khong lo van dm phoi kem thong lien that
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KHONG LO VAN ONG MACH PHOI
KEM THONG LIEN THAT
(Pulmonary atresia with ventricular septal defect)PGS. TS. Pham Nguyen Vinh
Vien Tim TP. HCM
Con goi T chng Fallot the khong lo van MP (TOF with pulmonary atresia TOF/PA)
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Tan suat
2% BTBSNghien cu Baltimore Washington Infant Study (BWIS) :* 0,07 TOF/PA/1000 tre ra i con song
* 1,4% BTBS
Nam > n
* 20,3%/tat ca cac the TOFTL : Ferencz C et al. The Baltimore Washing Infant Study 1981 1989
Futura Publishing Co. 1993 : 33-62
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Dch te
Yeu to moi trng :* Me T : nguy c con x 10 lan TOF/PA
* Me T phu thuoc Insulin : nguy c con
TOF/PA x 20 lan
Yeu to gia nh : coYeu to di truyen : co* Di George syndrome
* 22q11 deletion syndrome (DGS/VCFS hoac
CATCH 22)
* Vater syndrome, Alagille syndrome
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Giai phau hoc : 4 kieu KLVMP-TLT
TL : Castaneda AR et al. Cardiac Surgery for the Neonate and Infant . WB Saunders 1994, p 218
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Giai phau hoc : Kieu thong thng gia
ong mach bang he (APCAs-Aortopulmonary collateral arteries) va MPTL : Castaneda AR et al. Cardiac Surgery for the Neonate and Infant . WB Saunders 1994, p 219
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Giai phau hoc : KLVMP-TLT vi than MP (PT) va ong mach phoi trai (LPA) teo nho
TL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William & Wilkins 5th ed 1995, p. 988
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
D : ductus
(ong ong mach
Ao : MC
RAA : tieu nh phai
LV : that trai
RV : that phai
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Giai phau hoc : ong mach bang he
xuat phat t MC xuongTL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William & Wilkins 5th ed 1995, p. 988
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
LCCA : ong mach canh chung traiRCCA : ong mach canh chung phai
RSA : ong mach di on phai LSA : ong mach di on trai
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Giai phau hoc : ti mau phoi do 3 nguon
Ong ong machM bang he chu phoi (APCAs)Mang ong mach phe quan hoac ong mach mang phoiKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Giai phau hoc cac ton thng khac
Thong lien that : quanh mang hoac vung pheu MC ci ngaH van MC : do dan vong van hoac VNTMNTCung MC ben phai : 26-50% trng hpNh phai : dan va ph ai ; nh trai : bnh thngTLN2 hoac lo bau duc thong thng : 50% trng hpThat phai : ph ai ; that trai : thng bnh thng, oi khi ph aing dan truyen : bnh thngKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Giai phau hoc : ton thng phoi hp
Ton tai TMC tren trai o vao xoang vanhNoi lien bat thng TMP ban phan hay toan phanHep hay khong lo van 3 laKenh nh thatHoan v MHai lan bat tng hp (hoan v M co sa cha)Tim ben phaiKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Bieu hien lam sang
Trieu chng t luc mi sinh Giam oxy mau va tm (cyanosis)Trieu chng giam oxy mau tuy thuoc OM ln nho hay tuan hoan bang he Rat hiem : trieu chng suy tim do tang tuan hoan phoi (OM ln hoac nhieu mach bang he)KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Trieu chng thc the
Tm nhe luc mi sinh, tang dan khi OM ongMach ngoai vi va HA thng bnh thngMom tim thng v tr bnh thng ; tim khong ln T1bnh thng ; T2 n ocThng khong co ATTThu ; co the co AT lien tuc do OM hay do mach bang he MC-MP (nghe ro sau lng)KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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ECG
Giong T chng Fallot That phai ph ai ; truc QRS lech phai b/n co nhieu mach bang he : ph ai hai that va ln nh trai ECG/khong lo van MP kem VLT nguyen ven : giam san that phaiKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Xquang ngc sau trc
Tim hnh guoc (coeur en sabot)Cung MC ben phai (26-50% trng hp)Mach mau phoi dang li (reticular appearance) do tuan hoan bang heKLVMP TLT kieu 1 : ong mach phoi trung tam dan ln khi co OM lnKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Xquang ngc sau trc
TL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William & Wilkins 5th ed 1995, p. 985
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Sieu am tim
Giup gi y chan oan (MC ci nga -> 5 benh)Khong giup mo ta ro MP hoac nguon mau nuoi phoi; do o thng can thong tim chan oan (diagnostic catheterization)Mat cat tren hom c : giup thay MP, OM va mach bang he, s hp lu MPKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Mat cat canh c truc ngang, ngang van MC:
van MC khong lo, than MC nhoTL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.55
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Mat cat tren hom c truc doc :
ong mach bang he xuat phat t MCTL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Mat cat tren hom c truc ngang :
khao sat Doppler xung dong chay
do phau thuat Blalock TaussigTL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Thong tim va chup mach
Ch can khi khong ro MPPhau thuat tam thi : co the khong can thong tim khi thay ro MPPhau thuat triet e : thong tim la ch nh bat buocKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Chup chon loc ong mach bang he
t MC xuongTL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William & Wilkins 5th ed 1995, p. 992
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Chan oan phan biet (1)
The tm sm, khong suy tim :
T chng Fallot the nangHoan v MKhong lo van 3 laTP 2 ng ra kem hep hay khong lo van MPTam that oc nhat kem hep hay khong lo van MPNLBT/TMP toan phan kem nghen TMPKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
kem nghen TMP
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Chan oan phan biet (2)
The t tm kem suy tim :
TLTCon OM lnKenh nh nhatTP 2 ng ra hoac Tam that oc nhat khong kem hep MPThan chung ong machNLBT/TMP toan phan khong kem nghen TMPKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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Tien trien t nhien
Tuy thuoc vao ti mau phoi OM bt -> tm nang (neu khong co mach bang he)-> t vong
Tuan hoan bang he +++ -> suy timKHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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ieu tr
KLVMP TLT kem hp lu MP va OM : can phau thuat khan (nguy c bt OM)Khi 1 hay ca 2 MP khong c cung cap mau bi OM : can chup mach Phau thuat tam thi : mau len phoi Phau thuat nhieu giai oan (multi-stage operation)Hp nhat mach phoi (unifocalization)KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT
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KLVMP-TLT : chup MC
trc phau thuat va sau phau thuatTL : Emmanouilides GC et al. Heart Diseases in Infants, Children and Adolescent, William & Wilkins 5th ed 1995, p. 985
KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT