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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 carediaceEpidemiologie

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

  • Boli congenitale cardiace - Necianogene - Cianogene

    - Circulatie pulmonara bogata- Circulatie pulmonara saraca

  • BOLI CARDIACE CONGENITALE NECIANOGENE CU SUNT STG-DRunt stanga dreapta atrial DSA DSA + SM ( Sindr. Lutambacher)anomaliide insertie a 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 aortic root in cord dreptarterea coronara stg din AP fistula arteriovenoasa coronaraRuptura de sinus anevrism Valsalva

  • BOLI CARDIACE CONGENITALE CIANOGENE Cresterea fluxului pulmoanar transpozitia complata a marilor vase trunchiul arterial anomalie totala de conectie a VP ventricul unic flux pulmonar normal sau decrescut boala Ebstein atresia AP, atresia tricuspida tetralogia Fallot

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

  • Patogenie anomalii cardiocircultorii 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

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

  • 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 deformare redusa a eritrociteor in microcirculatie si sindr de hipervascozitate la Ht mai mic

  • ERITROCITOZA - flebotomia - 500ml in 45 minute- compensata volumic - sol izotona glucoza(5%)- tratamentul cu Fe* pentru hipervascoziatea cu microcitoza (atentie nu rapid pentru a nu stimula eritrocitoza rapida!)

  • Boli congenitale cianogene - hemostaza anormala - crestere volumica, angorjare vasculara - functie trombocitara anormala - anomalii coagulare extriseca + intrinseca - contravceptive 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 intararea venei cave 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

  • - 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 - HP se accentueaza prin- hipoxie (altitudine, etc) - scaderea compliantatei VS (BCI, varsta) - HP 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 reparat cu petec

  • - 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/adult- prognostic - supravietuire > 40 - 50 ani - simptome/deteriorare - hemodinamica ce se agraveaza progresiv la > 50 ani

  • - DSA - HP 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 - HP cu sunt bidirectional - cianoza - suflurile diminua - Zg II accentuat, fara dedublare - apare suflu de RT - ECG- deviatie axiala dreapta - bloc AV grad I (sinus venos) - deviatie axiala stanga ostium primum - aritmii atriale origine in AD- HAD, HVD

  • - DSA - Rx cord-plaman- AP > - 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 tarnsatrial stg-dr

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

  • - Tratament - inchidere chirurgicala cu petec de pericard, material prostetic - ideal: 3-6 ani, cand raportul: flux pulmonar / flux sistemic >2:1 - inchidere chirurgicala si reparie valvulara- ostium primum

  • - Defect septal ventricular DSV - cea mai frecventa anomalie congnitala izolata si cea mai frecventa anomalie din combinatii - simpome/evolutie - dependente de - marimea defectului - starea circulatiei pulmonare- asocierea IA, prolaps VA- DSV mic/moderat adolescent/adult - DSV mare prima copilarie

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

  • Prognostic DSV marimea suntului DSV hipertensiunea pulmonara marimea HP 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

  • Canal arterial (CA)

    AP la bifurcatie - Ao distal de a subclavie - CA prezent la fetus, dispare la nastere fluxul CA depinde de - diametru, lungimea CA - rezistenta sistemica/pulmonara - rezistenta sistemica sistolica si diastolica Ao> AP

  • Canal arterial examen fizic - suflu de masinarie RX cord plaman - AP > - circulatie pulmonara > - AD>,VD> ECG - HAD,HVD > ECO cord - AD,VD >

  • Canal arterial evolutie 1. - sunt stanga - dreapta cu incarcare volumica pulmonara - Sindr Eisenmenger - inversarea suntului dreapta- stanga inaorta descendenta cianoza degetelor picioarelor nu si a mainii - cianoza diferentiala

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

  • Canal arterial tratament

    1. ligatura dutului - in absenta HP severe

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

  • Ruptura anevrism sinus Valsalva . anevrismul sinusului Valsalva - lipsa de fuziune intre media Ao si inelul fibros al valvei Ao

    - ruptura intre cuspa coronara dr si VD, rar AD, incarcare de volum VD Manifestari clinice: - 30-40 ani - durere retrosternala - suflu continuu, acentuat diastolic Eco diagnosticaTratament sutura chirurgicala

  • Boli cianotice congenitale

  • Boala Ebstein anomalii de atasare 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: - PR >, BRD

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

    ECO Doppler - gradul de RT

  • Boala Ebstein Rx antero-posterior - cord sabot AD marit- Circulatia pulmonara