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BOLI CONGENITALE CARDIACE

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  • BOLI CONGENITALE CARDIACE

  • Malformatii prin anomalii embrionare cu insuficienta in dezvotarea embrionara sau fetala a cordului Definitie

  • 1% din nasteri 4% din nasterile mamelor cu boli congenitale cardiaceEpidemiologie

  • complex multifactorial genetic< 10% anomalii ale unei singure geneEtiologie componente in boli genetice cu determinari sistemice sindr. Down, Turner diagnostic prenatal anomalii cromozomiale fetale

  • - Necianogene - Cianogene

    - Circulatie pulmonara bogata- Circulatie pulmonara saracaBoli congenitale cardiace

  • BOLI CARDIACE CONGENITALE NECIANOGENE CU SUNT STG-DRunt stanga dreapta atrial DSA DSA + SM ( Sindr. Lutanbacher)Anomalii de insertie ale VP ventricularDSV DSV + IAla nivele multiplecanal atrioventricular comun

  • BOLI CARDIACE CONGENITALE NECIANOGENE CU UNT STG-DRunt stanga dreapta aorto-pulmonar canal arterial fereastra aorto-pulmonara

    din radacina aortei in cord dreptarterea coronara stg din AP fistula arteriovenoasa coronararuptura de anevrism sinus Valsalva

  • BOLI CARDIACE CONGENITALE FARA UNT cordul stang SM SA coarctatia de aorta insuficienta aortica cordul drept boala Ebstein SP, IP dilatatia de trunchi AP

  • Patogenie anomalii cardiocirculatorii modificari hemodinamice cardiace incarcare presiune - SA, SP - incarcare de volum - sunturile intracardiace - sunturi extracardiace - incarcare volum/presiune - HP sindromul Eisenmenger - formele cianogene: - eritrocitoza - sindrom hipevascozitate

  • HIPERTENSIUNEA PULMONARA - cresterea fluxului sau/si rezistenteivaselor pulmanare Sindromul Eisenmenger - modificari obstructive/obliterative a vaselor pulmonare (remodelare vasculara) progresive, independente - unt important stanga - dreapta - HTP initial de debit - HTP remodelare vasculara - evolutie ireversibila - fara raspuns la terapia medicamentoasa - tratament eficient: transplant pulmonar + corectia bolii cardiace

  • ERITROCITOZA - hipoxemie eritropoietina - eritrocitoza - eritrocioza compensata Ht 65% - sindrom de hipervascozitate- flebotomia - deperditie de Fe - microcitoza plasticitateredusa a eritrociteor in microcirculatie si sindrom de hipervascozitate la Ht mic

  • ERITROCITOZA - flebotomia - 500ml in 45 minute- compensata volumic - sol izotona glucoza(5%)- tratamentul cu Fe* pentru hipervascoziatea cu microcitoza

  • Generalitati - bolile cardiace congenitale anomalii cardiocirculatorii - diagnosticate la nastere tolerate pana in adolescenta / adult (bicuspidia aortica, DSA, DSV mic)

  • Coarctatia de aortaIngustare a Ao descendente, de obicei distal de originea A subclavii stangi

    B>F

    Fiziopatologie:

    TAS si TAD deasupra coarctatieiSub nivelul coarctatiei TAS< decat la mb sup

  • Coarctatia de aortaDate clinice:

    Gradient sistolic tensional Mb sup/inf

    Amplitudine scazuta puls femural

    Suflu sistolic ejectional interscapulo-vertebral/interscapulo-vert stg

  • Coarctatia de aortaECG: HVS+DAS

  • Coarctatia de aortaRX:

    Dilatare Ascl stgIndentatia CoAoDilatare post-stenotica Ao

  • Coarctatia de aortaEocardiografie:

  • Coarctatia de aortaEocardiografie:

  • Coarctatia de aortaCT:

  • Coarctatia de aortaRMN:

  • Coarctatia de aortaAngiografie:

