Capitolul26

CARDIOPATII LE CONGENITALE

Dana Pop, D. Zdrenghea

Cardioprtiilccongcnital€ reprezinttr un c&pitoldcoscbh 2D, scnrnili0rlianefiindintodcaun&corc0rinterprelata prjn

depatologicccogrrfctravandfu vedercprcvAlcnhcrcscuttr ecogralic$i deasenenea fiindposibilccrorid€diagnoslic, in
0 accstor{lccliuni, notatela 0,5-l0i dintrcror Drsouliivii. $i sensuldiagnosticarii deexefiplufluDuidcfectseptalatrialIa
avAndir vcdcrcfaptulci diagnosriculcslc ncccsara6 falcut un bolnl|vcu anevrisflal SlA, dArf!r[ $unlsrAnga-drcaptn
iDprimclclunisruin prirniianidcviall. candutilizarea altof
tchnicidcdiagnostic, inclusivcxpbrarcA hcnxrdina,nica in- .AccstcdoLi,lilnitc.ti irdcoscbiultimadintrcclc,au lbsr
vdzivl(catcterismul cardiacliaogioc diogrulia)cstcuneori inltrlurlttcf,rin ulilizarenccogruliciDonller.$inrprinrulrAnd
dilicildc cfcctuat. al ccografi ci l)opplercolor.carcdpcrmisAfirmarea semnilicR-
Inilill1.in nerbadade disponibililalccxclusiviI ccograffei lici luDUlknrale a detlcteloranst()nioc scsimteprinecogra li0
M, oportulacesteiain diagnosticulcardiopdliilorcong€nitnle 2D.Ecogrltfia Dopplerpcnnitc. dc 0scmcneq evaluarea prc-
crlt modcst$i d€ tip ,deductiv',in scnsulci cm nccesar ca siuniloriniracavitare$i. lhpldcoscbitdc importan!evanrarca
ddlclc ccocadiograffce saie illlcrprctrtccxclusiv in coniext dcbilLrlui iunturilorti o gradului hipcncns iu iipulmonarc.cu
clnric.l)c exenrplu. la uo subieclcu sunusistolicde ejectie imponante r€percusi!ni asupra irdic.rliciiempeuiice. Li ilc-
ir ariuaierei pu|norare,cu dedublaro lixi a zgonotuluill. Ic ccogrdfiei Dopplerswtrcprcrcniatc dcaceea$i insullcicnla
dilatlrcaVI) ii rri$carea paradoxalidc scptplcdauindir€ct cvalu rc r unorcazuridc cdrdioprtii oongen italeconplcrc.
pcntruun defed septalarrial. Totuii cuplarea ecograliei cu tchnic.rdc cxaminare Dopplcr
O driti cu apAritia$i dezlollarcdccogr fici 2l). aponul pc'mrtcca.in nlajonlalcacuurik)r intcnenlir chirurgiclh
ccocardiografiei in diagnosticLrl cardi('paliiloloolrgenitale saurchnicileinten'enlonnlcsi fic.rplicrteexclusi!nr bar.r
a crcscutconsiderabil, in naiotil.rlc! cazurilorfiind posibil rccstciexaminnrifil.namaili ncccsare cateterisrnulcardirc
firmarea diagrosrictllLi ana|omic. Rdmincauinsa doui nnri ii angtuoardiografia lll.
problernener€zohalc. Prinrdcrarcprczcntatar decardjopatiile ('.,rdopr| ( J"rg.n rrlr f,i I r'nplflrr(ir ||u l al
congenitale cornplexein carcccognfia2l) erademulteori ca- crlcgonr: cardiopadicongcnitalccu iLnrtsti gr-drcapl!.
pabilasadiagoslichcrcctoarum srunrai multecomponente. cardiorratii congcnilalc cu iunt dreapta-sti|ga ti cirdiopalii
daf nutoralitateacolnponcnlclorcardiopatiilorcongenilale. congcnitaletiri iunt. Dintrc accstecaregorii.ceacu itrDl
Ceadeadouascrclcrcrhscmnificatiatunctiomli$iheno . sti,nga-dreapla rcprczintanrajoritrteacaznrilorcxrc sunt
drnanici a d€1eclelor amtnnicc inregistrate prin eco$alie Dotatela varshrdultn.dc{rrrccccaftliopatiile cu iunt drcal
332 Ercguli.t t:.rrlului ti wtelor de la hdra ininii

ia-stanga(cianogene) au un prognostrc reTervat$i conduc in ariaatereipulmonarc $i nrsotitcdc dcdublar$Iiziologici

