Download - Cardiopatiile congenitale ale adultului
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Cardiopatiile Cardiopatiile congenitale ale congenitale ale
adultuluiadultuluiProf. Dr. Doina DimulescuProf. Dr. Doina Dimulescu
Clinica de Cardiologie Elias Clinica de Cardiologie Elias BucurestiBucuresti
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DefinitieDefinitie
Anomalii structurale ale inimii sau vaselor Anomalii structurale ale inimii sau vaselor mari care au/pot avea semnificatie mari care au/pot avea semnificatie functionalafunctionalaSant prezente de la nastereSant prezente de la nastereNu sant incluse: prolapsul de valva Nu sant incluse: prolapsul de valva mitrala, bicuspidia aortica si persistenta de mitrala, bicuspidia aortica si persistenta de canal arterial asociata cu prematuritateacanal arterial asociata cu prematuritatea
Crawford 2004
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PrevalentaPrevalenta
Aprox. 10‰Aprox. 10‰Similara in rasa alba si rasa neagra pentru Similara in rasa alba si rasa neagra pentru malformatiile majoremalformatiile majoreDefectele complexe, cianogene – mai Defectele complexe, cianogene – mai frecvente la populatia masculinafrecvente la populatia masculinaDefectele mai putin complexe – pop. Defectele mai putin complexe – pop. femininafeminina
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Factori de riscFactori de riscSubstante medicamentoase, droguri in timpul Substante medicamentoase, droguri in timpul sarcinii –alcool, cocaina,derivati de vit. A, sarcinii –alcool, cocaina,derivati de vit. A, cumarinice, thallidomida, litiu, ac. valproic, cumarinice, thallidomida, litiu, ac. valproic, estrogeni (posibil)estrogeni (posibil)Boli materne: diabet insulinodependent, Boli materne: diabet insulinodependent, coagulopatii, rubeola, toxoplasmoza, hepatita coagulopatii, rubeola, toxoplasmoza, hepatita viralaviralaAgregare familiala (DSA)Agregare familiala (DSA)Alte dezordini genetice: S. Down, S. TurnerAlte dezordini genetice: S. Down, S. Turner
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EmbriogenezaEmbriogeneza
Crawford 2004
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Circulatia fetalaCirculatia fetala
50% - AD-VD - AP- canal art- aorta fetala50% - AD-VD - AP- canal art- aorta fetala
45-50% -AD – Foramen ovale – AS - VS45-50% -AD – Foramen ovale – AS - VS
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PrognosticPrognosticAmeliorat in ultimele 3 decenii prin corectie Ameliorat in ultimele 3 decenii prin corectie chirurgicala, valvulotomie cu balon, inchidere chirurgicala, valvulotomie cu balon, inchidere interventionala a DSA si canalului arterial interventionala a DSA si canalului arterial persistentpersistentterap. cu PGE1 si indometacin pentru inchiderea terap. cu PGE1 si indometacin pentru inchiderea canalului arterial patentcanalului arterial patentPacientii corectati chirurgical – risc de aritmii, Pacientii corectati chirurgical – risc de aritmii, MS, leziuni reziduale sau recurente, sechele ale MS, leziuni reziduale sau recurente, sechele ale chirurgiei, endocardita, disf. contractilachirurgiei, endocardita, disf. contractilaPacientii neoperati – HTP severa, complicatiile Pacientii neoperati – HTP severa, complicatiile hipoxemiei cronice, insuf. cardiaca, aritmii, hipoxemiei cronice, insuf. cardiaca, aritmii, supravietuire redusasupravietuire redusa