  • Coarctatia de aortaTratament:Interventional de electie

  • Coarctatia de aortaTratament:Interventional de electie

  • Coarctatia de aortaTratament:

    Chirurgical

    La pac cu Co A si HTA semnificativa

    Asimptomatici cu CoA stransa normotensivi in repaus, dar cu raspuns exagerat la efort

  • Coarctatia de aortaTratament:

    Chirurgical

  • Boli congenitale cianogene - hemostaza anormala - crestere volumica, angorjare vasculara - functie trombocitara anormala - anomalii coagulare extriseca + intrinseca - contraceptive contraindiacate !!!!!- risc stroke - boli cianogene - eritroza asociata cu: - deshidratare - aritmii atriale - endocardita infectioasa

  • - Defectul septal atrial- cea mai comuna anomalie congenitala la adult, mai frecventa la femei- de tip sinus venos - aproape de intrarea VCS/VCI in AD - asociaza anomalii de conectare a VP - de tip ostium primum - aproape de valvele AV , deformari valve AV mai frecvent cele posterioare, +/- DSV, frecvent in S Down - de tip ostium secundum - medioseptal, anomalie anatomica si functionala - foramen ovale - fara consecinte functionale

  • - Defectul septal atrial

  • - Marimea fluxului prin DSA - dependent de - marimea DSA - proprietati distolice AS,VS - impedanta pulmonara, sistemica

    Patients with atrial septal defect are usually asymptomatic in early life, although there may be some physical underdevelopment and an increased tendency for respiratory infections; cardiorespiratory symptoms occur in many older patients. Beyond the fourth decade, a significant number of patients develop atrial arrhythmias, pulmonary arterial hypertension, bidirectional and then right-to-left shunting of blood, and cardiac failure. Patients exposed to the chronic environmental hypoxia of high altitude tend to develop pulmonary hypertension at younger ages. In some older patients, left-to-right shunting across the defect increases as progressive systemic hypertension and/or coronary artery disease result in reduced compliance of the LV.

    Copyright 2001 McGraw-Hill. All rights reserved.- determina - HTP se accentueaza prin- hipoxie (altitudine, etc) - scaderea compliantei VS (BCI, varsta) - HTP determina sunt bidirectional prin aparitia sunt dr-stg

  • - DSA

    Patients with atrial septal defect are usually asymptomatic in early life, although there may be some physical underdevelopment and an increased tendency for respiratory infections; cardiorespiratory symptoms occur in many older patients. Beyond the fourth decade, a significant number of patients develop atrial arrhythmias, pulmonary arterial hypertension, bidirectional and then right-to-left shunting of blood, and cardiac failure. Patients exposed to the chronic environmental hypoxia of high altitude tend to develop pulmonary hypertension at younger ages. In some older patients, left-to-right shunting across the defect increases as progressive systemic hypertension and/or coronary artery disease result in reduced compliance of the LV.

    Copyright 2001 McGraw-Hill. All rights reserved.- asociaza - aritmii atriale - infectii pulmonare - insuficienta cardiaca- risc mic de endocardita infectioasa daca: - nu asociaza IM

  • - DSA

    Patients with atrial septal defect are usually asymptomatic in early life, although there may be some physical underdevelopment and an increased tendency for respiratory infections; cardiorespiratory symptoms occur in many older patients. Beyond the fourth decade, a significant number of patients develop atrial arrhythmias, pulmonary arterial hypertension, bidirectional and then right-to-left shunting of blood, and cardiac failure. Patients exposed to the chronic environmental hypoxia of high altitude tend to develop pulmonary hypertension at younger ages. In some older patients, left-to-right shunting across the defect increases as progressive systemic hypertension and/or coronary artery disease result in reduced compliance of the LV.

