120, suprancrcareVD, unde P ample)Insuficien tricuspidian

Cardiomegalie important(ICT>0,75)

Anomalie Ebstein (EKG: QRS mici, bloc ram drept,tulburri de ritm sau conducere)


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Tratament: diuretice, inotropice pozitive (digo-xin, dopamin, dobutamin), oxigenoterapie, ven-tilaie asistat n cazurile severe, corecia tulburrilormetabolice care pot agrava asistolia acut (acidoz,hipoglicemie), terapia farmacologic de nchidere acanalului arterial cu unt important (fenilbutazona),obstruciile stngi severe (coarctaia de aort) duc-todependente (perfuzie cu PGE1), terapie medica-mentoas a tahiaritmiilor, pacemaker pentru bradi-aritmii severe (bloc a.v. grad III congenital), tratament

chirurgical sau cardiologie intervenional (8).Cauze particulare de insuficien cardiac lanou-nscut (n afara MCC) sunt (2): asfixia lanatere, hipoxie, hipoglicemie, hipocalcemie, ane-mie, acidoz, sepsis.

Cardiomiopatia postasfixic este o entitate defi-nit clinic prin hipotensiune arterial, tahicardie,raluri de staz pulmonar, hepatomegalie, suflusistolic de insuficien mitral sau/i tricuspidian,galop, accentuarea zgomotului II n focarul pulmo-narei. Paraclinic, EKG relev modificri ale seg-

mentului ST/unde T de tip ischemie, cretereafraciunii MB a CPK, radiologic staz pulmonari cardiomegalie, i creterea PVC. EcocardiografiaDoppler deceleaz insuficien tricuspidian, sc-derea fraciei de scurtare i de ejecie a ventricululuistng, prelungirea intervalelor de timp sistolic,disfuncie diastolic a ventriculului stng. Anomaliimetabolice asociate: acidozmetabolic, hipoxemie,hipoglicemie, hipocalcemie, policitemie. Tratament:

TABELUL 2. MCC complicate cu insuficien cardiac nfuncie de vrst (adaptat dup 7, 8, 9)

Vrsta Malformaia congenital de cord

Nou-nscut Stenoz aortic, insuficien pulmonarsau tricuspidian severe, tetralogieFallot cu absena valvei pulmonare

Primele 7 zile

de viaStenoz aortic critic, sindromul

cordului stng hipoplazic, ntoarcerevenoas pulmonar anormal total,trunchi arterial comun

2-8 sptmni Tetralogie Fallot noncianotic, canalatrioventricular, coarctaie de aort,canal arterial persistent, defect septalventricular

2-6 luni Anomalie de origine a coronarei stngi,canal arterial persistent, defect septalventricular

TABELUL 3. Elemente de diagnostic n MCC la nou-nscutuli sugarul cu insuficien cardiac

Aspectul cordului Alte manifestri orientative MCC

Cardiomegalie(ICT=0,60-0,70)

Suflu sistolic; ECG HVS; colaps precoce Stenoz aortic valvular medie/severDiferen de puls i TA n favoarea membrelorsuperioare

Coarctaie de aort izolat

Insuficien cardiac precoce i diferen depuls

Sindrom de coarctaie

ECG: deviaie axial stng (-90 -120) Canal atrioventricular completSuflu sistolo-diastolic, puls amplu Canal arterial larg permeabil (prematur)Suflu sistolic i adesea diastolic, puls amplu,cianoz moderat

Trunchi arterial comun

Sufluri diverse/fr suflu Ventricul unicCardiomegalieimportant (ICT>0,75)

Puls carotidian sltre, suflu continuu (celecerebrale)

Fistule arteriovenoase sistemice (cerebrale,hepatice, periferice, angiom placentar)

Infecie septicemic Pericardite purulente (rar serofibrinoaseidiopatice sau chilopericard)

Semne evocatoare pentru scleroza tuberoasBourneville

Tumori-rabdomioame

Context familial (diabet), hipoxie la natere Cardiomiopatii (diabetice, metabolice,ischemice)

Clinic i ECG FC>220/min Tahicardii heterotopeCord mrime variabil Ecografie fetal: anasarc fetoplacentar Tahicardie fetal, flutter atrial

Clinic i ECG FC


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pruden prin perfuzie cu dopamin n doze maimari 15-20 g/kg/minut, doze la care se meninei efectul ei inotrop pozitiv. Meninerea hipotensiuniiarteriale se face cu epinefrin 0,05-0,5 g/kg/minut

perfuzie intravenoas (4).Un aspect frecvent ntlnit n practica curent

este prezentarea la serviciul de urgen a unui sugarcu simptomatologie nespecific, fr antecedentecunoscute de cardiopatie. Afeciunile cardiace cumanifestri severe la nou-nscut i sugari care seadreseaz cel mai frecvent la serviciul de urgene

pentru copii pot fi mprite n: boli cardiace con-genitale structurale; aritmiile cardiace; boli cardiacedobndite miocardita.

