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MCC CIANOGENE
Tetralogia Fallot (trilogie, pentalogie) Atrezia de tricuspid Hipoplazia de cord stng Trunchiul arterial comun Transpoziia de vase mari (D- sau L-) Maladia Ebstein Drenajul venos aberant
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 n sabot
AD
VS
TETRALOGIA FALLOT
ECG deviere axial dr, HVD Ecocardiografia
TRILOGIA FALLOT
5% Anomalii stenoz pulmonar (valvular) - DSA ostium secundum - HVD Clinic Radiologic ECG Ecocardiografic
PENTALOGIE FALLOT
Anomalii stenoza arterei pulmonare - DSA - DSV - dextropoziia aortei - HVD
ATREZIA TRICUSPIDIAN
2-5% Anomalii - absena-imperforaia valvei - HAD - AS mrire progresiv - DSA - VD hipoplazic - DSV - artera pulmonar - hipoplazie - PCA
ATREZIA TRICUSPIDIAN
Clinic Radiologic ECG axa la stnga, HAD, HVS Ecocardiografic
AD
VS
AD
VS
AD VS
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
MALADIA EBSTEIN
AD
VS
COARCTAIA AORTEI
7-10% n sd. Turner Reducerea - lumenului cu 50% - diametrului cu 80% Preductal tip infantil Postductal tip adult Circulaie colateral Asociere - PCA
COARCTAIA AORTEI
Consecine HTA n amonte - hipoTA n aval - HVS La sugar puls la femurale absent - gradient peste 20 mm Hg (absent dac se asociaz PCA) La copilul mare complicaii
COARCTAIA AORTEI
Clinic Radiologic ECG Ecocardiografie
Complicaiile MCC
Insuficiena cardiac dreapt - stng Crizele hipoxice Infecioase endocardit - abces cerebral Stroke HTA - Hipervscozitate
TRATAMENT
Medical Digoxin - Diuretice - PgE2 - Indometacin Chirurgical corector - paleativ Intervenii prin cateterism
CHIRURGIE PALEATIV
SHUNT AP-Ao - Blalock-Taussig - Potts Aod-APs - Waterston Aoa-APd Mrirea DSA Rashkind - Blalock-Hanlon
CHIRURGIE - CORECIE
DSA, PCA intervenii prin cateterism Intervenii pe cord deschis - TVM (D-) Jatene arterial - Mustard atrial - Hipoplazia de cord stng - Norwood VD-Ao - Ao-AP