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ClasificareClasificare
Cardiopatii cianogeneCardiopatii cianogene – tetralogia Fallot – tetralogia Fallot (sunt+obstacol)(sunt+obstacol)Cardiopatii cianogene prin inversare de Cardiopatii cianogene prin inversare de sunt (sunt (Sd. EisenmengerSd. Eisenmenger))Cardiopatii necianogeneCardiopatii necianogene – cu sunt stg-dr – cu sunt stg-dr (DSA, DSV)(DSA, DSV)- - cu obstacol - coarctatie de aortacu obstacol - coarctatie de aorta
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Fiziopatologia suntuluiFiziopatologia suntului
Suntul stg-dr : P=QxRSuntul stg-dr : P=QxRLa rezistente normale arteriolare –PAPs La rezistente normale arteriolare –PAPs creste usorcreste usorLa rezistente crescute arteriolare – La rezistente crescute arteriolare – crestere marcata a PAPs (HTP de crestere marcata a PAPs (HTP de rezistentarezistentaSuntul dr- stg – hipoxemie sistemica, Suntul dr- stg – hipoxemie sistemica, poliglobuliepoliglobulie
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Fiziopatologia suntuluiFiziopatologia suntului
Debitul si sensul fluxului prin orificiul Debitul si sensul fluxului prin orificiul comunicant depind decomunicant depind de
-dimensiunile orificiului-dimensiunile orificiului-gradientul de presiune-gradientul de presiune-complianta relativa a VS, VD-complianta relativa a VS, VD-rezistentele arteriolare pulmonare si -rezistentele arteriolare pulmonare si sistemicesistemice-fluxul preferential-fluxul preferential
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Criterii de suspiciune clinica Criterii de suspiciune clinica pentru cardiopatii congenitalepentru cardiopatii congenitale
Intarziere in crestereIntarziere in crestereDispnee, fatigabilitate la tineriDispnee, fatigabilitate la tineriCianoza de tip central, hippocratismCianoza de tip central, hippocratismManif. de insuficienta cardiacaManif. de insuficienta cardiacaAuscultatie cardiaca patologicaAuscultatie cardiaca patologicaAritmiiAritmii
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Defectul septal atrialDefectul septal atrial
Rel. frecvent, corectabil usor chirurgicalRel. frecvent, corectabil usor chirurgical6-10% din totalul malformatiilor cardiovasc6-10% din totalul malformatiilor cardiovascfrecventa dubla la femeifrecventa dubla la femeiSd. Holt-Oram –asoc DSA cu anomalii ale Sd. Holt-Oram –asoc DSA cu anomalii ale extremitatilor superioareextremitatilor superioareSunt stg-dr asimptomatic in prima parte a Sunt stg-dr asimptomatic in prima parte a vietiivietii
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DSA- anatomieDSA- anatomie
Tip ostium secundum, tip foramen ovale Tip ostium secundum, tip foramen ovale patentpatentTip ostium primum (asoc frevent cu cleft Tip ostium primum (asoc frevent cu cleft de valva mitrala)de valva mitrala)Tip sinus venos (asoc. frevent cu retur Tip sinus venos (asoc. frevent cu retur venos pulmonar aberant in AD)venos pulmonar aberant in AD)
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DSADSA Anatomie Anatomie
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DSA cu sunt stg-drDSA cu sunt stg-dr
Topol 2007
