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