la decesdacanu suntdiagnosticate $i dacn nu se efectueazi a Tgomotului 11. fiecvent notatclNlirstr copiladci&loritS
corecliachirusicalain prnnii ani de viali. l)e asemenea o sindromului hiperkinetic cardl(N asculrrrrida|oriudnncnsiu-
parteinsennatia cardiopatiilor con-qenita le liri $urtpern t nilor relativcrescule aleA1'conrparati\ cuAo.
supnviefuirea la vArstaadulta,cum ar fi coarclalia de aoni. CelmaifrecventDSAeste detip ostiumsccundum lsilu{l
prolapsuldevalvenitrala etc. in portiuneamediea SIA). Mai rar estcsituat porliunca
Dc xcccalr vershxdul6scinlelncsc indcosc'bi doui catc supedoara a SIA (detip sinusvenot sauin p4iunca inti-
goriidecardiopati i congenitalei cardiopatii itale
congcn simple rioari a SIA(de tip ostiumprimun),defapt.nrlccastnullinra
(hrn $unlsar cu iunt slanga-dreapta) saxcardiopaliicongc- situatie, o ionni a canaluluiatrioventricular oomun.
nitalecirora li s'a efectuat corecliachirurgicaliin copilirie. in eco-M 9iB, nr canrite cn iunt sernnificati\
seevidenfi
Cardiopatiiconeenitale cu tunt stanga-dreapta aza cretlerea DTDVD ii mi$care paradoxald a SIV (fi9.26.l).
l)in punctdevedere fiziopatologic toatcaccsiccadiopatii E!idcnlicrca solu(iei de continuitate (lipseide ecouri)
rcalizcaz i o incarcare devolumacnculatieipxlmo are(llTP la nivchrl SIA sc cltclucaza in cco-B. pozitie subcostali. in
dedebit).oincirrcarc de!olum a civitnlik)rdrcptc $icvcntual pozilic {picah tiind posibila abscnF ecourilor in zona lui
o incarcare devoluma cavitdlilorstansi(Lrnele cardiopatil). scprumsccundunr, chiarin abscnlaDSA(1ig.26.2).
in stadiilcavansltcaplrc hipcrtcnsiunca pulnonarndc rc in J,ozilic subcostall. h cco-B,sc cvidentiazilipsade
zislenli, inversareatxDtului,caredevinedreapto-stinga ccouriin po4unc! mcdic. snpcrio{rl*u inlcrioaria SIAii
(sindionrf,iscnnlcnscr). se poatc nrisura dimcnsiuca accs(ci$tulii dc conlrnur(aic
Din punctde vedereclinic toateacestecafdiopatiie!o- a SIA (fg.26.3, 26.1).
lucazlcu sindromhipcrkinclic cardio!ascular (l.rhicardic
relalivi sau,rbsoluti.crettereaintensitlliiTgomotelor caF
diLrcc). cu rcduccrcrcapaciritlidc clbrr a bolna!ilordirccL
prcpo4ional cu Sradul)urrtului.cu hipc(cnsiurlc pulnxnra|i
Je Jr\rt 1.,$arxnrl niiL'ligiJtJirrul.rtr,: nulIlx'rllr.1Lrc=
cLrtn. inclusi!irrtrcinlcs cxlcml{ cinrpik)r pul,nonAri).
l)i purrctdevedereecocodiogr!fic!entricululdrepr( r
0triuI drcpl)prcrinlildirncnsiui diaslolice crescLrlc.hipclcon-
truclililalc ii miicrrc plr dorah dc scpt. (mai pulin nrc{/ul
PCA). I)c scmcncr nr anLrmilo situiilii(dcox.PCIA) ic no-
tcrzilcr!gcrclldir ensiunilofAS iiVS 0u hipcrcont|rlclilitate.
Calculafea dcbilelorpLrllrro0or 9iaol1icpernrilciDlo0lcoir-
/urilcstabilircar.rporluluidintrcQP/QAo.rmport{ irslahr-
lircadcci/icilnlsnrcntului chil'tngical.dur$iaf,()gnostlcului
boln0\,ilor. I)e asernenea. esteposibil6stabilireapfesiunilol
inluc0viluretiiD prirnulrind 0 prcsiutilorh a|(crapuhno-
nari. liTt carcpcnnitcdjltrcnlicrcahipcncDsiunii pulmuurc
dc dcbir \ii a hipcrtcnsrunii pulDr(nrarc dc rczisrcnli1.21. &;rj.?il/. l)SA. se4iuncprruslenrull!r lung Eco-M VD nihl.
Dcfoctulscptalatrial SIV cn miscarcpamdoxali.