Clasificarea MCC n funcie de modificrilefiziopatologice induse de anomaliile structurale:

MCC cu cianoz;

MCC cu obstrucie a ejeciei VS sau insu-ficiena funcional a VS care prezint semnede oc datorit scderii fluxului de snge sis-temic;anomalia de origine a coronarei stngi, care

poate prezenta infarct miocardic i oc;insuficiena cardiac congestiv.

MCC cianogene

Leziunile cele mai frecvente care determin

cianoz includ: atrezie pulmonar sau stenoz pul-monar sever, tetralogie Fallot forma cianotic,retur venos pulmonar total aberant, transpoziia devase mari, atrezia de tricuspid, trunchi arterialcomun (2, 3). De obicei aceti copii sunt diagnosticaila natere sau imediat nainte de externarea dinmaternitate, dar pot fi externai i nediagnosticai,iar canalul arterial permeabil poate avea un ase-menea debit, nct ofer suficient flux de snge

pentru circulaia pulmonar, permind meninereaunui nivel relativ ridicat de oxigenare pentru

circulaia sistemic i astfel mascheaz leziunile,iar hipoxia nu este evident clinic. n cele din urm,atunci cnd canalul arterial se nchide, urmeaz oscdere a fluxului de snge pulmonar, iar aceticopii devin acut hipoxici. Acest lucru se produce,n general n primele 2 sptmni de via, darimai trziu.

Cianoza poate fi neobservat la examenul fizic(copii cu anemie sau tegumente hiperpigmentate).Pulsoximetria este dificil de efectuat sau rezultatele

pot fi modificate la un copil agitat sau cu detres,

sau la care circulaia periferic este compromisdin cauza acidozei. Dac instalarea cianozei nu estesurprins la timp, aceasta fiind un indicatorfidel

pentru prezena hipoxemiei, copilul poate fi n ocsau se instaleaz insuficien multiorganic.

Pentru cianoza central, testul de hiperoxie esteesenial n cadrul diagnosticului diferenial etiological cianozei (cauze cardiace sau extracardiace) (8).

(Fi O2100% 5-10 minute). Atenie! creterea PaO2poate induce nchiderea canalului arterial, de aceeaeste necesar monitorizare clinici ecocardiogra-fic, fiind periculoas n cardiopatiile ductodepen-dente.

Radiografia cardiotoracic poate releva maimulte aspecte: creterea fluxului pulmonar n trans-

poziia marilor vase, retur venos pulmonar anormalparial, trunchi arterial comun; scderea fluxului pulmonar n atrezia de tricuspid cu sept intact,atrezia arterei pulmonare cu sept intact, tetralogieFallot, anomalie Ebstein, stenoz

pulmonar

critic

cu sept interventricular intact; staz venoas pul-monar n returul venos pulmonar anormal total,cord stng hipoplazic. Pentru diagnostic este esen-ial efectuarea EKG i ecocardiografiei.

La nou-nscut i la sugarul de vrst mic lacare intensificarea cianozei s-a datorat nchideriicanalului arterial, dup stabilizarea iniial cu per-meabilizarea cilor aeriene i asigurarea respiraiei(uneori prin intubaie), care poate rezolva hipoxiai detresa respiratorie sever, se pune problemaredeschiderii canalului arterial-perfuzii cu pros-taglandina E1 (PGE1), concomitent cu terapia o-cului. n acest moment, devine obligatorie solicitareaunui cardiolog pediatru ct mai curnd posibil

pentru diagnostic definitiv i stabilirea indicaiilorn continuare. Dac situaia pacientului o permite,se impune transferul pacientului ntr-un serviciu cuterapie intensiv cardiologici posibiliti de in-tervenie chirurgical paleativ sau reparatorie (8).

Leziuni cu obstrucia tractului de ejecie al VS iinsuficien ventricular

Aceste tipuri de MCC constituie alte leziuniductodependente pentru circulaia sistemic: coarc-taia de aorti stenoza aortic sever. n majoritateacazurilor cordul stng hipoplazic, de asemenea

TABELUL 4. Elemente de difereniere ntre cianozacentral (buze, limb, mucoase) i periferic (perioral,mini, picioare = acrocianoza)

Periferic(acrocianoz)

Elemente deurmrire

Central

Roz culoare: mucoase,limb, buze,

trunchi

albastr

Reci extremiti calde reciSczut perfuzie periferic normal sczutNormal PaO2, SaO2 sczutDe obicei maifavorabil(sepsis, oc,MCC)

prognostic necesit de obiceitratament deurgen (pulmonar,MCC, oc, sepsis)


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ductodependent, are o evoluie mult mai rapid, cuagravarea strii generale i oc neonatal, impunndintervenie chirurgical precoce, n absena creiaevoluia este total nefavorabil. nchiderea canaluluiarterial la aceste leziuni obstructive VS este urmatrapid de hipoperfuzie sistemic, oc i insuficien

cardiac, uneori cu semne nespecifice: iritabilitate,letargie, marmorare a tegumentelor care sunt reci,mai ales la extremiti. Recunoterea faptului cinstalarea ocului este datorat nchiderii canaluluiarterial este esenial pentru indicaia de perfuzii cuPGE1. Pacienii cu scderea perfuziei perifericeevolueaz rapid spre acidoz metabolic severidisfuncie organic multipl. n afara administrriide perfuzii cu PGE1, terapia inotrop, soluia de

bicarbonat de sodiu i medicaia de scdere a rezis-tenei vasculare sistemice pot fi utile pn la re-

zolvarea chirurgical sau prin cateterism interven-ional (9,10).

