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)

Tan suat

2% BTBSNghien cu Baltimore Washington Infant Study (BWIS) :

* 0,07 TOF/PA/1000 tre ra i con song

* 1,4% BTBS
* 20,3%/tat ca cac the TOF

Nam > n

TL : 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

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

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

Giai phau hoc : Kieu thong thng gia
ong mach bang he (APCAs-Aortopulmonary collateral arteries) va MP

TL : 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

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

Giai phau hoc : ong mach bang he
xuat phat t MC xuong

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

LCCA : ong mach canh chung traiRCCA : ong mach canh chung phai

RSA : ong mach di on phai LSA : ong mach di on trai

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 phoi

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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 thng

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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 phai

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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

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

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 phai

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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 ln

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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

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 MP

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Mat cat canh c truc ngang, ngang van MC:
van MC khong lo, than MC nho

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.55

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Mat cat tren hom c truc doc :
ong mach bang he xuat phat t MC

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Mat cat tren hom c truc ngang :
khao sat Doppler xung dong chay
do phau thuat Blalock Taussig

TL : Atlas Sieu am tim 2D va Doppler mau. NXB Y hoc 2000, p.56

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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 buoc

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

Chup chon loc ong mach bang he
t MC xuong

TL : 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

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 TMP

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

kem nghen TMP

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 TMP

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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 tim

KHONG LO VAN ONG MACH PHOI KEM THONG LIEN THAT

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

KLVMP-TLT : chup MC
trc phau thuat va sau phau thuat

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