Dupndell'clLrl scpl01v.JlriculatDSAeslecealnailiccvcnr.l
cardtuparic congcn ilah intiln rrdla vrirstrrdultd.(\rcxccplia In cco-B in sccliuDcpal.ls|cm.tll ax scurl sus situall, se
cazurilofincaredebituliuntuluistringa-dreapta esteexcesiv, po.rtc cvidcnlia crcljtcrcadimcnsiunilorAl c.lrc dcpdscitc
DSA€steunadidre cardiopaiiiLe corgenltale celemiribine di nrcnsi uni lAc o (fig.26.5).
tolc|xc.pcnniliindsup|avicluirca oligosrlnplonaticl p|]nnla lico- l)opplcr contnruu. rji indcoscbipulsdril.fot cvidcnlir
virsl.r.dLrlfi\ru chirr pnnala o vlirstainairrtrti.Dc accca, fl u\ul A S -A l ). Jrr .,1.-rI c.J \ i /ul i /.,r. i n 1.((.r.i | .'/ it i
in absenlaunei exa'ninarinredicalesistenraticediagnosticul subcostali.iideosebi in l)Co, carc e!idcnliazi ilux nn,zaic
esiedemuiteori ignorat,incetdepistarea D SAesteficutnin sistolo-diastolic..in pachere"din AI) insprc AS (fi9.:6.6).
stadiuldchipcrtcnsiunc pullnonarl.uncorila vislc inaintatc. lntensirateaflu\ului mozaic AS-AI) nu coreleaz,rinli
Pcdc altapafte,I)SAesreunuldintrediagnosticele frec- caniitaliv cu merinea Suntului.Aceasta poate li evaluati
lenl presupuse in cazulunorsulluniimclronale inrcgistfttc prin deteflninareasi comparareadebitului pulmonar (l)l')
Cor.{iopati i Ie ongen itale 331

ri&26.J. DSA. Eco-B.Secfiune4 cameresubcostal. Solutiede

conl i nul l al e.
S l A i ' 1po4i Mc.nrcdi c- D S A OS.

fk26.4 DSA.nco-R. Scctiunc4

cmcrcsubcosral.Sohrtiedecon-
rinuiute i h-Iflia-i- DSAutsinu.\cni-.
i SlAh tonrnear)Edr<
Fl&26.2Subiectrdnitos.
Eco-B.Se4tur|e4camereApicnl.
SIAnU
poiiuncamcdic(A).Scctiunc
scclidcntiszaln subcorisl4
camcfc
cnreprobeazi SIA(B).
htegritatea Mentiondmii faptulc! in DC, la nivelul a(erei pulnronare
se evidenliazAun gadient tmnsvalwlar pan, h 20 l,ImlHg
ti debiluluiaortic(DAo). Acesleapol fi evalualeprin de- (SPdedebit),depaqirea acesteivalori avandsemilica(ia unei
lerminareadiamelruluili ariei crosseclionale a AP ii Ao SPvahrlar€ asociate.
in eco-B.in asocierecu determinalea velocittrlilorin AP Eco-Doppler esteimpotantsi Iabolnaviisupu$icorectiei
$i Ao (VTI) in DC (rnairar DP) dupaurmltoareafbrmultrl chirurgicalea DSA, evidenliind pe de o pa{e inlegriiatea
DP (DAo) = rR: x VTI x FC. Clasic,un rapon DP/DAo septuluiinteratrial(evidenlier€ade€couriinlenseale,,patch"-
> 1,5 ccrtificl un $unrsenniffcativ$i impuneinte enlia ului la acestnivel),iar p€ d€ altnparteconlirmandabsenla
chirurgicall.dar la ora aclual5sc considcri c, prczcnla fluxuluisanguininte celedoxeatii.
$unlului,indifcrenrde n&imea sa,aduceindicalialbrmaltr in evaluareaprognosticuluiestede asemenea importanin
in DC la pulno-
dc rczolvarcchirurgicaliadclaclului12,31. €galizareadebituluipulmonar$i a debituluiaoriicsi urrnnrirea
nardsc cvidenliazlflux de velocinlc sclzud (1-1,5m/s). in timp a evoluliei valoilor presiuniiin arterapulmonarn.La
134 Iirgrurta .odulni ti wselor de ]d hazd itli,tii
nr oarcHTP dc rczistcnlnsc iDsralcuar.rpidcu mversarea
funtului.iarpcdcaltlUotcrqrrcrnrEcarcliopada congenitala
carepoatcdisprrcs|onlrDtc p{rcuNulprini lor xni dc !ia!a
(pini la 8 ani).nrdeoschi
in cazulI)SV mici fi al slrudliikrr