Anomaliile de origine ale arterelor coronare iinfarctul de miocard

Infarctul de miocard este excepional la sugar,fiind cel mai adesea datorat anomaliei coronareistngi, care are originea n artera pulmonar. Ische-mia miocardic iniial este intermitent, survenindla un efort de tipul alimentaiei sau a plnsului i

apare n primele sptmni postnatal (ntre 2 sp-tmni i 6 luni). Simptomatologia nu este speci-fic: episoade recurente de nelinite, iritabilitate,

plns nencetat, i dispneea, deseori asociat cupaloare i transpiraii, care survin cel mai adesea ncursul alimentaiei. Creterea consumului de oxigenn miocard prin afeciuni intercurente, de exemplu

pulmonare, poate precipita infarctul de miocard lanivelul VS i concomitent insuficiena cardiac:tahipnee, tahicardie, ritm de galop, cardiomegalie,hepatomegalie, un suflu de insuficien mitral,

alteori sugarul prezint o respiraie uiertoare gre-it interpretat adesea ca fiind o broniolit (7,11).Investigaii recomandate: radiografia toracic rele-v cardiomegalie cu edem pulmonar interstiial;ECG clasic arat unde Q anormale n DI, AVL,

precum i V4-V6, precum i supradenivelarea seg-mentului ST n derivaiile V4 V6, leziuni deinfarct antero-lateral; ecocardiografia confirm ori-ginea anormal a unei artere coronare, Dopplerulcolor arat fluxul de snge care trece din arteracoronar n artera pulmonar, insuficiena mitraleste variabil; mai pot fi observate scderea funcieicardiace, i anomalii de kinetic regionale ale pe-reilor ventriculului stng. Diagnosticul diferenialse face cu cardiomiopatia dilatativ.

Cateterismul cardiac are un grad ridicat de riscla aceti pacieni, fiind necesar doar atunci cnddiagnosticul ecocardiografic nu este cert. Trata-mentul este chirurgical. Peste 80% dintre sugarii cuaceast anomalie dezvolt simptome de afectarecardiace n copilrie, i aproximativ 65- 85% mor

nainte de vrsta de 1 an dac nu se intervinechirurgical. Pacienii diagnosticai dup vrsta de 1an dezvolt colaterale pentru circulaia coronarian.La copil, infarctul de miocard, foarte rar, poatesurveni n: alte anomalii congenitale ale arterelorcoronare, boala Kawasaki, cardiomiopatia hiper-trofic, postoperator n MCC etc.

Cardiomiopatii neonatale de etiologie ischemic(ischemie miocardic tranzitorie)

Sunt consecina afectrii cardiace de ctre hi-poxia perinatal. Deseori manifestrile clinice suntsevere n primele zile postnatal, dar sunt posibilemanifestri clinice tardive, chiar dup externareadin maternitate. Asfixia fetali neonataldeterminsuferinmiocardic, infarcti deces, cardiomiopatiedilatativ aparent idiopatic relevat la sugar.Majoritatea au evoluie spontan favorabil. Clinic,aceti copii prezint cianozi semne de insuficiencardiac. EKG relev hipertrofie atrial i ventri-cular dreapt, subdenivelare ST i inversarea

undelor T n precordialele stngi. Radiografi

a car-diotoracic deceleaz cardiomegalie cu vascula-rizaie pulmonar normal. Ecocardiografia vizua-lizeaz insuficien tricuspidian, dilatarea atriuluidrept i unt dreapta-stnga prin foramen ovale.Majoritatea cazurilor prezint ameliorare n ctevazile sptmni (1,2).

Incompetena miocardictranzitorie

Clinic, simptomele sunt manifeste imediat de lanatere sau apar n maxim 24 ore: detres respi-ratorie, cianoz, insuficien cardiocirculatorie cu

colaps. EKG deceleaz tulburri difuze de repo-larizare ventricular tip ischemie. Radiologic seobserv cardiomegalie, staz interstiiali venoas

pulmonar, aspect de edem pulmonar. Ecocardi-ografia exclude alt cardiopatie congenital saucardiomiopatie hipokinetic dilatat. Tratament:oxigenoterapie (ventilaie asistat), digoxin, izo-

proterenol, dopamini dobutamin, diuretice.Evoluia este favorabil n majoritatea cazurilor,

dar apari decese.

