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KHONG LO VAN ONG MACH CHU
(Thieu san that trai)
BS AO HU TRUNG
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NH NGHATam that trai khong phat trien: do cac c cau van MC, van
2 la va oan MC len khong phat trien.a dang v s thay oi a danh cac c
cau tren.1-2% cac benh TBS. 10-25% nguyen nhan t vong tre s sinh:
nam nhieu hn n. Co the gia nh. TBS tuy thuoc ong ong mach.
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GIAI PHAU BENH LYTon thng c ban:That trai: giam san nhieu dangLo
van MC Bt mang chan 1-2mm Co van nhng giam san hep 6mmVan hai la:
Vong van nho mang chan hoac co van nhng thieu san MC len: thanh
mong 2 3mm neu khong co lo van. 4- 5mm neu ch hep vanCung MC: giam
san 3-5mm co the ngat oanHep eo 80% benh nhan
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GIAI PHAU BENH LYTon thng phoi hp:Hau qua Ha lu/ Thng lu anh hng
Sinh Ly benh: mau thng lu: TMP NP TP- MP day dan ln OM danGiam tuan
hoan ha lu.Cac ton thng phoi hp khac: TL Nh ++ han che type OS F.O
TL That khi TT co kch thc tng oi Bat thng MV: Khong co lo Do Xoang
(Sinusoids). Hep MV
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Sinh ly benh
Trc sinh: Tuan hoan phoi t Mau TP -> OM ->M chu xuong
(xuoi) -> Quai MC ( ngc = mach mau co, MC len M vanh)Sau sinh: e
khang phoi giamLu lng TP giam anh hng en tuan hoan he thong Neu OM
m: thang bang e khang phoi/ He thong O2 -PH + CO2 tuan hoan phoi:
kch thch ong OM -> Tai hoa
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Hau qua:1/ Hoi chng mau tnh mach phoi:Neu TLN han che -> phu
phoiShunt tai phoi.Tang tai tam trng TP.
2/ Hoi chng giam tuan hoan he thong-> Tru mach, thieu mau
nuoi (ischemie) cac tang di: Than, Gan , Mac treoCac bo phan tren:
Nao bo, M vanh
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LAM SANG - CHAN OANBenh canh cap cu 24/48 gi khoang trong sau
sanh: suy ho hap tm Ha than nhiet Giam trng lc c.
Kham: s sinh tai tm noi bong thi gian Co mau lai -> Gan -
mach
Nghe: T2 oi vang Clic phun MP - Nhp nga phi ATTT dang phut
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X Quang: Tim ln RCT 0.6 0.65 Dan b phai (NP)B trai nho cao: Tim
hnh bong bau duc may TMP
ECG: Nhanh xoang tang ganh NP TP
Sieu am: Xac nh chan oan: C cau that trai Thieu san - TTT
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Hnh anh Xquang cua tre s sinh co hoi chng thieu san tim trai.
Bong tim ln: ph ai nh phai va that phai
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ien tam o mot tre s sinh co hoi chng thieu san TT trai.Dau hieu
quan trong: Truc lech phai, R cao V1 S sau V5-6 nh la dau hieu cua
ph TP
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SIEU AM TIM TRONG THIEU SAN THAT TRAI
Xac nh chan oan:Khao sat c cau that traiTng hp nh- that tha ong
machVan nh that traiThat trai va ng thoat that trai LVED
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SIEU AM TIM TRONG THIEU SAN THAT TRAI
Ton thng phoi hp:TLN TMP TP- MPOM TLT MV
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Mat cat 4 buong tmom van 2 la la mot mang x day (mui ten) (A).
Mat cat 4 buong t mom : Doppler mau dong h van 3 la nang tren benh
nhan khong lo van 2 la (B)
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Mat cat canh c truc doc-A. Thieu san that trai va MC , that P
dan. B.Vo san that trai, giam san MC va dan that P. ( LA nh trai;
PA: MP; Ao: MC; LV: that trai; RV: that phai )
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CHAN OAN PHAN BIETChan oan cac trng hp suy tim phu phoi:TTMHep
eo MCGian oan quai MCBat thng TM phoi co nghenHep van MC nang
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DIEN TIEN
T nhien rat nang T vong trong vai gi au tien.The thieu san nang
: OM rong- Shunt T->P- Cao ap phoi nang
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IEU TRNoi khoa: On nh tnh trang huyet ongPGE 1 Ong ong mach 0.03
0.1g/kg/phutHo hap ho tr ~ SatO2 (~PVR SVR)P CO2 toan mau.Nitrogen
gas
- IEU TRNgoai khoa:Chong ch nh: S sinh thieu thang
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IEU TRCh nhieu tr ho tr: 3 giai oan:Giai oan mot: NORWOOD: tai
tao quai MC.Giam mau TMP tang nh (RASHKIND)Kiem soat tuan hoan phoi
(BTM)Thi gian 3 ngay 3 tuan tuoi.Giai oan 2: Hemi
FONTANBidirectionelle GLENN(k PVR thap) Giam the tch mau TPChuan b
cho PT FONTANThi gian 1 thang 6 thangGiai oan 3: FONTAN: tach rieng
mau TM he thong/ TM phoi (ieu kien FONTAN)Thi gian # 2 tuoi ca so
nh (Fenedtration)
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S o phau thuat Glenn: Noi TMC tren vao MP phai (th trung gian)
TMC TMC mi(neo Aorta)MP
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Ghep tim: Bailey 1985V/ danh sach chV/ B/cthai ghepThuoc UCMDM
vanh