In ecoV e\retr(/enri'r',inrc:.I).l)\'D.,lar r'1-.,r.d

(lV e.r<Je reELli,,lr..rr:,.J,,l'i.l.r ir. arf.,ri
tarrJo\rli r
concomitenle devoluma VS l)'l I)VS crcscutcu hipcrcon
tfactilitatea SIV qi 1'f.
in eco-tlseevidenliaza aceeaiidilatare a VI) ficlcntuil
a VS, cu hipercontractilltate(nitciri ampleale SIV iri I'P).
Poatefi evidenliatd creitereadiamelrului AP caredcpa$e$t€

Soluliade conlinxitatea a SlVpoatefi evidenliatiatetjn

(o-V.fl )irnB.Lrisie),clu:rr Incd,4rlD\\membrJnos.ln
cazulcco-Msoluliade conlinuilate estemairar evidentiati,
ri&26..i loo-R.Sccln[cpa,astcnraliaxscu,lsussituati.Crc$lcrca inrcgis|mndu-sc o lipsadc ccourila scanorea. in modulM.
dirnerr!lui Al'.rre deditefLedixmeLr!lAo. in momcnLul (rcccriidcla inrcgislrarcaaortcila inregistrarea
SIV (in nrodnornialpcrctclcanlcriorel Ao se continll cu
SIV). Soluliadc contiruitatccslcmultm0i {iccvcntinrceis-
lr.t$ in nx)dulB, in pArastcrnAl ax lung,parastcmal ax scurl
saunl poziticapicah,fiind J)osibiliiti mlisurarc0 dinrcnsiu-
nilorDSV(fi9.26.7. 26.8).
In cllzulI)SV dc tip nruscular. ccl nrailtdcscrtoribrilbnn,
cvidcnlicrcraccstuia cstcimposibill, rdl ir modulM ci( ii,
de celefrai nrulteofi, in nxrdult] f2l.
l,icoSrafirl)opplcrcstc csenlialain diagnosticulDSV
fiind utiletortecelenei nrodaliulideexrminarc.,Ati( j^rrl)C:
cit $i irr I)P.in crzul dirccfionlriifluxuluiultftNonicp0rulol

ti&26.6.l)SA.Secliunc4
od'ncrc
subcorral.
I)(i). fluxcoL(tr
nr)za-
ph $4).
ic.AS-ADc.rccorimrntuniulsrirgGdrcr$o(!czicolff

rivelxl nitmleise poaieevidenliaadeseo PVM saxcletl de

VM. Potfi preze|i€de irsenlertea anevrismele de SlA.
Delectulseptalyentricular(DSV).
DJprur ii ruro i DSV-Jle\r(frin. inr'JfdrJiu|jlr'(,,n
gcnilahiniiilnirih lal.(aadu116.deii dupi ullinclc slalistici
prolapsuluide vahnnrirali ii rcvinexce( loc.
DSV cslcin principaldc doundptrd ncnrbrnos (sus
srruausaumuscuar.
DclcclulscpLal vcnLricularpoalcji dc dilcrsc nrtcnsirdli rik2tt Z DSV ttco-B.Secliuncpuruslenraldax lNg. Sevizualizeaz!
in lnnclc dc dimcnsiunilc solulicidc condnuihlca septului cld solula decolliruitare a SIV (tu dreapL).h srAtr8.,DP la nivelul
inlcr!cnlricularpulind rcprczcnla o cardnrpaticcongenitali detctului ouinregi(rarcauneilcbcitlli sistolicetozitivenrVD.
Cut tli opati i le ongenilale 335

ftk26.& DSVEco-B.Secliuneapjcul{4camer€SevizualizeAzi rid.26./0. DSV SecliunepdrustcmaLad lung.D( pcdifcctiaDSV

abs€nla
SMn tortiunc!slpcfioffii- DSVnrembrunos. SeinregisLredz!vclocihlesistoli.Apozlti!! ilr "degeldemAnug'

cu direcliafluxuluisanguinsislolicVS-VD,sc cvidcnliazn.
lr seclilneparastcmallax lung,fll]xpozilivir \tD tn sistold
(fi8.26.7),acesroliind cvidcnliatexchrsivin DC in cazul
I)SV severde tip rcstrictiv(vezimaijos).Vclocitutcu sisto-
licl pozitivdestein bandnin clt7ulDSVlarg (1ig.26.9) ii in
degddeminu$d(dctip srcnotic) ln DSVdemici dimcnsiun i,
cu gradientpresionalmArcVS-VD (fig.26.10). DCo oferA
diagnosticul oplimal DSV cvidenliindln parostcmll I ax lung,
parastenrclax icun iau&!ical4camere ilur col,)rnrozticcJre
treceir sisldil din VS in VI) (fig.26.11). Mlrimer fluxului
mozaicnucorclcazicuseveritatea iunlului.DC:opcrnrile atat

tiA.26.//.DSVSecliune axl ng.DCo.Fluxnrczaic

narastcrnal!
VS-VDcareconfirmn ntuls$ngi-dreapra
(vczicotor
plang5).