Miocardita

Etiologia cea mai frecvent la sugari este viral:enterovirusurile, inclusiv Coxsackie tip B, i ade-novirusurile. Debutul este adesea nespecific, frecvent


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n anamnez se deceleaz boli virale infecii alecilor respiratorii superioare sau gastroenterit.Sugarii pot prezenta iniial simptome nespecificeatribuite infeciei virale (letargie, iritabilitate, di-ficulti de alimentaie, paloare), sau pot fi diag-nosticai greit ca broniolit, deshidratare acut

sau sepsis. Deseori pot fi prezente simptome deinsuficien cardiac: diaforez sau tahipnee, sauaritmii care nu sunt rare. La nou-nscut, debutul

poate fi mai brutal, cu agravarea rapid a striigenerale, colaps i chiar moarte subit. Radiologicse constat cardiomegalie, EKG poate arta tahi-cardie sinusal, aritmii, sau microvoltaj QRS. Eco-cardiografia Doppler vizualizeaz dilatarea ven-triculului stng, disfuncie a contractilitii coronarecu aspect i origine normal, permind difereniereade anomalia de origine a coronarei stngi (12).

Diagnosticul diferenial al etiologiei insuficieneicardiace ntre miocardita acut, cardiomiopatiadilatat sau leziunile ischemice miocardice se reali-zeaz prin dozarea enzimelor cardiace troponina Ii fraciunea MB a fosfocreatinkinazei, dar diag-nosticul de certitudine l stabilete biopsia endo-miocardic.

Terapia suportiv respiratorie i cardiac este peprimul plan, intubaia este necesar pentru cei nstare de oc cardiogen. Suportul inotrop i de redu-cere a postsarcinii trebuie iniiat de la nceput dac

sunt suportate de pacient. n cazurile deosebit degrave, cnd tratamentul inotrop nu mai este eficient,ECMO poate contribui la ameliorarea prognosticului.Dozele mari de imunoglobuline intravenos iterapia imunosupresiv, cu rezultate ncurajatoarela aduli nu au aceeai eficacitate i la copil.

TULBURRI DE RITM I DE CONDUCERESEVERE LA NOU-NSCUT

Majoritatea aritmiilor cardiace decelate la nou-nscut i sugar sunt benigne: aritmia sinusal, tahi-cardiai bradicardia sinusal, blocul atrioventriculargrad I i grad II tip Mobitz I, extrasistolia atrial,

joncional sau ventricular nesistematizat, mono-topi cu o frecven 200 bti/min (180-350/min); interval R-Rfix iregulat; modificri minore ale frecvenei corduluila efort (plns, alimentaie, apnee). TPSV poateaprea sau disprea brusc spontan sau prin tratament,care poate induce trecerea brusc n ritm sinusal, cuscderea brutal a alurii ventriculare la aceea co-respunztoare vrstei sau meninerea la valori ri-dicate ridicate i fixe (legea totul sau nimic), maiales n cazul reintrrii. Complexele QRS pot fi:nguste, cel mai frecvent, cu sau fr fenomen T+P,cu sau fr unda P prezent; largi (bloc de ramur

preexistent/dependent de frecven /sindrom de pre-excitaie ventricular), cnd se impune difereniereade tahicardia ventricular. Tratamentul depinde destarea clinic a nou-nscutului sau sugarului (1,2,37): instabilitatea hemodinamic impune cardio-

versie electric 0,5-2 J/kg; dac sugarul este stabilhemodinamic, se ncearc manevre vagale: pungcu ghea aplicatpe fa. Tratamentul farmacologicconst n: adenozin bolus i.v. 0,05 mg/kg, repetat la1-2 min, 3 administrri, dublnd doza; dac devineinstabil hemodinamic: cardioversie electric 0, 5 2 J/kg (5,6); digoxin intravenos (nu n sindroamelede preexcitaie ventricular); propranolol intrave-nos, sotalol, amiodaron (efecte adverse). Verapami-lul este contraindicat sub vrsta de un an datoritriscului de colaps cardiovascular. Profilaxia recuren-elor se face cu digoxin (excepie)/propranolol timpde un an. Lipsa de rspuns la antiaritmice impuneexplorare electrofiziologici ablaia prin radiofrec-ven a cii accesorii (13).

BRADIARTMII NEONATALE SEVERE

La nou-nscut i ulterior la sugar bradiaritmiilepot avea mecanisme de producere diferite: tulburrin formarea impulsului (bradicardia sinusal, pauza

sinusal, blocul sinoatrial de ieire, ritmul ectopiclent, sindromul tahicardie-bradicardie-boala nodu-lului sinusal), sau tulburri n conducerea atrioven-tricular-bloc atrio-ventricular (BAV) grad I, II,III.


