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NOI LIEN BAT THNG TNH

MACH PHOI
(Anomaly of pulmonary venous
connection)
PGS.TS PHAM NGUYEN VINH
VIEN TIM TP HO CH MINH

Noi lien bat thng TMP ban phan


Noi lien bat thng TMP toan phan
Noi lien bat thng TMP toan phan a
chiem 2% tong so benh tim bam sinh
Noi lien bat thng TMP ban phan:
0,6/1000 s sinh (khao sat giai phau t
thi)
Tim 3 buong nh (Cor triatrium)
Hep n oc TMP
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Cac the giai phau benh NLBT


TMP/ ban phan

A. NLBT TMP vao TMC tren. The nay thng co TLN kieu
xoang tnh mach
B. NLBT TMP vao TMC di. Phoi phai thng c dan mau 3
bang 1 TMP bat thng.Thng co bat thng nhu mo

Cac the giai phau benh NLBT


TMP/ ban phan

C. NLBT TMP vao TM vo danh qua TM thang ng


D. NLBT TMP vao xoang vanh (CS)

A. Lo TMP phai gan thong lien nh hn TMP trai, do o mau t


TMP phai o vao nh phai
B. Phan ln mau t TMP phai o vao nh phai, rat t o vao
nh trai. Mot phan mau TMC tren o vao nh trai. Phan ln
mau TMP trai o vao that trai, phan nho vao nh phai
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SVC: TMC tren
RUPV: TMP tren phai RLPV: TMP di phai

Cac the giai phau benh NLBT


TMP/ toan phan

A. NLBT TMP / toan phan vao TM vo danh


B. NLBT TMP / toan phan vao xoang vanh

Cac the giai phau benh NLBT


TMP/toan phan

C. NLBT TMP/ toan phan vao nh phai


D. NLBT TMP/ toan phan vao TM ca
PV: TM ca

SMV: TM mac treo tren

SV: TM lach

RH:

NLBT TMP toan phan the di tim:


de nghen mach

a. Illustration showing the confluence of pulmonary veins giving rise to a vascular


channel that enters the abdominal cavity rough the diaphragmatic hiatus and
terminates in the portal vein (RPV, LPV : right and left pulmonary veins,
respectively) b. Illustration showing the anomalous left vertical vein compressed in
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a hemodynamic vise between the pulmonary trunk (PT) and left bronchus

Phoi thai hoc he thong TMP


thi ky phoi thai, phoi, thanh quan va kh phe
quan bat nguon t ruot nguyen thuy (intestin
primitif)
Phoi c ti mau bang mang mach mau ruot:
plexus splanchnique
Mang mach mau phoi khong o vao tim ma o
vao mang splanchnique
Mang mach mau phoi se noi vi TMP nguyen
thuy (veine pulmonaire primitive)
Mang mach mau phoi se khong con noi lien vi
tnh mach cardinales va tnh mach ombilico vitelline
Bon TMP noi vi nh trai qua trung gian TMP chung
bon TMP se gan thang vao nh trai
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Phat trien bnh thng he


thong TMP

A. Mang splanchnic dan mau phoi tao thanh mang mach mau
phoi. Mang nay khong thong thng vi tim ma noi vi
TM cardinal va TM umbilico vitelline
B. TMP chung bat nguon t nh trai, thong thng vi mang
splanchnic. Do o mau TMP thong thng vi nh trai
C. S noi lien gia mang mach mau phoi vi TM cardinal va
TM umbilico- vitelline khong can na nen bien mat
D. S phat trien cua nh trai, giup TMP chung hoa nhap vao.
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Do o tng TMP gan vao nh trai

Can ban phoi thai hoc cua noi


lien bat thng TMP

Trai: TMP chung a noi vi nh trai nhng s noi lien TM nguyen


thuy con ton tai (TMP noi vi TM cardinal va TM umbilico
vitelline)
Tren phai: Neu TMP chung b bt lo vao luc nay, mau t TMP
khong con chay trc tiep vao nh trai. Mot hoac nhieu
mach noi lien TM nguyen thuy ton tai va ln hn. o la 11