diagnosticul DSV sussiluet(mcmbranos) catgi al DSVjos

situal,muscular. cel mai adcscn dc tip cribrifonn[3].
Pcntrucalcularea severittrlii
iuntuluiserecurgcla accca$ i
deteminarea DP (debitpulrtonar)$i I)Ao (debitaortic)$ia
raportuluidintreacestea (vcziDSA).
Ca fiin cazxlDSA, cco-Doppler esteudln nr cvaluarea
rezultatclortratamennrluichimryical.evidentiind inlcgriratea
sepluluiinterventricular
$j ibscnlafruxuluiinrc VS iiVD ir
s\t ,lil.Deasemened po3| tr(.(i,,e\ohIid\d onl.rrlrc\iunii

fi8.?6 9.DSV SectlmenonsLemal,!x lue. DPo !rcbDsilu!6 in vD Menlionin$ilaplulcI, dacar

in unndcudouldccade.indi-
lanivelulDSV Scinrcgislrcazi
velociLlrsisdicnpoziliva.in bddi. caliadetralament
chirurgicalal DSVeraelectuatldoarinca-
336 ErcgruJia cordului tinxelot de la hdzdininil

zulin carerapofulQP]QAdep5$ea 1.5,laoraacn,ili $ con-

sidericeaceastar co.ccliccst€indicatdla totalilalcacazurilor
cu DSV avandnrvedcrcpcyicolxlpoienlialal in$alnrii in tnnp
al HTPderezistenliii aparilieisindronuluiEisenncngcr [4].
Casi in cazulDSA,princcografie, DSVpoatefi diagnos-
ticat,evaluat9iformulatn indicaliadehtervenliechirurgicala
carepoatefi efectualn.cxclusivin bazaexaninnrii cco-
Doppler.Iira a mai fi ncvoicd€ a serecufgela investigali.t
hcmodinamici invazivi(catclcrisn1 cardiac, angiocardiosm-
iic).Acestfaptreduceconsidcrabil costurileinv€stiSalieiri
tralamentului copiilorcu DSV
Canrlul atrlovontricularcomun.
Existi o forml incomplotna canolxluiatrioventricrar
comun(CAV). respectivDSA osriumprimun (DSAOP)
asociatcu insuficienl5mitrah. prin cleft de valvi nitald,
rji o fbnnl completi.in carcestcabscnltr irtetpoiliuneain- FiA.26.lJ.CAVib|mnoomplctn. Eco"B.Secuncsubcostrla 4
fcrioaria SIA catti po4iunea$upcrioaria SIV cu saufirI carncrc.DS^jor snuatasociat cuDSVncmbrMos (saseile).
prstrarcaambelororificii vahrularc0nitral ti lricuspidian).
in cazulcxistenleiunui singuroriJiciuvalvularalbcliunea
estcscvcri $i, de regultr,conducerrpid ]a dcces,incateste Ecogralla Doppler.lndcoscbi DCo,este$enlialain disg-
rarintilnittrin practicamedjcaltr a adullului. nosticulUAV in form! incomplctn (DSAostiumprimum)sc
in coo-Mii B seevidenliaza mtrrircadimcnsiunilor VD, cvidcnfaztr, in sectfune sub0ostrld,llux mozaicsislolodias!o-
VS,AP,AD$iAS,in tuncliede intcnsilalca $uniuluistanga- lic AS-AD.lanivelulsolulicidccondnuitale (fi9.26.14),
isrnr
dreaphrcalizat[1,2]. poziliaepical4calnercsauparaslcnral sx lungseevldenliazl
in cco-Bscnoteaza, in plus,crcilcrcadiametrului AP gi cxislcnlaii severitater insulicicn{cimilrale(fig.26.15).
seevidcnliazi dclcctulseprdl atrialjossitust(1ig.26.l2)ca9i in lbnDacolnpLeta se cvidcn{iazn0ux color AS-AI) fi
..clefiul"dc valvi nilrala (ilr eco-l'lsccliuncpAroslcmola ax flux colorVS-VD in secliunilcdcsoriscla DSA 9i I)SV In
scurt.lr nivclulVM). in cazulfonnci conrplclcsc asociaza cazuI cxistcnlei Inui singurofificiuvrlvularseevidelllia7n
un
iicvidcDlicrca DSV in po4iunea supcri{Hri (mcmbranoasa) singur ll ux mozaic care irtrcgsp{tliul
unrplc dintrcpo4iuncl|
a SIV(tig.26.l3). Totineco-Bsecvidcnlirzicxistcnta unuia supcrioarn a SIV 9i po{iunc inlcrioorda SlA, la nivelullui
sauI doui orilicii velvularcatrioventrioularc [5]. cmxoordis (lig.26.l6).

rik2t12 CAV ibnni incomtlel!. Eco-ts.Secllunesubcostal

4 subcostalli
rii+2d /4 CAV lbrmi incornplcti.Sec(iune 4 camcrc.
SLAjossituat!- DSAOP.
camcrc.SoluliedeconLinuilare. DCo.Ilux mozdicAS-ADjossltu.t (vczicolorplanld5).
Cadiopatiile conge itale

Avendin yederece gradientul prcsbnaldintreAo giAP

tl(.26 /t C'AVtbn)d incomtletl. Secliuieparxslcnuliur lung.DCo.
lM excslrlclprih Ucil"dcnitn i (vencobrpldr$ 5).
Seevideitiaz:i
eslecrcscutalalin sistoli cAtSiin diasldi debitul$untului
eslei otdcauna mareii va rezultainlHr hipetensiurepul-
nronarnde debitimportanh.Accaslav! conducerapid,pe
parcursula calivaani,la aparilixHTPd€rezistenli.Deaceea
dcti persistenta decanalancrialcsre ceamaiuior derezohat
cardiopatie congenilala din punctde vederechinrrgical,in
cazulnesanctionirii tcrapcuricc.,pronrpte' poateevoluarapjd
'prc H rf )i \r)dromullr
apdnria Fr.enrnerger cu progno\c
infaustal bolnavilor
Ecocardiogralia M fiB ofe.i pulinedatein diagnostjcul
PCA.Acestarcalizcazloincircaredevoluma inimii slangi
incAt,in eco-M,h cazurilecu iunt stanga-drcapla scmnifi-
caliv saumarc.se inregistreazio mirirc a dianclruluiAS
(util indcoscbimporul Ao/AS<1),iar DTDVScsrccrcscut,
cu hipcrcontruclilitate a SIV $i PP Dimcnsinilc VD lunt
nonnalcfinu cxislnnri$care paradoxah a STVdcc^l in stadiile
avansarc (si dromEisemnenset II].
Eco-Bpennite.h anumitccazuri.!izurlizarea.in pozitie
supnstcrn{ll,a caMluluiartcrial.ln(rlj Ao descendentl, la
nivclulirsc4ieislbclavieisdngi,$i AP st'1n84 (fi9.26.17).
Ecogralial)opplereste escntiahin dirgnostic0lfCA. in
DP sc poateelidenliallux sislolo-diustolic retrograd in AP
(vclocitatcpoTitivacu lnaxrmunrin tclcsistola, in AP), in
scoliLrnc parasternali 0x scunsussiluatl (ff9.26.18).
DiAgnosticul cel mai &cunitsc rcaliTelziilt DCo c8rc
c!idcnliaz,in artcrapulmonsrn,in sistoltr9i diasroli,llux
rctrogradcolor,moAic curcscindrcrptainsprevalvclcpul-
monare. Avandin vcdcrc.,rnrcsteculil) sistolidl lluxurik)r
pulmonarli dl caDululuirrtcfirl. esteescn{iallldcpistrrcu
velocitlliidiastolicc pozitivc nrAP (ffg.26.19).Ncccsi!I ti