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BAV GRADUL II TIP MOBITZ II

Poate aprea la nou-nscut: cu cord normal; dinmam cu LES i poate progresa spre BAV complet;

postchirurgie cardiac. Poate fi 2:1, 3:1, 4:1 i poate progresa spre BAV complet, se poate asocia cu

tulburri de conducere intraventriculare (QRS largi).Tratament: nou-nscutul neoperat, cu BAV gr.IIMobitz II i QRS fine, cu morfologie normal, lacare impulsurile sunt blocate ocazional iar ritmulventricular de sc pare nu este foarte lent va fimonitorizat, existnd riscul evoluiei spre BAV gr.III, care necesit pacemaker implantabil; nou-ns-cutul cu bloc susinut i conducere intraventricularntrziat (QRS largi) are risc crescut de moartesubit, de aceea necesit pacemaker; nou-nscuiicu BAV gr. II Mobitz II postchirurgie cardiac, cu

bloc persistent i ritm ventricular de sc pare lentpot beneficia de pacemaker implantabil.

BAV GRADUL III (COMPLET)

Debutul afeciunii este aproape ntotdeaunaintrauterin (raportat nc din sptmna 17 degestaie). Etiologie: BAV complet asociat cu defectestructurale cardiace (25-30% din cazuri) (TVMcorectat, CAV cu izomerism atrial stng); nou-nscut din mame cu LES (strns legtur ntrenivelul crescut de autoanticorpi materni i prezenaBAV complet la nou-nscut), boli infecioase (dif-terie, oreion); cord normal structural tulburare

primar a sistemului de conducere. Diagnostic: laft bradicardia fetal 55/min;


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Cardiovascular emergencies in 0-1-year-old children

A.G. Dimitriu1, Georgiana Russu21Gr. T. Popa University of Medicine and Pharmacy, Iasi, Romania

2St. Mary Children Hospital, Pediatric Clinic I, Iasi, Romania

ABSTRACT

Cardiovascular emergencies occur in newborn and child more frequent than in other ages, becuase of some

congenital cardiac malformations, but also due to other aquired heart conditions. In newborn, congenital

cardiac heart defects with refractory hypoxemia and/or acute heart failure and those determined by hypoxic

perinatal suffering have a reserved prognosis, which impose an early diagnosis and therapy. Difficult problems

of diagnosis and treatment can occur also in congestive heart failure, miocarditis, arrhythmias. Such difficulties

in the management of cardiac emergencies in the first year of life are amplified if the heart condition was

unknown, as frequently is encountered in the clinical practice.

Key words: newborn, infant, cardiac emergencies, congenital heart disease, dysrrhythmias

Children with various cardiovascular diseasescan be admitted in the emergency departments di-rectly from birth, from the first days of life or laterduring the first year of life, due to a condition diag-nosed in the neonatal period or to a cardiac diseasewhich is congenital but still undiagnosed. The firstyear of life is characterized by the high incidenceand severity of the emergencies of various organs,compared to other ages. The postnatal hemodynamicchanges to the adult type circulation last about 3

months, especially when associated with congenitalstructural defects and can determine severe neonatalproblems, like neonatal distress of cardiac origin.

Clinical manifestations of cardiovascular suffer-ing can be observed from the first admission or canoccur like a complication of a previously diagnoseddisease (1).

NEONATAL LIFE THREATENING DISTRESS OF

CARDIAC ORIGIN

In the moment of birth and then during the firstmonths of life there are some physiologic cardio-vascular changes: disappearance of placental circu-lation, increasing pulmonary blood flow by de-creasing pulmonary vascular resistance (alleviationof blood oxygenation), occlusion of ductus arterio-sus and foramen ovale, maturation of pulmonaryvessels. A congenital heart defect can determinehemodynamic events during these events, with se-vere clinical manifestions (2).

Congenital heart defects with severeA.

neonatal manifestations

Isolated refractory hypoxemia. The differential1.diagnosis includes other causes of cyanosis

at this age (3): hypoventilation of centralorigin, respiratory disorders, methemoglo-

binemia, abnormal oxygen supply, hypo-glycemia, polycytemia.The syndrome of cardiocirculatory insuf-2.ficiency. The differential diagnosis includes:normal or malformed heart (4), pericardialdisease, pulmonary arterial hypertension.Persistent pulmonary hypertension inB.

newborn

Severe cardiac dysrrhythmiaC.Neonatal cardiomyopathies of ischemicD.

origin, myocarditis

The clinical picture that suggests a congenitalheart defect in newborn or in the first months of lifeare: feeding problems (the child is receiving themilk too slow or cannot eat the entire quantity, hascyanosis or sweating or dyspnea while eating),

presents cough, vomiting, constant superficial tac-hypnea (during sleep), stridor, respiratory distresssyndrome, tachycardia or bradycardia, cyanosis in

crisis or generalized, pallor, wet skin, cold extremi-ties, diminished cutaneous perfusion, agitation orinstability.

Congenital heart defects with severeA.

neonatal manifestations

1. Isolated refractory hypoxemia. The majorclinical sign is the isolated cyanosis, without respi-ratory failure, without response to oxygen adminis-tration. Sometimes the child is apparently well, andthe auscultation of the heart is in normal limits,without murmurs. But the degradation occurs rap-idly without medical and/or surgical treatment (4,7). The x-Ray aspect of the heart and pulmonarycirculation suggest the diagnosis (Table 1).