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DSA - fiziopatologieDSA - fiziopatologieSuntul prin DSA este determinat de complianta Suntul prin DSA este determinat de complianta relativa a celor 2 ventriculi si nu de dimensiunea relativa a celor 2 ventriculi si nu de dimensiunea orificiuluiorificiuluiVD este in general mai compliant ca VSVD este in general mai compliant ca VSDirectia fluxului sanguin prin DSA –conditionat Directia fluxului sanguin prin DSA –conditionat de gradientul de presiune intre cele 2 atriide gradientul de presiune intre cele 2 atriiSuntul frecvent stg-drSuntul frecvent stg-drFluxul pulmonar creste de 3-4 ori, rezistentele Fluxul pulmonar creste de 3-4 ori, rezistentele pulmonare sant initial normale si presiunile pulmonare sant initial normale si presiunile arteriale pulmonare cresc putinarteriale pulmonare cresc putin
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DSA –Efectul pe circulatia DSA –Efectul pe circulatia pulmonarapulmonara
Supraincarcarea cr. de debit- dilatatia Supraincarcarea cr. de debit- dilatatia patului vascular , hipertrofia arteriolara a patului vascular , hipertrofia arteriolara a medieimedieiCca 10% - HTP severa, de rezistentaCca 10% - HTP severa, de rezistenta
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DSA – efecte pe structurile DSA – efecte pe structurile cardiacecardiace
Suntul important stg-dr produce dilatatie Suntul important stg-dr produce dilatatie de cavitati drepte si HVDde cavitati drepte si HVDDilatatia inelului tricuspidian si pulmonar si Dilatatia inelului tricuspidian si pulmonar si incompetenta valvularaincompetenta valvularaDilatatie importanta de trunchi al a. Dilatatie importanta de trunchi al a. pulmonarepulmonareCavit. stg de obicei nedilatateCavit. stg de obicei nedilatate
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DSA – ex. clinic -simptomeDSA – ex. clinic -simptome
Asimptomatici in copilarie (inf. pulmonare Asimptomatici in copilarie (inf. pulmonare recurente)recurente)Dispnee de efort Dispnee de efort FatigabilitateFatigabilitate
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DSA –ex. obiectiv(I)DSA –ex. obiectiv(I)
Impuls parasternal (VD)Impuls parasternal (VD)Dedublare larga, fixa de S IIDedublare larga, fixa de S IISuflu sistolic ejectional gr II-III in Erb (debit Suflu sistolic ejectional gr II-III in Erb (debit crescut pulmonar)crescut pulmonar)Suflu diastolic parasternal(de debit crescut Suflu diastolic parasternal(de debit crescut transtricuspidian)transtricuspidian)
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DSA-ex. obiectiv (II)DSA-ex. obiectiv (II)
HTP de rezistenta – dedublarea larga de HTP de rezistenta – dedublarea larga de SII poate sa disparaSII poate sa disparaPII poate deveni mai intensaPII poate deveni mai intensaSuflul sistolic devine mai scurtSuflul sistolic devine mai scurtCianoza – marca a inversarii suntului prin Cianoza – marca a inversarii suntului prin DSA dat. HTP (SD. Eisenmenger)DSA dat. HTP (SD. Eisenmenger)
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DSA - diagnosticDSA - diagnostic
Rx. pulmonar – cardiomegalie, dilatatia Rx. pulmonar – cardiomegalie, dilatatia arcului inf. dr. (AD), proeminenta golfului arcului inf. dr. (AD), proeminenta golfului cardiac (AP), dilat. tr. AP in hiluri, desen cardiac (AP), dilat. tr. AP in hiluri, desen pulmonar accentuat (vascular)pulmonar accentuat (vascular)
ECG – deviere ax QRS dreapta (rsR), ECG – deviere ax QRS dreapta (rsR), aritmii supraventriculare, BAV gr.Iaritmii supraventriculare, BAV gr.I
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DSA – diagnostic (II)DSA – diagnostic (II)Ecocardiografie – dilatatie de cavitati dr. Ecocardiografie – dilatatie de cavitati dr.