fi&2d16.( AV i)nn! conr!]cln. Sccliunc .1carnere.

0picalil D(l).
|lLrxn]ozriic
lanilelulhricruxcordiscorclr()bcszlncccrc&"libcdl"
r sangeluiirlrc (vezicobrpl!n{'u5)
cclc4 caviliqicddiacc

RaporlLrlDP,/T)Ao. respectivseveritatea $untului.sc dc-

tcmlini similarcain cazlrlDSAsauDSV
Perristentsdc canalrrterial (PCA).
Candlul afi€rialestetunctionalpeporcursulintrcgiivieli
intrauicrin€,
realizandderivar€asengelui dinatcrapulmonar?l
tu circulalia
sistemica,avandin!ederccl circulaliapulmona|a
csicabsentipeparcursul perioadc.
acestei in cazulincarein-
chid€reacanaluluinu serealizeazn pc pircunul primelordoua
siptimanidevialdextraxterini,sensulcirculalieiin canalul
a*erialseinvers€a26. avendin vcdcrcpresiunilecrescute in /.i&26./Z PCA.Eco-ts.PdziLie
\upraslcnrala.
Soluliedeconri.ui-
circulaliasistemicicomparali\r cu circulaliapulmonaE[2]. t AP s|enga(sigeata).
taleinre Ao dcsccndcnti

liti.26,lil. Pl A. Seeliure pmncrnnlli rx scurl srs slLrtrli DP
flLrx sistolo d'astolicl)ozirl! in AP Estc pr!zcnli rqrLereadiroalrl
li&26.rr. llCA, Sccliurcplrrs(cnulli d seunsrs silurrii l)(i).
l['x dirsiolicru91'nhzric in,^P
dilereotixtde anerxcoronarilabern u dinxrtern puhnonari.
diagnosriccrrc depltcire cxdrul li ilal al hrcrlfli dc Ia(it
hr PC,\nici ll uxul Doptlcr prczin|i o vclocihlc marc.
c sl cco n l i nuus is t oli. odia s to l i cc,u p c a kl c l c s i s L o l i c i n s i s l ol d
|ltrd i vdID
. P CA c uf t r Dlbi d i rc c L i osni aIIT l Ps c !c rn .ftrD l u lcsl c
drcrplr sllnga in sistolaii sLlingadrcaplailr sisloh lardiva.
c xrn rsti Dar ladias ! , 1i.iDPC Ac u i u D td rc rp l as l i D g r.fiL r xul
cstcooDtinutr. ncgrtiv cu \ck)cilatcmrximn iD sistohprccooc.
I)nr monrcnlulstabiliriidiagnoslicuhridc PaA. prin DCo,
di rg n o sticdc r lt f c l f ac ilr ic l n ri c(s u 1 l us i s t(n i c od i ,rs l o l i pc a'
frsrcDul sting sussituat).cstcdc frinri impoftanli stabilires
L.ogrd/1d .onlllti ti wsek, .le ld hazd init,ii
dcbirului iunrului.rcspccli! r nl]ortului DP/DAo. delerminal
ftrp, mctodologir cxpusnla DSA l2l.
Un rapoftmultcrescut(>3 5) imfun. dc urgcnIfrcorccliN
chjnrfgicali a PCl,{,in scopnLelitifii instalarriiHl Pde reTis
lcnlri ii a sindrcmului Eiscnnc gcr.
Corcclia chiruryicrli cnc insa indrccrn in caruL PCA
indiferent dc mfonul QPlQAo, s{nctiunca .hirtrrgicrh .
cardi opati ei congeni talreal e i Tend practi c!i ndccarcNborn r
luhri si de altlel fiind cearnaiu|or de reaLizat,hrnajoritatea
cazurik)rncrnpunilndu-scb) prss-ul crrfdiopolmo ar.
l )rcnrj ul rbrrrnt rl l cncl or pul monarc.S c rcl nri rlr
drenajll1aberantal unorasauaLtutufor venelor pul'non,rfcnr
{rLdu1 drcpltu uldnul cazllindncccsari penhr supnvietuire
cxislcnlaunuiDSA sru DSVcrrc, h lirpl. rcalizcazi un iunl
drclptr slirgr, rspccl ljarc dcpiicflc crdrul lucririidc hlil
in cazLrldreDajLrhriabefaut rl uneia sau r doui venc
pul marc in atriul drcpr aspccruli eco-\rl esteidertlc cLr
ccl inrcgis(rrLin DS h cco B rspcc(ul cslc,dc ascnrcncd.
si nri l rrcu ccl i nrcgi n..t i n l )S A . drr v)l ul i ! dc conl rntrrl{lc
la nivelul SIA este absenla,fhpt care r'ebuic saioricntcz.
di asuosri cul spfedrcuaiabcrnnral veuel ofpul ,nofi rfe.
Diasnosticul dc cclri{Lrdlmc LrldrcrLriuluilcnos abcrunl
in AI) portc li clccluxLpri cco-t)opplcr. DP \il irxlcoscbi
lXl), d.r cvkl.nl crur .cclrui (lrcnq cstc ctificiLnfl Nrlc
| .r(Lr:' rr.I.Ir' i ,1. \1r((JL i l uf-..| i ), .\t\| ,' (rr.,i
N1rrirrnpoa.llrlacslodcLcfminirc.rr'.rportului DPIDAo ctlrc i
cr.,ul ni ctllc dcpiitcilc rrlo{|1l dc l. tl |r.ri .rlcs dc 1.5.irl
trczcn{{ l .rbkNel i ni cfi eeocfl (ti ogftrlsl i enl 1rr cl l cel
' rnLri
,II l )S \. \1.' rl ,i rr., ,. I S l A i r' r."r. nrl d(:./.i I.l l o r.i J,trJ . r
di ngrosl i c,l i i n(l i ndi ctl 1.pcnrrLprcci zrrcrdi rg| l osl reLLlur .
c l cl cri rnrulc.rrdi i re.
S i rdronl ul E i nscrnrongcr.R cfN /l nti sl .r(l i ull i ]r.rlrl
tuLrrorcrdi oprti i l or eorgel ri trl ecLrsLrntnri ngr-drerpr r .
A l drc r^DnN ncnl ul nrsrdl l ri iIITP .l c fczi sl c l i . Indnc nl
i r carc sc i nl crscrra i untul . crrc dc!i nc drcN pl r' sri ngr .
(l )rcstrndc nronrcntul ui i n c fc tol navul frc,i nl arci ano / i
lo irccsrmomcnl rl clolulici bolii. corec(iachlrurgicrh
.,,r.i rrrrfl rti .l ,rl ' :' (.,r' 1i .1' ri .i (, r' rJri r.,rr.r.,,r r' .L r i
ncindicotirci ii contraindicatr.dcoarcccvr condLrcolr crci-
terer substul i xl i a grnduhrihi penensi rni ipul nrorufe.l: r
prccipilI]rcasau rgr.t!arc.t tusulicicnlci vcorficuhre drcplc.
D i n { ccst momcnl s.| D tuldrcrptr sl i ngr dc!nrc n.ccsNr
pentrudi nri nuafcanrcatrci ridc i prc!i unc r vcnl ri cul ului
drcpt. chiar daci aceastase realizeazacu pretul hipoxenriei
.i .rJr' r.r fri 1.,r" .' ni l drc: .;r fLl ,.i : r. i : I JJ..i rsJ \.i " .
($unrdrc{pLr stingr).
i n eco-M!c c!i dcn!i aza aceeri i di l .rtaru a V l ). r,rLrca,c
|a
radoxalaa Sl\',la dveLrlvalvei pnLnonarelnegisrriindLr-se, nr
 Cardiopdtiile co Kenidle
339
fl m sinusal,dispadtiaundci a (lig.26.20).,\cccat i dilatarc
aVI) cu mitcnri paradoxalcale SlVsc nrregistrcadnr eco-B.
l-lsentiahpentru diagnosticestc ccogmlia Doppler carc
cvidentinzi pe de o plrtu H IP ii coDlecintclcsrte. iarpc de
rltn parlc irversafea$untuui.
HTP cste diagnosticNta (in DP lX fi ind.osebj in DCb)
prir apa.ilia inslllicicntcipulnonrrc ; i graduI acesteia.pAps
se poalc dcterlrrim, datuiti apariliei.ir lnaiorirateacalurj-
lor. a msuficie|rei tricuspidiencc!re. de ascnrenea. poalc fi
dragrosticrta!i evxlurtnin cele(rci'nodud cco I)opplcr(vezi
illsxlicicnla tdcuspidiani). calculareaPAPsfiind expusir.de
asemcnca,la capir)lul respecrivfsl