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Other causes of cyanosis in newborn: metabolic(hypoglycemia, hypocalcemia), hemolytic disease,neurologic problems (asphyxia, intracranial hem-orrhage, meningitis), neuromuscular disorders(Werdnig-Hoffman disease), hypothermia, sepsis,methemoglobinemia, respiratory distress due tomaternal medication (narcotics, magnesium sul-

phate), upper airways obstruction (choanal atresia,tracheal stenosis, Pierre Robin syndrome), poly-cytemia, neonatal shock, severe heart failure, con-genital defects (diaphragmatic hernia, hypoplasticlung, congenital lobar emphysema, pulmonary cys-tic adenomatous malformation).

Treatment: Rashkind atrioseptostomy (transpo-sition of great vessels with intact ventricular sep-tum), maintenance of ductus arteriosus patencywith intravenous prostaglandin E1 (tricuspid atre-sia with intact ventricular septum, tricuspid atresiawith ventricular septal defect and pulmonary atre-sia, pulmonary atresia with intact ventricular sep-tum, hypoplastic left heart syndrome); interven-tional catheterism (pulmonary valvuloplasty insevere pulmonary stenosis) (5).

2. The syndrome of cardiocirculatory insuffi-

ciency determines rapid aggravation and even deathof the patient. The clinical picture consists in respi-ratory manifestations acute pulmonary edemaand/or acidosis: effort or permanent tachypnea, se-vere respiratory distress leading to exhaustion ofthe newborn, hepatomegaly, tachycardia, gallopsounds, peripheral hypoperfusion: pallor, cold andcyanotic extremities, weak pulse, increased capil-lary refill time, arterial hypotension, oliguria oranuria, cyanosis alleviated by oxygen administra-tion (7). 90% of the cases of heart failure in chil-

dren occur in thefi

rst year of life and the main causeis a congenital heart defect (Table 2).There are also other signs and symptoms that

suggest the diagnosis in the newborn and infantwith heart failure (7, 8) (Table 3).

Treatment: diuretics, positive inotropic drugs(digoxin, dopamine, dobutamine), oxygen, me-chanical ventilation in severe cases, correction ofmetabolic inbalance that can aggravate bradycardia(acidosis, hypoglycemia), drug occlusion of patentductus arteriosus with important shunt (fenilbuta-sone), severe left obstructions (coarctation of theaorta) ductal dependent (prostaglandin E1), drugtreatment of tachyarrhythmias, pacemaker for bra-

dyarrhythmias.Particular causes of heart failure in newborn(except congenital heart defects) are: birth asphyx-ia, hypoxia, hypoglycemia, hypocalcemia, anemia,acidosis, sepsis (2).

Hypoxic cardiomyopathy is an entity manifestedby arterial hypotension, tachycardia, rales of pul-monary stasis, hepatomegaly, systolic murmur ofmitral and/or tricuspid regurgitation, gallop, andloud second sound in the pulmonary area. ECG re-veals ST changes or T waves of ischemic type, in-

creased MB fraction of CPK, x-Ray pulmonarycongestion and cardiomegaly, and also increasedcentral venous pressure. Doppler echocardiographyshows tricuspid regurgitation, decreased shorteningand ejection fraction of the left ventricle, prolonged

TABLE 1. Orientation elements for refractory hypoxemia in newborn (1, 2, 4, 7)

Pulmonary vessels aspect Heart shape Congenital heart defect

Increased or normal pulmonaryvascularization

Normal ovoid heart, thencardiomegaly

Transposition of the great arteries with intact ventricularseptum

Pulmonary venous congestion Small heart Total anomalous pulmonary venous returnPoor pulmonary circulation Normal heart Pulmonary atresia + ventricular septal defect

Boot-shaped heart Tetralogy of FallotBoot-shaped heart Tricuspid atresia (ECG: left axis, left ventricular overload)

Complex congenital heart defects with pulmonary atresiaModerate cardiomegaly(cardiothoracicindex=0.60 0.65)

Pulmonary atresia or severe stenosis with intact ventricularseptum (ECG: right axis deviation, right ventricularoverload, tall P waves)

Severe cardiomegaly(cardiothoracic index>0.75)

Ebstein anomaly (ECG: small QRS complexes, right bundlebranch block, rhythm or conductance abnormalities)

TABLE 2. Congenital heart defects with heart failure

(adapted after 7, 8, 9)

Age Congenital heart defect

Newborn Aortic stenosis, severe pulmonary ortricuspid regurgitation, tetralogy of Fallotwith absent pulmonary valve

First 7days of life

Critical aortic stenosis, hypoplastic left heartsyndrome, abnormal total pulmonary venousreturn, truncus arteriosus

2-8 weeks Noncyanotic tetralogy of Fallot,atrioventricular septal defect, aorticcoarctation, persistent ductus arteriosus,ventricular septal defect