- sunt interatrial(tip)- sunt interatrial(tip)- HTP (regurgit. tricusp., miscare - HTP (regurgit. tricusp., miscare
paradoxala de SIV)paradoxala de SIV)- prolaps de valva mitrala- prolaps de valva mitrala
TEETEEEco cu contrastEco cu contrast3D Eco 3D Eco
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DSADSA
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DSADSA
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DSADSA
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DSA – 3DDSA – 3D
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DSA – 3DDSA – 3D
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DSA – diagnostic (III)DSA – diagnostic (III)
Cateterism cardiac – nu este de obicei Cateterism cardiac – nu este de obicei necesarnecesarBoli asociate (BPOC, BCI)Boli asociate (BPOC, BCI)
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DSA - tratamentDSA - tratament
Reparare chirurgicala – tratamentul de Reparare chirurgicala – tratamentul de electie pt pac. cu flux pulmonar/sistemic electie pt pac. cu flux pulmonar/sistemic >1.5/1, daca pacientul este asimptomatic >1.5/1, daca pacientul este asimptomatic sau paucisimptomaticsau paucisimptomaticLimitari functionale asociate cu varsta – Limitari functionale asociate cu varsta – ICC, aritmii atriale, boala vasculara ICC, aritmii atriale, boala vasculara pulmonara (5-10% din pac. netratati)pulmonara (5-10% din pac. netratati)
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DSA - chirurgieDSA - chirurgie
Repararea electiva – frecent dupa varsta Repararea electiva – frecent dupa varsta de 4-5 anide 4-5 aniIndicata la copii cu ICCIndicata la copii cu ICCSternotomie mediana: abord submamar Sternotomie mediana: abord submamar dreptdreptSutura cu petec de pericard sau material Sutura cu petec de pericard sau material proteticproteticInchidere interventionalaInchidere interventionala
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Indicatii de interventie in DSAIndicatii de interventie in DSA
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DSA- prognosticDSA- prognosticPac. neoperati – simptome dupa decada a Pac. neoperati – simptome dupa decada a 4a4aInchidere spontana – 14-66% - nelegata Inchidere spontana – 14-66% - nelegata de simptome (mecanism necunoscut)de simptome (mecanism necunoscut)Pac. operati – supravietuirea (27 ani dupa Pac. operati – supravietuirea (27 ani dupa reparare) – 40%reparare) – 40%Prezenta HTP moderata Prezenta HTP moderata (PAPS>40mmHg) – efect advers marcat (PAPS>40mmHg) – efect advers marcat pe supravieturepe supravieture
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DSV - DefinitieDSV - Definitie
Defect congenital al SIV cu comunicare Defect congenital al SIV cu comunicare unica sau multiple intre VS si VDunica sau multiple intre VS si VDLocalizare in septul membranos sau in Localizare in septul membranos sau in septul muscularseptul muscularSunt stg-dr si incarcare de presiune a VD Sunt stg-dr si incarcare de presiune a VD (HTP de debit, iar ulterior HTP de (HTP de debit, iar ulterior HTP de rezistenta) si incarcare de volum a VSrezistenta) si incarcare de volum a VS
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DSV perimembranos cu sunt DSV perimembranos cu sunt stg-drstg-dr
Topol 2007
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DSVDSVSupravietuire mai redusa neoperatSupravietuire mai redusa neoperatConsecintele depind de diam orif si rezistentele Consecintele depind de diam orif si rezistentele pulmonarepulmonareDefectele largi – sunt mare stg-dr si egalizarea Defectele largi – sunt mare stg-dr si egalizarea presiunilor in VS, VDpresiunilor in VS, VDRezistentele mari inversarea suntului – cianoza Rezistentele mari inversarea suntului – cianoza (Sd Eisenmenger)(Sd Eisenmenger)Dispnee, fatigabilitateDispnee, fatigabilitateSuflu sistolic, freamat, rulment diastolic mitralSuflu sistolic, freamat, rulment diastolic mitralCardiomegalie, hiperpulsatilitatea VSCardiomegalie, hiperpulsatilitatea VSSemne de HTPSemne de HTP
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DSVDSV
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Anevrism al SIVAnevrism al SIV
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DSV multipleDSV multiple
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Indicatii pentru interventie in DSVIndicatii pentru interventie in DSV
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Persistenta de canal arterialPersistenta de canal arterial
Clinic – suflu continuu subclavicular stang Clinic – suflu continuu subclavicular stang cu red TA diastolice, rulment cu red TA diastolice, rulment diastolic mitraldiastolic mitral
- semne variabile de HTP- semne variabile de HTP
ECG - HVS, HVDECG - HVS, HVDRx – semne de HTPRx – semne de HTP
