CURS Boli Congenitale 2012

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<ul><li><p>BOLI CONGENITALE CARDIACE</p></li><li><p>Malformatii prin anomalii embrionare cu insuficienta in dezvotarea embrionara sau fetala a cordului Definitie</p></li><li><p>1% din nasteri 4% din nasterile mamelor cu boli congenitale cardiaceEpidemiologie</p></li><li><p> complex multifactorial genetic&lt; 10% anomalii ale unei singure geneEtiologie componente in boli genetice cu determinari sistemice sindr. Down, Turner diagnostic prenatal anomalii cromozomiale fetale</p></li><li><p>- Necianogene - Cianogene</p><p>- Circulatie pulmonara bogata- Circulatie pulmonara saracaBoli congenitale cardiace </p></li><li><p>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</p></li><li><p>BOLI CARDIACE CONGENITALE NECIANOGENE CU UNT STG-DRunt stanga dreapta aorto-pulmonar canal arterial fereastra aorto-pulmonara</p><p> din radacina aortei in cord dreptarterea coronara stg din AP fistula arteriovenoasa coronararuptura de anevrism sinus Valsalva</p></li><li><p>BOLI CARDIACE CONGENITALE FARA UNT cordul stang SM SA coarctatia de aorta insuficienta aortica cordul drept boala Ebstein SP, IP dilatatia de trunchi AP</p></li><li><p>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</p></li><li><p>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</p></li><li><p>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</p></li><li><p>ERITROCITOZA - flebotomia - 500ml in 45 minute- compensata volumic - sol izotona glucoza(5%)- tratamentul cu Fe* pentru hipervascoziatea cu microcitoza </p></li><li><p>Generalitati - bolile cardiace congenitale anomalii cardiocirculatorii - diagnosticate la nastere tolerate pana in adolescenta / adult (bicuspidia aortica, DSA, DSV mic) </p></li><li><p>Coarctatia de aortaIngustare a Ao descendente, de obicei distal de originea A subclavii stangi</p><p>B&gt;F</p><p>Fiziopatologie:</p><p> TAS si TAD deasupra coarctatieiSub nivelul coarctatiei TAS&lt; decat la mb sup</p></li><li><p>Coarctatia de aortaDate clinice:</p><p>Gradient sistolic tensional Mb sup/inf</p><p>Amplitudine scazuta puls femural</p><p>Suflu sistolic ejectional interscapulo-vertebral/interscapulo-vert stg</p></li><li><p>Coarctatia de aortaECG: HVS+DAS</p></li><li><p>Coarctatia de aortaRX:</p><p>Dilatare Ascl stgIndentatia CoAoDilatare post-stenotica Ao</p></li><li><p>Coarctatia de aortaEocardiografie:</p></li><li><p>Coarctatia de aortaEocardiografie:</p></li><li><p>Coarctatia de aortaCT:</p></li><li><p>Coarctatia de aortaRMN:</p></li><li><p>Coarctatia de aortaAngiografie:</p></li><li><p>Coarctatia de aortaTratament:Interventional de electie</p></li><li><p>Coarctatia de aortaTratament:Interventional de electie</p></li><li><p>Coarctatia de aortaTratament:</p><p>Chirurgical</p><p> La pac cu Co A si HTA semnificativa</p><p> Asimptomatici cu CoA stransa normotensivi in repaus, dar cu raspuns exagerat la efort</p></li><li><p>Coarctatia de aortaTratament:</p><p>Chirurgical</p></li><li><p>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 </p></li><li><p>- 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</p></li><li><p>- Defectul septal atrial</p></li><li><p>- Marimea fluxului prin DSA - dependent de - marimea DSA - proprietati distolice AS,VS - impedanta pulmonara, sistemica </p><p> 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.</p><p>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</p></li><li><p>- DSA </p><p> 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.</p><p>Copyright 2001 McGraw-Hill. All rights reserved.- asociaza - aritmii atriale - infectii pulmonare - insuficienta cardiaca- risc mic de endocardita infectioasa daca: - nu asociaza IM </p></li><li><p>- DSA </p><p> 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.</p><p>Copyright 2001 McGraw-Hill. All rights reserved.Evolutie - asimptomatica simptomatica - adolescent/adultAlterare hemodinamica clinic evidenta daca debit pulmonar/debit sistemic &gt;1.5/1 </p></li><li><p>- DSA </p><p> 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.</p><p>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 &gt; 80 mm Hg efortul fizic este de obicei factorul declansator</p></li><li><p>- DSA </p><p> 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.</p><p>Copyright 2001 McGraw-Hill. All rights reserved.Prognostic: - supravietuire &gt; 40 - 50 ani - simptome/deteriorare - hemodinamica ce se agraveaza progresiv la &gt; 50 ani </p></li><li><p>- 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</p></li><li><p>- DSA - ECG- deviatie axiala dreapta - bloc AV grad I (sinus venos) - deviatie axiala stanga ostium primum - aritmii atriale origine in AD- DAD, HVD</p></li><li><p>- DSA - Rx cord-plamanAP &gt; - AD &gt;, VD &gt; - circulatie pulmonara crescuta </p></li><li><p>- ECO ( parasternal, apical, subcostal) - ECO 2D - AD &gt;, VD &gt;- miscare paradoxala SIV - ECO transesofagian - informatii neclare la ECO transtoracic- anomalii congenitale complexe - ECO Doppler flux color- flux transatrial stg-dr </p></li><li><p>- Cateterism cardiac - Hipertensiune pulmonara - anomalii congenitale asociate- suspiciune boala coronara asociata </p></li><li><p> Indicatiile inchiderii DSAQp/Qs &gt; 1.5/1RVP/RVS &lt; 0.7:1</p><p>Inchiderea percutana a DSADe electie DSA OII forma necomplicataDSA &gt; 25 mm- Tratament </p></li><li><p> Inchidere chirurgicala a DSA - ideal: 3-6 ani, cand raportul: flux pulmonar / flux sistemic &gt;2:1 - inchidere chirurgicala si reparie valvulara- ostium primum - Tratament </p></li><li><p>- Defect septal ventricular DSV - cea mai frecventa anomalie congenitala izolata si in combinatii </p></li><li><p>Defect septal ventricular DSVLocalizare anatomica </p></li><li><p>Defect septal ventricular + IASdr Pezzi-Laubry</p></li><li><p>- 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</p></li><li><p> Defect septal ventricular DSV - DSV mic, restrictiv: PAPs N, sunt sistolicDSV moderat restrictiv: PAPs , dar &lt; Pao, sunt sistolic + diastolic mic DSV nerestrictiv PAPs=Pao, sunt sist+diast</p></li><li><p>- 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 </p></li><li> Prognostic DSV marimea suntului DSV hipertensiunea pulmonara marimea HTP influenteaza prognosticul - rezistenta pulmonara </li><li><p> Rx cord plaman</p><p> - VD&gt; - VD+VS&gt; - AP &gt; - circulatie pulmonara crescuta </p></li><li><p> ECO - 2D-Eco VD&gt;, VS&gt; - Doppler - suntul si directia fluxului interventricular Cateterism caracteristici circulatie pulmonara - anomalii asociate </p></li><li><p> Tratament DSV</p><p> DSV mare/moderat - fluxul pulmonar/sistemic: 1,5 - 2 - rezistenta pulmonara/sistemica </p></li><li><p> Tratament DSV</p><p>Tratamentul interventional:</p></li><li><p> Tratament DSV</p><p>Tratamentul interventional:</p></li><li><p> Persistenta canal...</p></li></ul>