t1.rr.26.21
DSVSindonr,iscnmengcr SccliLrne
rubcostali4
crn!
cfc.SevlzMlizeui,DSA ir D( o fluxalbrsrnr
AD-VD cartiticind
nrvcNared (\cTicolorptaNa5).
rufluhI {drcrrrtcstiflgr)

dcbi$lui cArdiacprin tulbuflrilchcmodiramice consccutive

dcfectelorrnrtomicc caracterisricc cardioplttiei,agmvend
hblo!lcliric. Deaccca, precnniidrtoriti lirpruhici! irrge-
ncrJl.(rc{sr.Lardiofsrr| \unt cordx\lntir comll.\e. gri\ iLrtcJ
rcesloracstccrescu!tr, In consccinlaInaioritatea conuucla
dccesir primul.saupri'nii oni dc vial5.drc, nu suntrczot-
vrte prir inl€rveDlic 0himrgicln. Aceslacste9i unul dintre
nrotrvclclenlru cnrccleslntrarintdl0irc h varsraadrlm, cu
exccplrr citoNddinrfeelc1.61.
rih26,rA siftlroDEisenmcrscr . Dirtrenccstc{vom cxpuncdoartctrAlogja Fnllot,cea
Slcliurcprmncmdtr sringisL$
srlunlllho-M.l$grum!v0lvci
puhnonlr!
cudispdriti! mailiccvent[cardi(]patie congcnitalilcu funrdrenpt&stanga
urdei1i,-I tTp
carepcnrlte suf,ravjeluirca pinl la varsraadulti.Anrinriln
ctrtclralogia Fallotasociazistcnoza pullnonarisubvnlvu lori
[,a nivcluldcfecteloran{tomicercspcctive (DSA, I)SV (uncorivalwlarll) cu aortacllrre (pc SIV). DSV sussitual
clc.)seevidcDliszl, in DCo.fluxbidircclioral sau.ir cazurile (nrcmbranos) $ihipertrona sccundff,a VD.indeoscbi ac?iiidc
:t!rn{r|e.cr(lusi\ sLrnl drcrpl -stangr.Jcnisrattr:n llreTent.r teirrca VD.cnrcrealizeaztr ii stenoza pulmonartr sublnhularlt.
nr('zaicului. dc aceastidrill cu dircclicinversald (tig.26.21). Tneco-Mfi B dimersiunileVS $i Vl) snntdc rcgutrnor-
( alculalcrtI)1,$i DAo cvidenliaziun raponDplt)Ao l/l nrdlc.se-mn ul principalinrcgisrral liird acela&1DSVti aorrci
(funrbidircclioral)sauun raportin liivoareaDAo (< l). cllarc. tnrcgistrarer DSV in ambclcnroduri(M $i B) csrc
Ap0dli.rsindrotnului lis€mncngcrsemnalc.tztr, defegu, snnrlara cu cc expus!llrDSVAorracilaresccvjdenriazr nl
Itr.moncntuld€p?ltinicorecfieichirurgicalca cardioparici modulM f,rinaceea cI ccourileSIV la scannnrg-utin mo&rl
congenjL.llc. pertru stabilir€axrilililii saxcontraiMicalci M, seafl! intreceledoui econnalcperelilorao(ci. in nodul
acesteia liind n€cesrr i dcterminarc{ rezislenlchrpulmonarc B seevidcnlinzn denscmenea. ll1aiclar.situdrcaeconlujslv
(crielmsm cardiac)fi aspecruluj morfologicat arreriolclor nrtreecourilepercliktrAo (1ig.26.22).
pxllnonarc(biopsicpulnron0r.)[61. Ecografia Dopplcr.indeoscbi DCo ce iticardiagnoslicul
Cardiopatiicongonitalecu funt dr€Nptr-stang!. prinevidcnliereanr sistolAaxnuifiuxcolormozaicla nivctul
Cardiopatiilccongenitalccu iunt drcapla-sriingn sunt I)SV dinspreVD inspreVS ii evidenticrca fluxxlui cotor
denunire)i cr-rd,,nrriicianog.n( JeoJrer(Irin corLrmrr- mozaicdin VS ti VD inspreAo[7]
rca.rderiahcu singevenossc realizeazr gradeditntc. dar Stcnozapxlmonaraesregrcu de diagnosticar, inclusiv
s€mnificative, dc hipoxemie. Ilipoxenia scadaugdsciderii in (co Dopple..:rr ru,,utsrrr,lrI sJtesub\.,1\trr(. in (arul
140
ti8.?6.2?. ltl'!k)gie Fnll)r.l'lco-ll.Sccvid€tlluli l)SV (sig.rla)
locAliznrii\ral!uhrc.li{grrosticul poziti! $i dc scvcritatcsc
clcctucrzl similar cu ccl cxprs la srcnozapulnnrrari [81.
Calcrl.rca DP/DAo cstcdc nccastndatain l-avodrch I)Ao.
dircc( pft)po4jonrl cu scvcrivrtc{ iuntului drcaptr-stnnBn.
I)iAgnoslic.I'cl lclr{loSici lrillk,l impLrncrczolvrfca chi-
rurgiulA carc sc rczolvl pri corccl c.l conconrilcntl. prin
pctcc.Adclcctului scptrl vcntriculrr $i originii mrtci din rnr-
bii vcntriculi,prccunr\riprin corcctArcA r)arli hrobstrcolului
: rh \rl ru l nr f ulnx ' r ur f ' nnn n d ' mrc i i n r' \f.rs u .: i n (.1 / n l L
irr carc srncliuncr lcrapcuticl s-n rplical prccocc,inrinte de
rprrilia hipcriiofici vcntricul fc dfcptc imf'orrrnte cvolulia
<, rcri \n rr hr ' ii; neS is r , in d u -re J rs p a ri rrJ J i :,n os 7neo n u -
lizo re ttd e bit uluic r ir e S i r c rp a c i ri l l i i d ce tb d r b o l n A v i lor
Aiio curr aur dlitrt nLrvonr descrierlte cadiopalii con"
geoilale cirnogelre. drr deoareceacesler sunt coorplere.
deti ecocrfdiogfr6 e esteinrport ril tiitrdispelrsrbili pentru
din g |o stl c de. c eleur alr r L rl l eo ri e s teu e c e sri . p e n ttuc o n-
l n.ifea ii e,relrtualcornplerffeadiagnosiicului.explorarea
hcurodonlrici i !ui!x. fesp€cli! cirlererivnul c.i(lioc ri
r n g i o cxdiogr nlirlll.
Cl rd i opr lii c ong€nil a l €fi rn ttrn 1 .
('u diopaLiilccongcnihlc lira tuDtpol r!c! conrflcxitdrc
ii sc!crilNlc!rrirbiln. rcprcrcnlrlDduncori sirDplccoDsl.liiri
clinicc ii ccocardiogr.li.c l:rri sn crcczc boh !ultri Dici o
simttrnnrto k)gic.lirici. pc cind. rltcori. cvolulir l(r'fo{lc h
scvcrar I i unnat.rrapictctcct.ccsulbohravik).Amnrtim cl prnl
rrc ca iofatiilc congcnilalcftra iunt sc numin.rdcxtrooardia
(i7()lalisauiDcadrulunuilitus invcrsus).majoritarcaoazurilo|
dc tdaf! idiotatic de valvatmitrali, bicuspidianorlicaetcf ll.
f:<'ogrufid er.lului ti vdselot de la hdza itiinii
A!ind in !cdcr. scr)pu lc accstuimtrlr dc ccograliege-
ncyaldnu vom erpune decil doui dintre cardiopalirleconge
nitale iira $unl.atatpentrue{emplincare,cit filcntN fiptul
ca sunt rclaLi! liccvcnt inlrilllitc ti rc'prcrinli. pirna dinte
ele, o cauzi corectabili de HTAsecundari. iarceade adoua