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Persistenta de canal Persistenta de canal arterialarterial
Topol 2007
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Persistenta de canal art.-Persistenta de canal art.-evolutie, complicatiievolutie, complicatii
Suflu sistolic atipicSuflu sistolic atipicInversarea suntului – cianozaInversarea suntului – cianozaRisc de endocardita, Risc de endocardita, Insuficienta cardiacaInsuficienta cardiacaHTP severaHTP severaAnevrisme, ruptura de canalAnevrisme, ruptura de canal
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Persistenta de canal arterialPersistenta de canal arterial
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Persistenta de canal arterialPersistenta de canal arterial
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Tehnica embolizarii in PCATehnica embolizarii in PCA
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Indicatii pentru interventie in Indicatii pentru interventie in persistenta de canal arterialpersistenta de canal arterial
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Stenoza pulmonaraStenoza pulmonara
Clinic – suflu sistolic ejectional sp. II ic stgClinic – suflu sistolic ejectional sp. II ic stgFreamat sistolic Freamat sistolic ECG - HVD severa, cu fortare de VDECG - HVD severa, cu fortare de VDRx – dilat. trunchiului AP, plaman clarRx – dilat. trunchiului AP, plaman clar
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Indicatii de interventie in obstructia Indicatii de interventie in obstructia tractului de ejectie al VDtractului de ejectie al VD
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Coarctatia de aortaCoarctatia de aorta
Clinic – HTA la membrele superioare, la Clinic – HTA la membrele superioare, la tineritineri - puls arterial absent la aa. femurale - puls arterial absent la aa. femurale bilateral, sufluri art. toracicebilateral, sufluri art. toracice - hipotrofia trunchi, membre - hipotrofia trunchi, membre inferioareinferioare
Rx – eroziuni costale, dublu buton aorticRx – eroziuni costale, dublu buton aorticECG - HVSECG - HVS
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Coarctatie de aortaCoarctatie de aorta
Topol 2007
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Coarctatia de aorta - Coarctatia de aorta - consecinteconsecinte
HVS severaHVS severaAnevrisme artAnevrisme artCirc colaterala (subclavia –ao, intercost – Circ colaterala (subclavia –ao, intercost – periscapular, mamara int – epig)periscapular, mamara int – epig)Insuficienta cardiacaInsuficienta cardiacaSupravietuire spont – 35 aniSupravietuire spont – 35 ani
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Coarctatia de aorta -RxCoarctatia de aorta -Rx
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Coarctatia de aortaCoarctatia de aortaPlastie cu balonPlastie cu balon
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Indicatii de interventie in coarctatia Indicatii de interventie in coarctatia de aortade aorta
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Tetralogia FallotTetralogia Fallot
Topol 2007
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Indicatii pentru interventie in Tetralogia Indicatii pentru interventie in Tetralogia FallotFallot
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Maladia EbsteinMaladia Ebstein
Cianogena – prin DSA cu sunt dr.-stg. dat. Cianogena – prin DSA cu sunt dr.-stg. dat. presiunilor mari din AD presiunilor mari din AD Anomalie de implantare a valvei tricuspideAnomalie de implantare a valvei tricuspide““atrializarea” unei parti din VDatrializarea” unei parti din VDInsuficienta cardiaca dreapta “pura”Insuficienta cardiaca dreapta “pura”Auscultatie de stenoza/regurgitare Auscultatie de stenoza/regurgitare tricuspidianatricuspidiana
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Anomalia EbsteinAnomalia Ebstein
Topol 2007
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Indicatii pentru interventie in Indicatii pentru interventie in anomalia Ebsteinanomalia Ebstein
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Transpozitia completa de vase Transpozitia completa de vase mari-reparare chirurgicalamari-reparare chirurgicala
Topol 2007
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Sindrom EisenmengerSindrom EisenmengerHTP cu inversarea suntuluiHTP cu inversarea suntului
Topol 2007
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Tetralogie Fallot reparata, cu IC drTetralogie Fallot reparata, cu IC drDextrocardie, situs inversusDextrocardie, situs inversus
MRIMRI
Topol 2007
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Recomandari de tratament in HTP din Recomandari de tratament in HTP din cardiopatiile congenitalecardiopatiile congenitale