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MALADII CONGENITALE

CARDIACE

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MCC CIANOGENE

Tetralogia Fallot (trilogie, pentalogie) Atrezia de tricuspid

Hipoplazia de cord stîng Trunchiul arterial comun Transpoziia de vase mari (D- sau L-)

Maladia Ebstein Drenajul venos aberant

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TETRALOGIA FALLOT

10% din MCC ± 35-75% din MCCC Anomalii ± stenoza pulmonar (infundibul)

- DSV- dextropoziia aortei- HVD

Presiuni sistolice egale ± VS, VD, Ao Clinic Radiologic ± cord în ³sabot´

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AD

VS

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TETRALOGIA FALLOT

ECG ± deviere axial dr, HVD Ecocardiografia

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TRILOGIA FALLOT

5% Anomalii ± stenoz pulmonar (valvular)

- DSA ± ostium secundum- HVD Clinic

Radiologic ECG - HAD Ecocardiografic

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PENTALOGIE FALLOT

Anomalii ± stenoza arterei pulmonare- DSA

- DSV- dextropoziia aortei- HVD

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 ATREZIA TRICUSPIDIAN

2-5% Anomalii - absena-imperforaia valvei

- HAD- AS ± mrire progresiv- DSA- VD hipoplazic

- DSV- artera pulmonar - hipoplazie- PCA

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 ATREZIA TRICUSPIDIAN

Clinic ± suflu - DSV, PCA Radiologic -

ECG ± axa la stînga, HAD, HVS Ecocardiografic

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HIPOPLAZIA DE CORD STÎNG

3% - mai ales la Anomalii±valve aortice ± atrezie-hipoplazie

- aorta ± hipoplazic- valva mitral ± atrezie-hipoplazie- DSA ± ostium secundum- PCA- DSV- HVD

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AD VS

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HIPOPLAZIA DE CORD STÎNG

Clinic - Radiologic

ECG ± axa QRS la dreapta, HAD, HVD Ecocardiografic

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TRANSPOZIIA DE VASE MARI (D-)

15-20% - primele 2 luni Anomalii ± Ao din VD

- AP din VS- DSV- stenoz pulmonar- PCA- Foramen ovale permeabil

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ADVS

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TRANSPOZIIA DE VASE MARI (D-)

Clinic Radiologic ± pensarea pediculului vascular 

- ³ou´ culcat pe diafragm ECG ± axa ± hiperdeviat la dreapta Ecocardiografia

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TRANSPOZIIA CORECTAT (L-)

Inversiune ventricular concomitent Anatomic ± AD ± valv mitral ± VS ± AP

- AS ± valv tricuspid ±VD ± Ao- artere coronare inversate- în asociere cu alte anomalii

Clinic Radiologic ± 2 arcuri convexe în stînga ECG ± Q în precordiale drepte, bloc AV

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TRUNCHI ARTERIAL COMUN

1-4% Anomalii ± o valv semilunar comun

- un vas de calibru mare- se ramific ± Ao,AP,A.coronare- DSV- foramen ovale persistent

Calibrul AP ± tetralogie Fallot- transpoziie de vase mari

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AD

VS

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TRUNCHI ARTERIAL COMUN

Clinic ± semne de shunt stg-dr - cianoz- pulsul foarte amplu

Radiologic ± cord ³în sabot´- cord ³ovoid´

ECG ± HAD, HBV Ecocardiografic

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MALADIA EBSTEIN

0,5-1% Anomalie de inserie a valvei tricuspide

Reducere a VD Asocieri ± DSA,stenoz-atrezie pulmonar Clinic Radiologic ± bombarea arcului drept ECG ± BRD, tulburri de ritm Ecocardiografic

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MALADIA EBSTEIN

AD VS

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DRENAJ VENOS PULMONAR ANORMAL

2% - total Anomalii ± vene pulmonare ± AD

(supracardiac - 55%,retrocardiac ± 25%,infracardiac ± 15%)

- DSA ± obligatoriu

Clinic Radiologic ± ³om de zpad´ ECG ± HAD marcat, BRD

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COARCTAIA AORTEI

7-10% - În sd. Turner 

Reducerea - lumenului cu 50%- diametrului cu 80% Preductal ± tip infantil Postductal ± tip adult

Circulaie colateral Asociere - PCA

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COARCTAIA AORTEI

Consecine ± HTA ± în amonte- hipoTA ± în aval- HVS

La sugar ± puls la femurale ± absent- gradient peste 20 mm Hg(absent dac se asociaz PCA)

La copilul mare ± complicaii

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COARCTAIA AORTEI

Clinic Radiologic

ECG ± iniial HVD, apoi HVS Ecocardiografie

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Complicaiile MCC

Insuficiena cardiac ± dreapt- stîng

Crizele hipoxice Infecioase ± endocardit- abces cerebral

Stroke ± HTA- Hipervîscozitate

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TRATAMENT

Medical ± Digoxin- Diuretice- PgE2- Indometacin

Chirurgical ± corector - paleativ

Intervenii prin cateterism

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CHIRURGIE PALEATIV

SHUNT ± AP-Ao - Blalock-Taussig -- Potts ±Aod-APs- Waterston ± Aoa-APd

Mrirea DSA ± Rashkind- Blalock-Hanlon

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CHIRURGIE - CORECIE

DSA, PCA ± intervenii prin cateterism Intervenii pe cord deschis

- TVM (D-) ± Jatene ± arterial- Mustard ± atrial -- Hipoplazia de cord stîng

- Norwood ± VD-Ao- Ao-AP