Giai phau benh (1)


1. Noi lien bat thng TMP ban phan:
-

TMP trai se o bat thng vao TMC


tren trai va
xoang vanh, TMP phai se o vao TMC
tren phai, TMC di va nh phai
Tan suat bat thng cua TMP phai
gap oi TMP trai
Cac bat thng t gap: TMP phai o
vao xoang vanh hay tnh mach azygos
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Giai phau benh (2)


2. Noi lien bat thng TMP toan phan:
- Noi lien TMP tren tim (50% trng hp); 4/5 o
vao TMC tren trai, 1/5 o vao TMC phai hay tnh
mach azygos
- Noi lien TMP vao tim (25%): 4/5 o vao xoang
vanh, 1/5 o vao nh phai
- Noi lien TMP di tim (20%): o vao tnh mach
ca hoac 1 trong cac nhanh cua tnh mach ca
- Noi lien TMP hon hp (5%): th du 3 TMP o vao
xoang vanh, 1 TMP con lai o vao TMC tren trai
- ac iem chung cua cac loai nay la cac TMP gom
vao 1 ong thu thap (tube collecteur)
- Nghen mau hoi lu mau TMP: tang ap MP
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Lam sang
(Noi lien bat thng TMP toan
phan)
Bieu hien lam sang thay oi theo:
- Lo trnh phai trai qua cua mau TMP
e ti nh phai
- Co hay khong co tac nghen tren
lo trnh o
- Mc o thong lien nh
Benh thng nang, 75 90% khong
song en 1 tuoi
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1. Trieu chng c nang/ NLBT TMP toan phan:

Kho th, ngay cang nang


Kho nuoi an
Nhiem trung phoi thng xuyen
Tm tng luc hay tm lien tuc, thng la
tm nhe. Mc o tm tuy thuoc mc o
tac nghen TMP
T vong thng xay ra trong benh canh
phu phoi va suy tim phai
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2. Trieu chng thc the/ NLBT TMP toan phan


Vung trc ngc thng ap manh, co
dau ln that phai
T2 s thay c
Dau hieu thc the giong thong lien nh lo
th 2 khong han che
Co the nghe thay am thoi lien tuc b
trai hay b phai xng c do mau TMP lu
thong qua ong thu thap o vao TMC tren
phai
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ECG

ECG b/n nam


49 tuoi, b
NLBTHT/ TMP
the tren tim:
co TLN ln
lo th 2,
shunt trai
phai ty le
2,5 en 1. AL
MP50/20
mmHg.
Khoang PR
dai, song P
rong kem
bloc nhanh
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phai khong

ECG

ECG em gai 13
tuoi co NLBTHT/
TMP the tren
tim. AL MP gan
bang ap lc
ong mach he
thong. Song P
nhon D2, aVF.
Truc QRS lech
phai. Day that
phai bieu hien
bang R n pha
V1, song T
ao V1-4 va 18
song S sau

ECG

ECG b/n nam


ln tuoi co
NLBTHT/ TMP
vao TMC tren
phai. Truc QRS
lech phai.
Song P cao
D2, 3, aVF. Day
that phai
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ECG

ECG be trai 9
thang tuoi co
NLBTHT/ TMP
vao nh phai.
Truc QRS +
900. Day that
phai bieu
hien bang R
cao, n pha
V1 va S sau
V6
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Xquang
Noi lien TMP toan phan vao xoang vanh, co
hnh anh Xquang giong thong lien nh lo th
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Noi lien bat thng TMP toan phan vao
TMC tren phai: bong tim co dang so 8
hoacngi tuyet
Khi noi lien bat thng TMP toan phan co
kem tac nghen TMP se co thay oi ro
Xquang hai phoi: dan n TMP va mach bach
huyet, phu phoi mo ke va phe nang, oi
khi thay Kerley B. Hnh anh nay tng phan
vi bong tim khong ln
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Sieu am tim
1.