2-6 months Anomalous left coronary artery fromthe pulmonary artery, persistent ductus

arteriosus, ventricular septal defect


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systolic times, diastolic dysfunction of the left ven-tricle. Associated metabolic abnormalities are: met-abolic acidosis, hypoxemia, hypoglycemia, hy-

pocalcemia, polycitemia.Treatment. Arterial hypotension is the conse-

quence of the direct depressing effect of hypoxia on

myocardial contractility associated with the de-creased systemic vascular resistance by decreasingarteriolar tone, so it is not a real hypovolemia, thisis why volemic resuscitation will use bolus crystal-loid solutions, monitored by central venous pres-sure because of the risk of heart failure occurrence.Positive inotropic drugs will be given, such as dop-amine 2-4g/kg/min and dobutamine 5-20g/kg/min. Digoxin is contraindicated in the newborn ofdiabetic mother because of the myocardial hyper-trophy, mostly septal. Systemic vascular resistance

will be carefully augmented with high doses of in-travenous dopamine 15-20g/kg/min (4).A frequently encountered situation is the presen-

tation at the ER of an infant with nonspecific symp-toms, without any known cardiac conditions. Thecardiac diseases with severe manifestations in new-

born and infant that are most frequently addressingto the ER are: congenital heart defects, cardiac ar-rhythmias, acquired heart disorders myocarditis.

Classification of congenital heart defects de-pending on physiopathologic changes induced by

the structural anomalies is:Congenital heart defects with cyanosisCongenital heart defects with obstruction ofleft ventricular ejection tract or functionalinsufficiency of the left ventricle, with signs

of shock induced by the decreased systemicblood flowAnomalous left coronary artery origin from

pulmonary artery, which can present myo-cardial infarction and shockCongestive heart failure

Cyanotic congenital heart defects

The most encountered lesions with cyanosis in-clude: pulmonary atresia or severe stenosis, tetral-ogy of Fallot, total anomalous venous return, trans-

position of the great vessels, tricuspid atresia,truncus arteriosus (2,3). Usually, these children arediagnosed at birth or immediately after discharge,

but can be sent home without diagnosis, and the patent ductus arteriosus can has such a flow that

offer suffi

cient blood for the pulmonary circulation,and thus can mask the true lesion and the hypoxia isnot clinically evident. Finally, when the ductuscloses, the pulmonary blood flow decreases and the

patient becomes acutely hypoxic. This happensduring the first two weeks of life but also later.

The cyanosis can be missed in children withanemia or hyperpigmentation of the skin. Blood pres-sure of oxygen is difficult to perform or can give er-rors in an agitated child or a child with depression orin which the peripheral circulation is compromised

due to the acidosis. If the acidosis is not early detected,as an indicator of hypoxemia, the child can developshock and/or multiple organs failure.

For the central cyanosis, the hyperoxia test is es-sential in the differential diagnosis of cyanosis (8).

TABLE 3. Diagnosticfindings in the children with congenital heart defects

Heart appearance Other suggestive manifestations Congenital heart defect

Cardiomegaly(cardiacindex=0,60-0,70)

Systolic murmur; ECG left ventricle hypertrophy; earlycolapse

Medium/severe aortic valvar stenosis

Pulse and blood pressure difference between arms andlegs

Isolated coarctation of the aorta

Early heart failure and pulse difference Coarctation syndromeECG: left axial deviation (-90 -120) Complete atrioventricular septal defectContinuous murmur, high amplitude peripheral pulse Large patent ductus arteriosus (in the

premature baby)Systolic and often diastolic murmur, moderate cyanosis Truncus arteriosusVarious murmurs or no murmur Single ventricle

Severe cardiomegaly(cardiac index>0,75)

High amplitude of carotidian pulse, continuous murmur(the cerebral ones)

Systemic arteriovenous fistula (cerebral,hepatic, peripheral)

Sepsis Purulent pericarditisSigns for tuberous sclerosis Tumors rabdomiomasFamilial history (diabetes), birth hypoxia Cardiomyopathies (diabetic, metabolic,

ischemic)Clinic and ECG heart rhythm>220 beats/min Heterotopic tachycardia

Variable size of theheart

Fetal and placental anasarca Fetal tachycardia, atrial flutterClinical and ECG heart failure


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and neonatal asphyxia determine myocardial suf-fering, infarction and death or dilated cardiomyo-

pathy without obvious etiology, discovered duringinfancy. Most of them have spontaneous favorableevolution. The patients present with cyanosis andsigns of heart failure. ECG reveals right heart hy-

pertrophy, underwaved ST segment and inverted Twave in the left leads. Chest x-Ray shows cardio-megaly with normal pulmonary vessels. Echocar-diography reveals tricuspid insufficiency, dilatedright atrium and right-left shunt by patent foramenovale. Most of the cases improve in a few days-weeks (1, 2).

Transient myocardial incompetence

Clinically, the symptoms are clinically apparent

immediately from birth or in the first 24 hours: re-spiratory failure, cyanosis, heart failure and circu-latory insufficiency. ECG reveals diffuse ventricu-lar repolarization disturbancies of ischemic type.Chest x-Ray shows cardiomegaly, venous stasis or

pulmonary edema. Echocardiography excludes an-other congenital heart defect or dilated hypokineticcardiomyopathy. Treatment: oxygen, isoproterenol,digitalis, dopamine, dobutamine, diuretics. The

prognosis is good, but death can occur.

