asd,avsd ,vsd
TRANSCRIPT
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ASD, AVSD, VSD
Doç. Dr. Halil Aslan
Kanuni Sultan Süleyman EAH Perinatoloji Kliniği
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Embriyoloji
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Atrial Septum Defekti: Anatomi
• Sağ ve sol atriumlar arası komünikasyonlar
• İnsidans: 1/1500 canlı doğum
• Tüm KKH %8-9
• Sekundum (ASD Tip II)
• Primum ASD (parsiyel AVSD) (Tip I)
• Sinus venozus ASD
• Koroner sinus ASD
Yagel ve ark. 2006
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Sekundum ASD (Tip II ASD)
Foramen Ovale 20 hf 3mm Term 7-8 mm
KSS Perinatoloji
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Triküspit Atrezisi : ASD VSD zorunlu ilişki
Sekundum ASD (Tip II ASD)
KSS Perinatoloji
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• Atrioventriküler kanal defekti
• Endokardial yastık defekti
• İnsidans : 0.24-0.31/ 1000 canlı doğum
• Tüm KKH %4-5
Anatomik Spektrum
Klinik spektrum
Atrioventriküler Septal Defekt (AVSD)
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Neden Önemli ?
• Tr 21 kuvvetli birliktelik • Heterotaksi sendromları kuvvetli birliktelik • AD kalıtım formu • Genetik sendromlarla birliktelik • İzole olabilir
Atrioventriküler Septal Defekt (AVSD)
KSS Perinatoloji
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Atrioventriküler Septal Defekt (AVSD)
Carvalho JS ve ark. 2009
KSS Perinatoloji
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1. AVSD var mı, büyüklüğü ?
2. AV bileşke ventrikül ilişkisi
3. Her iki ventrikül boyutları
4. AV kapakta kaçak var mı, derecesi ?
5. Eşlik eden anomali ?
Atrioventriküler Septal Defekt (AVSD)
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Komplet AVSD
• AV kapak tek orifisli
• Atrial ve ventriküler septal defekt var
Parsiyel AVSD • AV kapak iki orifisli
• Sadece atrial defekt, sadece ventriküler defekt ya da atrial ve (küçük) ventriküler defekt
Atrioventriküler Septal Defekt (AVSD)
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Balanced AVSD • AV kapak orifisleri
ventriküllere eşit olarak açılır
• Ventriküller eşit büyüklüktedir
Unbalanced AVSD
• AV kapak orifisleri daha büyük oranda bir ventriküle açılır
• Bir ventrikül diğerinden küçüktür
Atrioventriküler Septal Defekt (AVSD)
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• AVSD, komplet, balanced (%75’i Tr 21)
• AVSD, parsiyel, balanced (%25’i Tr 21)
• AVSD, komplet, unbalanced (sıklıkla heterotaksi)
• AVSD + konotrunkal anomali+ azigos devamlılığı : heterotaksi* (Tr 21 yok)
• AVSD, komplet, balanced + Fallot* (Tr21 tamanına yakın)
Atrioventriküler Septal Defekt (AVSD)
Chaoui ve ark. 1999, Paladini ve ark. 2000
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AVSD +
AVSD + DORV
KSS Perinatoloji
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AVSD +
AVSD sol atrial izomerizm AVSD sag atrial izomerizm
AVSD + sağ arkus aorta
KSS Perinatoloji
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Yalancı AVSD
Persistan sol vena kava süperior dilate koroner sinüs, Co A
KSS Perinatoloji
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• Postnatal dönemde en sık KKH (%35)
• Fetal dönemde (%10)
– 4 odacık kesitini çok değiştirmez
– Basınç gradyenti yoktur
– Genellikle küçüktür
Ventriküler Septal Defekt (VSD)
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Ventriküler Septal Defekt (VSD)
VSD tipleri:
– Perimembranöz VSD • İnlet
• Outlet
– Müsküler VSD • İnlet
• Trabeküler
• Outlet
– Doubly comitted VSD
– Malalignement VSD
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Ventriküler Septal Defekt (VSD)
Perimembranöz > Müsküler > İnlet > Outlet Abuhamad ve ark. 2010
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Ventriküler Septal Defekt (VSD)
• VSD var mı yok mu?
– Evet: • Eşlik eden anomali
var mı ?
• Karyotip yapalım mı?
• Doğal seyri nedir?
• Prognozu nasıl?
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Ventriküler Septal Defekt (VSD)
Her zaman doğru insonasyon açısı ile çalışılmalı !
Drop out Yanlış hizalama
Koroner sinüs
Ao kapak annulusu
KSS Perinatoloji
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Perimembranöz İnlet VSD
KSS Perinatoloji
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Müsküler VSD
KSS Perinatoloji
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Malalignement VSD
VSD li Pulmoner atrezi MAPCA
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Subpulmonik- Doubly committed VSD
KSS Perinatoloji
Yagel ve ark.
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Doğal seyir
Paladini ve ark. 2000
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1. Sinus venozus ve koroner sinüs tipi ASD pratikte prenatal tanı yok
2. Sekundum ASD (Tip II) prenatal tanı yerine ‘’şüphesi’’
ASD, AVSD, VSD Sonuç (1)
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AVSD Tipi
Kardiak prognoz
Genel Prognoz
AVSD, komplet , balanced + Fallot, CoA
İYİ
Tr21
AVSD , parsiyel , balanced
ÇOK İYİ
Tr21
AVSD, komplet, unbalanced +DORV, KKH Heterotaksi
ÇOK KÖTÜ
ÇOK KÖTÜ
ASD, AVSD, VSD Sonuç (2)
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• VSD intrauterin dönemde yeri ve büyüklüğüne göre spontan kapanabilir
– Malalignement VSD kapanmaz
– Küçük outlet perimembranöz VSD yarısı intrauterin dönemde kapanır
• VSD tipi anöploidi riskini belirler
– Malalignement VSD %56 Tr18
– İnlet VSD %50 Tr 21
– Outlet VSD %88 Normal karyotip
ASD, AVSD, VSD Sonuç (3)
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