Muc tieu chan oan


Xac nh situs
nh danh cac buong tim
Khao sat s tng hp nh that, van nh that
va s tng hp that ai ong mach
Xac nh v tr cac TMP
Khao sat xoang vanh, than vo danh (tronc
innomine) va TMC tren trai (neu co)
Khao sat o thong cua TMP va ap lc MP
Khao sat ton thng phoi hp: thong lien
nh, thong lien that...
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Sieu am tim
2. Cac mat cat hu ch
TMP v tr bnh thng: co the thay c
bang cac mat cat:
- 4 buong t mom
- 4 buong di sn
- Vong quanh tren hom c (suprasternal
coronal plane)
- Canh c truc ngang cao
TMC tren trai (hay TM thang ng veine
verticale)
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Sieu am tim

Mat cat tren hom c: Hnh anh Doppler mau cua noi lien
hoan toan TMP the tren tim vi hnh anh ong thu thap dan
mau t cac TMP ve TM len ben trai (A).Hnh anh noi lien
hoan toan TMP the tren tim vi hnh anh ong thu thap dan
mau t cac TMP ve TM len ben trai, sau o o vao TMC
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tren phai vave nh phai (B)

Sieu am tim

Mat cat 4 buong t mom: hnh anh thong lien nh lo th


phat vi hnh anh noi lien bat thng TMP vao ong thu thap
va o vao nh phai (A). Mat cat di sn 2 buong: hnh anh
ong thu thap o vao TMC tren, va hnh anh thong lien nh
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kieu xoang TM (B)

Sieu am tim

Mat cat di
sn cho thay hnh
anh noi lien bat
thng TMP the
tren tim vi cac
TMP o vao ong
thu thap, ong nay
o vao TMC tren
(C D E). Doppler
ghi nhan pho lien
tuc cua dong 26
mau t ong thu

PHU LUC VE PHOI THAI HOC VA CAC DANG


GIAI PHAU HOC BENH NLBT TMP
Phan loai theo phoi thai cac bat thng TMP
I. Bt lo (Atresia) TMP chung (sm) trong khi con thong thng TMP
vi TM he thong
A. NLBT TMP/ ban phan
B. NLBT TMP/ toan phan:
* co tac nghen TMP
* khong tac nghen TMP
II. Bt lo TMP chung (cham) sau khi khong con thong thng TMP
vi TM he thong
A. Bt lo TMP chung
III. Hep TMP chung (common pulmonary vein stenosis)
A. Tim 3 buong nh
IV. Hoa nhap bat thng TMP chung vao nh trai
A. Hep tng TMP n oc
B. So lng TMP bat thng

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Can ban phoi thai cua bt lo (Atresia) TMP chung (A, B


va C) va
tim 3 buong nh (D, E va F)
A. TMP chung a noi nh trai. Mach noi TM nguyen thuy
bien mat

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B. Bt lo TMP chung vao luc nay, se khong con ng

Can ban phoi thai cua bt lo (Atresia) TMP chung


(A, B va C) va
tim 3 buong nh (D, E va F)
D. TMP chung a noi nh trai. Mach noi TM nguyen
thuy bien mat

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E. Neu hep xay ra, mau TMP vao nh trai qua cho hep

Cac the tim 3 buong nh


A, B, C: buong nh phu thong nh trai
A. The co ien, buong nh phu nhan cac TMP
B. The co thong thng gia buong nh phu vi nh
phai
C. Tim 3 buong nh kem NLBT TMP

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Cac the tim 3 buong nh


D, E: buong nh phu khong thong vi nh trai
D. Buong nh phu thong vi nh trai
E. Buong nh phu thong vi TMP chung, thong vi TM
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ca

Cac the tim 3 buong nh


F, G, H: Tim 3 buong nh ban phan
F. Buong nh phu nhan mau TMP phai
G. NLBT TMP trai vao TM vo danh
H. Buong nh phu nhan mau TMP phai thong vi nh32
phai