    Copyright 2001 McGraw-Hill. All rights reserved.Evolutie - asimptomatica simptomatica - adolescent/adultAlterare hemodinamica clinic evidenta daca debit pulmonar/debit sistemic >1.5/1

  • - DSA

    Patients with atrial septal defect are usually asymptomatic in early life, although there may be some physical underdevelopment and an increased tendency for respiratory infections; cardiorespiratory symptoms occur in many older patients. Beyond the fourth decade, a significant number of patients develop atrial arrhythmias, pulmonary arterial hypertension, bidirectional and then right-to-left shunting of blood, and cardiac failure. Patients exposed to the chronic environmental hypoxia of high altitude tend to develop pulmonary hypertension at younger ages. In some older patients, left-to-right shunting across the defect increases as progressive systemic hypertension and/or coronary artery disease result in reduced compliance of the LV.

    Copyright 2001 McGraw-Hill. All rights reserved.Complicatii HTP 10% cazuri- Emboliile pulmonare si sistemice- Aritmii SV- Bradicardie sinusala, BAV II,III post-operator- Aritmii ventriculare severe si MS la pacienti cu DSA op/neop si HTP > 80 mm Hg efortul fizic este de obicei factorul declansator

  • - DSA

    Patients with atrial septal defect are usually asymptomatic in early life, although there may be some physical underdevelopment and an increased tendency for respiratory infections; cardiorespiratory symptoms occur in many older patients. Beyond the fourth decade, a significant number of patients develop atrial arrhythmias, pulmonary arterial hypertension, bidirectional and then right-to-left shunting of blood, and cardiac failure. Patients exposed to the chronic environmental hypoxia of high altitude tend to develop pulmonary hypertension at younger ages. In some older patients, left-to-right shunting across the defect increases as progressive systemic hypertension and/or coronary artery disease result in reduced compliance of the LV.

    Copyright 2001 McGraw-Hill. All rights reserved.Prognostic: - supravietuire > 40 - 50 ani - simptome/deteriorare - hemodinamica ce se agraveaza progresiv la > 50 ani

  • - DSA - HTP cu sunt bidirectional - cianoza - - suflurile diminua - Zg II accentuat, fara dedublare - apare suflu de RT - examen fizic- suflu mezosistolic pulmonar de ejectie - dedublare fixa Zg II, neinfluentata de respiratie - suflu mezodiastolic tricuspidian de debit - Zg I intarit si dedublat la tricuspida

  • - DSA - ECG- deviatie axiala dreapta - bloc AV grad I (sinus venos) - deviatie axiala stanga ostium primum - aritmii atriale origine in AD- DAD, HVD

  • - DSA - Rx cord-plamanAP > - AD >, VD > - circulatie pulmonara crescuta

  • - ECO ( parasternal, apical, subcostal) - ECO 2D - AD >, VD >- miscare paradoxala SIV - ECO transesofagian - informatii neclare la ECO transtoracic- anomalii congenitale complexe - ECO Doppler flux color- flux transatrial stg-dr

  • - Cateterism cardiac - Hipertensiune pulmonara - anomalii congenitale asociate- suspiciune boala coronara asociata

  • Indicatiile inchiderii DSAQp/Qs > 1.5/1RVP/RVS < 0.7:1

    Inchiderea percutana a DSADe electie DSA OII forma necomplicataDSA > 25 mm- Tratament

  • Inchidere chirurgicala a DSA - ideal: 3-6 ani, cand raportul: flux pulmonar / flux sistemic >2:1 - inchidere chirurgicala si reparie valvulara- ostium primum - Tratament

  • - Defect septal ventricular DSV - cea mai frecventa anomalie congenitala izolata si in combinatii

  • Defect septal ventricular DSVLocalizare anatomica

  • Defect septal ventricular + IASdr Pezzi-Laubry

  • - Defect septal ventricular DSV - simpome/evolutie - dependente de - marimea defectului - starea circulatiei pulmonare- asocierea IA, prolaps VA- DSV mic/moderat adolescent/adult - DSV mare prima copilarie