o cardiopaticcongcnitaIn ..currcas.f'nr c.rc tosibiliLi!i1cdc
sancliunctclapcutca, inclusiv chirurgicala,suntfednse.
Coarcta{ia de aortn
llstc dc lirpto slcnozi lorlicnposllalularndrstali,sitmli la
nivelulaoneidescendente, drncolode inse4ias bclavieisiingi.
fizjopatologic se realizeazi o hipcftcnsiunc rcgionrlai
in arnonlcdc slcnozn,hipcrlcnsiuncdirccl propo{jonali cu
sevedtateacoarcta(iei,$i ln acelati tinp rin sindronrde hi
poperfuziedistali.in avald! coanalic.Rcprcrinli trn! drn(rc
cauzclcIITA sccundarcla copii ii lineri, cauzi carenebuie
diagnosricatipfecoce.inairte de aparilia intercsnriircnrlcri
pcrnuncntiznriiHTA indifcrcntdc pcrsislcnlasaurczolvarca
chirurgicLrl.sau nncrvcnlionalaa coarctaliei[1.2].
in eco M nu se inregistreaTar decit scnrnc indircclc rlc
coxrclalici dc aorll. crrc lrcbuic inlcnrcLalc ir corclalic cu
simptoLrntologiacliricd.
i n prczcntaIl l A scm i fi cati vcsc poatccvi ci cnl i a mi ri-
rca dnncnsiunii aonci (:1U nnn), hiperlrofia VS (cretterea
di nrensi uni l or S IV $i I' l i ) $i . eventualhl
. pcrconl rrcl i l i tt lt cx
!cnl ri c l { ri (S IV 11P P ).
iri eco-U se evideniiazi aceleatjelernentede diagnostic
dcscri sci n nrodul M. tl ncori . i n pori {i c supftrsl crnalsc a
porl c cv cnl i a. h ni vcl ul orl ci dcscc[denl e,i rl gustare.
di ani etrul uirorti c {fi 9 26.23).dar dn' gnosti culcsrc di l i cil
chi afi n c zrl ccogrnfi $ri l (' cxpcri
r nrcnrti .
Coarci.licdc !or1i. lco I Sccln[c nDnscDnla. sc
lct&?6.2-1.
e\ idcntinzringu(rrealunrenului Ao descdtderte
C d ftl iqnn i i I e LoDg0 itd I e 341
t)iagrosticulacunlalCoAo sc clcctucaTn jrr eco l)oppler nonnali. l,steulil indeosebi in diagr)osticxrca u uiPalArso
nrdeosebiin Dco [:]. cixt.trnr c! identierea llLrxului diasrolic rclrogrddin lrunchiul
Ldliz ar e a a c c s l u i a .d i n fo 7 i l i c s u tra s tefnal ipermi te rrtcrcitulmonare(seciiune paraslcmali rx sctrrtstrssituarn).
eviderlicru unui sradicDtin ro.t{ dcscendenia:'l0 rnnfig. IeoreticDl nlrcgistrxtla un nilcl superiorcoarctatiei
nr : lr i ! . r :rJ.n rr ,r | | ,L,l I r. I tr" fu " o r l i c,r,< \eri ur< d ). lJ un r,i\( ,r:r,i rf(JrrJ f<a,re i ce enl.r..c In..l
'.r
coarcL.rlici dc aortir(fi9.26.2,1). un gndient peste,10-50mml Ig acurahr gmdi.ntuluitranscoarclalie. dar diagnostrcul cstc
(tu sisloh) nnfunfird intcNcntir chirurgicali 16,91.DClonu dc cclcrnaimulteori imposibilda1oi6 vclocitartilor nrd.iii
pcnnilc dccit un dirgnostic de suspiciune,prh evidenlierc{ lparifieifeno renu1ui dc rcplicrc.
duxultli mozric. sist(tic (fig.26.25).dar Sidiastolc h CoAo De me lionarcn DCo pcrmitcii evaluarea rezuitatelor
scvcrn)in aort! dcsccndcntar. distal de coarctatie.dar sccs( mler\cnlici dc corcoliea CoAo (dilataresau inrcrlcnlic
llrx moTaicscpollc inregistrauneoriin sistoli ii la subicclii chirurgicali),prin inregjstrarea disparilicisln drminuarii
scmnilicative a gradlcnluluitr{nscoarctrlie.
ln cazurilcin carclalorilc'lA conlinuisdlie crescurc nr
abscnhrc.rnrrilieiunuigradientsemnillca(i!lmnscoartalic.
lrcbuicluati in coNiderorernenlincrca ITTAndnmccanrsnr
renal(relrina-angrcLcnsini). in rccst caz valorileTA fiind
crescLrlc nliiLl{ ni\clul rncnrbrulor superioafe cit ii la uivclul
rnunlrrcl('rinfcrio,rrc.
llorlr }]brtcin,Rea irlirr cri.rcc{skmahdi. sccamc
lcrizerzd.din punc(dc vcdcnan{lonlic.prin inse4ifljoasia
valveilricuspidc.cu .ttri.rliTarc.r uneiporliunidin VD.
Dir puncldcvcdcrccljnicboalase lrnrnilcsliindcoscbiprirr
ituluccrcn unortulhrliri deritrnparoxinicc. supmvcntrcularc.
0tcumii pdnsci1derca. npcrenl nccxplic0bih, acnprcitAlii deetbd
acopiilorsnu.r Adolcsccnlilorprffit(n i ri acestei n0onralii ||.:1.
Dc\tiirr cpocAdistonibilitlfii exchrsi!cfl cco,M ru li)sl
f , & 26, ?d( o rrc r{ rl idc cn o rl i lS e c ti u nscrtru s l carall i)(.(nadii - doscrisc o seriede senne0lcaccstci boli.rccstcrsuniin-
er r s is t o l jscc D tri l i c rL(,1
i ! 0In f' l l !) c rrcc c Li i l i cicorN l nl i r. corsrrtnte ti inrprccisc.
Dingnoslicul sc clcctucr/ilLr ofr lcrualllin cco-B.in
pori(rcaprcAlar srusrbcosull.
Scmnulpfincipalestereprczcrrl0l dc dccrlarc insclici
v,rlvciInitrale$ilficLrspidc.c rcirlnodno nalcstc..5 rrrnr.
decalarca > ll nurrrcprczcnllnd ur senrn dirgnosticdeccarlu,
dincalmdhclicilibstcin(fi9 26.26).S€eviden(i0zl. dc scnrc-
nc.r.lnlrirer oparent?i aAD pfina(drlizarcaunciporliunidin
Vl). VI) liind dedincnsiuninrullrcduscconrparAtlv cu AI).
Seporcvidurlia sislolicc
curtrdclii alcpo{iunii atrializrte aVD.
Ecografial)oppler.prin InodurileDCl.DP fi nidcoscbi
D(i). fc nite diagnosticul poziti! ii dc sclcrilalcal insu,
frJicnr<r Ir(rFtrdiercinr"rJ.arn:, r'\i- 1 r,.1i..i i...,.1.1
valveltricuspidc(1ig.26.27).
In hr.rlul oesruicapitoldorinrsa rearninri lnplul cii
I'ili/.,r.J r(ocardioeraliei |etalef. L ||.. inr .rn r'.rnil
semnificati,r!lcazu lor,dcpistdrc.incidinaceastipcfioa
alllroradir t cndiopariilccongcnitalc alclinrlni. I)episia rea
cslcncccsrripcntrustabilirea uoeiatiirdtuilcrapcuticc imc
dirLdupar naitereprecnm$i drloritAlaptuluicn.ir aDum ilc si
('oAo Sccliunc$'prastcrn.li
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