malformatii cardiace congenitale
TRANSCRIPT
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MALADII CONGENITALE CARDIACE
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MCC CIANOGENE
• Tetralogia Fallot (trilogie, pentalogie)
• Atrezia de tricuspidă
• Hipoplazia de cord stîng
• Trunchiul arterial comun
• Transpoziţia 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 pulmonară (infundibul) - DSV - dextropoziţia aortei - HVD
• Presiuni sistolice egale – VS, VD, Ao
• Clinic
• Radiologic – cord în “sabot”
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ADVS
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TETRALOGIA FALLOT
• ECG – deviere axială dr, HVD
• Ecocardiografia
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TRILOGIA FALLOT
• 5%
• Anomalii – stenoză pulmonară (valvulară) - DSA – ostium secundum - HVD
• Clinic
• Radiologic
• ECG
• Ecocardiografic
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PENTALOGIE FALLOT
• Anomalii – stenoza arterei pulmonare - DSA - DSV - dextropoziţia aortei - HVD
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ATREZIA TRICUSPIDIANĂ
• 2-5%• Anomalii - absenţa-imperforaţia valvei
- HAD - AS – mărire progresivă - DSA - VD hipoplazic - DSV - artera pulmonară - hipoplazie - PCA
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ATREZIA TRICUSPIDIANĂ
• Clinic
• 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 – hipoplazică- valva mitrală – 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
• Ecocardiografic
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TRANSPOZIŢIA DE VASE MARI (D-)
• 15-20% - primele 2 luni
• Anomalii – Ao din VD - AP din VS - DSV - stenoză pulmonară - PCA - Foramen ovale permeabil
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ADVS
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TRANSPOZIŢIA DE VASE MARI (D-)
• Clinic
• Radiologic – pensarea pediculului vascular - “ou” culcat pe diafragm
• ECG – axa – hiperdeviată la dreapta
• Ecocardiografia
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TRANSPOZIŢIA CORECTATĂ (L-)
• Inversiune ventriculară concomitentă
• Anatomic – AD – valvă mitrală – VS – AP - AS – valvă tricuspidă –VD –
Ao - artere coronare inversate - în asociere cu alte anomalii
• Clinic
• Radiologic – 2 arcuri convexe
• ECG – Q în precordiale drepte, bloc AV
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TRUNCHI ARTERIAL COMUN
• 1-4%
• Anomalii – o valvă semilunară comună - un vas de calibru mare - se ramifică – Ao,AP,
Acoronare - DSV - foramen ovale
persistent
• Calibrul AP – tetralogie Fallot - transpoziţie de vase mari
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ADVS
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TRUNCHI ARTERIAL COMUN
• Clinic – semne de shunt stg-dr - cianoză - pulsul foarte amplu
• Radiologic – cord “în sabot” - cord “ovoid”
• ECG
• Ecocardiografic
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MALADIA EBSTEIN
• 0,5-1%• Anomalie de inserţie a valvei tricuspide• Reducere a VD• Asocieri – DSA,stenoză-atrezie pulmonară• Clinic• Radiologic – bombarea arcului drept• ECG – BRD, tulburări de ritm• Ecocardiografic
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MALADIA EBSTEIN
AD VS
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DRENAJ VENOS PULMONAR ANORMAL
• 2% - totală • Anomalii – vene pulmonare – AD
(supracardiac - 55%, retrocardiac – 25%, infracardiac – 15%)
- DSA – obligatoriu• Clinic• Radiologic – “om de zăpad㔕 ECG – HAD marcată, BRD
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COARCTAŢIA AORTEI
• 7-10% - • În sd. Turner• Reducerea - lumenului cu 50%
- diametrului cu 80%• Preductală – tip infantil• Postductală – tip adult• Circulaţie colaterală• Asociere - PCA
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COARCTAŢIA AORTEI
• Consecinţe – HTA – în amonte - hipoTA – în aval - HVS
• La sugar – puls la femurale – absent - gradient peste 20 mm Hg (absent dacă se asociază
PCA)
• La copilul mare – complicaţii
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COARCTAŢIA AORTEI
• Clinic
• Radiologic
• ECG
• Ecocardiografie
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Complicaţiile MCC
• Insuficienţa cardiacă – dreaptă - stîngă
• Crizele hipoxice
• Infecţioase – endocardită - abces cerebral
• Stroke – HTA - Hipervîscozitate
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TRATAMENT
• Medical – Digoxin - Diuretice - PgE2 - Indometacin
• Chirurgical – corector - paleativ
• Intervenţii prin cateterism
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CHIRURGIE PALEATIVĂ
• SHUNT – AP-Ao - Blalock-Taussig -- Potts –Aod-APs- Waterston – Aoa-APd
• Mărirea DSA – Rashkind- Blalock-Hanlon
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CHIRURGIE - CORECŢIE
• DSA, PCA – intervenţii prin cateterism
• Intervenţii pe cord deschis - TVM (D-) – Jatene – arterial
- Mustard – atrial -
- Hipoplazia de cord stîng- Norwood – VD-Ao
- Ao-AP