  • Defect septal ventricular DSV - DSV mic, restrictiv: PAPs N, sunt sistolicDSV moderat restrictiv: PAPs , dar < Pao, sunt sistolic + diastolic mic DSV nerestrictiv PAPs=Pao, sunt sist+diast

  • - DSV hemodinamica unt initial stanga dreapta - simptome: - dispnee - tuse +/- hemoptizie - durere toracica - sincopa incarcare volumica circulatie pulmonara- HTP-Sindrom Eisenmenger (evolutie progresiva, independenta)unt dreapta stanga - cianoza, hippocratism digital, - eritrocitoza- hipervascozitate

  • Prognostic DSV marimea suntului DSV hipertensiunea pulmonara marimea HTP influenteaza prognosticul - rezistenta pulmonara
  • Rx cord plaman

    - VD> - VD+VS> - AP > - circulatie pulmonara crescuta

  • ECO - 2D-Eco VD>, VS> - Doppler - suntul si directia fluxului interventricular Cateterism caracteristici circulatie pulmonara - anomalii asociate

  • Tratament DSV

    DSV mare/moderat - fluxul pulmonar/sistemic: 1,5 - 2 - rezistenta pulmonara/sistemica

  • Tratament DSV

    Tratamentul interventional:

  • Tratament DSV

    Tratamentul interventional:

  • Persistenta canalului arterial (PCA) AP la bifurcatie - Ao distal de a subclavie - CA prezent la fetus, dispare la nastereF/B= 2-3/1

  • Persistenta canalului arterial (PCA)

    Consecintele fiziopatologice depind de: Marimea comunicarii Rezistenta vasculara pulmonara Prezenta grd. De detressa respiratorie Capacitatea functionala a VS

  • Persistenta canalului arterial (PCA) evolutie 1. - sunt stanga - dreapta cu incarcare volumica pulmonara - Sindr Eisenmenger - inversarea suntului dreapta- stanga inaorta descendenta - cianoza diferentiala

    2. insuficienta cardiaca 3. endocardita infectiosa 4. anevrism, ruptura, calcificare duct

  • Persistenta canalului arterial (PCA) examen fizic - suflu sistolo-diastolic de masinarie RX cord plaman - AP > - circulatie pulmonara > - AD>,VD> ECG - HAD,HVD > ECO cord - AD,VD >

  • Persistenta canalului arterial (PCA)

  • Persistenta canalului arterial (PCA)

  • Persistenta canalului arterial (PCA) Tratament - in absenta HP severe

    2. inchiderea cu device intravascular (umbrela, etc..)

  • Persistenta canalului arterial (PCA) Tratament

    2. ligatura ductuluiEficienta si sigura la copiii mici, fara by-pass cardio-pulmonarLa adulti risc op crescut datorita calcificarilor ductale.

  • Boli cianotice congenitale

  • Cresterea fluxului pulmonar transpozitia completa a marilor vase trunchiul arterial ventricul unic Flux pulmonar normal sau scazut boala Ebstein atrezia AP, atrezia tricuspida tetralogia Fallot

    BOLI CARDIACE CONGENITALE CIANOGENE

  • Boala Ebstein anomalii de insertie a VT - VT displazica inserata cu 5mm mai jos decat VM +/- RT - AD marit atrializarea unei parti din VD- VD mic, adesea hipoplazic - DSA (25% cazuri) cu sunt dreapta / stanga

  • Boala Ebstein Clinic: - aritmii atriale, Zg 1 si Zg2 dedublate- suflu sistolic de RT ECG: P inalte gigantice - PR >, BRD (Hiss, RD comprimate de endocardul ingrosat)Asociere cu cai accesorii!!!! TPSV

  • Boala Ebstein ECG:

  • Boala Ebstein ECO 2D anomalia de insertia a VT 5mm sub insertia VM

    ECO Doppler - gradul de RT

  • Boala Ebstein Rx antero-posterior - cord minge de rugby AD marit- Circulatia pulmonara