Myocarditis

The most encountered etiology is viral: entero-viruses (including Coxsackie B) and adenoviruses.The onset is usually nonspecific, anamnesis revealsviral diseases respiratory or digestive infections.Infants present initially with the symptoms of theviral infection (lethargy, irritability, feeding diffi-culties, palor) or can be misdiagnosed as bronchi-olitis, acute dehydration or sepsis. Sometimes pa-tients can have clinical picture of heart failure:

diaphoresis, tachypnea, arrhythmia. The newborn canpresent sudden onset, with collapse and sudden death.Chest x-Ray reveals cardiomegaly, and ECG showssinus tachycardia, arrhythmias or small QRS waves.Doppler echocardiography discovers left ventriculardilation, disturbed contractility, normal aspect andorigin of the coronary vessels, allowing the differetio-al diagnosis with ALPACA syndrome (12).

The differential diagnosis for the etiology of heartfailure is performed by myocardial enzymes troponinI and MB fraction of creatinkinase, but the positive

diagnosis is established by endomyocardial biopsy.The supportive respiratory and cardiac therapy

is the first step, the patients in cardiogenic shockrequire intubation. The inotropic support and after-load reduction must be instituted from the begin-

ning. In the very severe cases, when the inotropictreatment is no longer efficient, ECMO can allevi-ate the prognosis. High doses of intravenous im-munoglobulines and immunosupresive therapyhave encouraging results in adults, but the efficien-cy is not the same in children.

SEVERE CARDIAC DYSRRHYTHMIAS IN

NEONATE

Most of the cardiac arrhythmias in newborn andinfant are benign: sinus arrhythmia, sinus tachycar-dia and bradycardia, 1st and 2nd degree atrioven-tricular block, atrial, jonctional or ventricular extra-systoles, 200 beats/minute (180-350/min); fixedR-R interval; minor variation of heart rate duringcrying, feeding, apnea; sudden onset and ending ofthe crisis spontaneous or with treatment, which caninduce suddenly the sinus rhythm, with decreasingof the heart rate to normal values according to ageor maintenance at high and fix rates (the all ornothing law), especially if the case of reentrancemechanism. The QRS complexes can be: thin, mostfrequently, with or without T+P phenomenon, withor without P wave; wide complexes (preexistent

bundle branch/rate-dependent/ventricular preexci-tation syndrome), when the differential diagnoseswith ventricular tachycardia is required. The treat-ment depends on the clinical status of the newbornor infant (1,2,3,7): hemodynamic instability re-quires electric cardioversion 0,5-2 J/kg; if the in-

fant is unstable, the vagal maneuvers can be effi-cient (ice bag on the face). The medical treatmentis: adenosine bolus i.v. 0,05mg/kg repeated at 1-2minutes, 3 doses, with double dose; if the child be-comes unstable: electric cardioversion0,5-2 J/kg


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(5, 6); intravenous digoxin (not in the ventricularpreexcitation syndrome); intravenous propranolol,sotalol, amiodarone (side effects). Verapamil iscontraindicated under the age of 1 year because ofthe risk of cardiovascular collapse. Recurrences

prophylaxis is made with digoxin or propranolol

for 1 year. Lack of response to antiarrhythmics re-quires electrophysiologic studies and radiofrequen-cy ablation of the accessory pathway (13).

Severe neonatal bradycardias can have differentpathophysiologic mechanisms: troubles in the im- pulse formation (sinus bradycardia, sinus pause,sinoatrial block, slow ectopic rhythm, tachycardia-

bradycardia syndrome, sick sinus syndrome), ortroubles in the atrioventricular conduction system(1st, 2nd or 3rd degree atrioventricular block).

2nd degree atrioventricular block Mobitz II: can

occur in newborn with normal heart, in newbornfrom a mother with systemic lupus erhytematousand can progress to complete atrioventricular block,or after heart surgery. It can be 2:1, 3:1, 4:1 and can

progress to complete atrioventricular block, can as-sociate intraventricular conduction abnormalities(wide QRS waves).

Treatment:the newborn with normal QRS complexes inwhich the impulses are occasionally blockedand the escape ventricular rhythm is not very

slow will be carefully monitorised, becausethere is the risk of evolution to completeblock, requiring implantable pace-maker;the newborn with sustained block and delayedintraventricular conduction (wide QRScomplexes) has a high risk of sudden deathand requires pace-maker;the newborn with persistent block after cardiacsurgery and slow escape ventricular rhythmmust receive implantable pace-maker

Complete atrioventricular block (3rd degree):

the onset is almost always intrauterine (from the17th week of gestation) and can be determined by

other congenital heart defects (25-30% cases),mothers with systemic lupus erythematous, infec-tions, or can occur on a normal heart, as a primaryconduction disturbance. The diagnosis in the fetusis established by the fetal rhythm 55/minute and requires treatment for heart fail-ure;

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