KHONG LO VAN ONG MACH CHU

(Thieu san that trai)

BS AO HU TRUNG

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.

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

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

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

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

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

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

Hnh anh Xquang cua tre s sinh co hoi chng thieu san tim trai. Bong tim ln: ph ai nh phai va that phai

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

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

SIEU AM TIM TRONG THIEU SAN THAT TRAI

Ton thng phoi hp:TLN TMP TP- MPOM TLT MV

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)

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 )

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

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

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

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)

S o phau thuat Glenn: Noi TMC tren vao MP phai (th trung gian) TMC TMC mi(neo Aorta)MP

Ghep tim: Bailey 1985V/ danh sach chV/ B/cthai ghepThuoc UCMDM vanh