THONG LIEN THAT (TLT)BS. ao Hu Trung

nh ngha: Khiem khuyet tai VLT -> thong thng TH HT - Phoi

Tan suat: 20% cac benh TBS

PHOI THAI HOC - GIAI PHAU BENH

Qua trnh hnh thanh VLT

V tr:- 85% phan mang ( lan tren, sau, di)- 15% con lai phan pheu, phan c, phan nhan

Conditions that may mimic large VSDSingle (common) ventricle without pulmonary stenosisDouble-outlet right ventricle without pulmonary stenosis.Note that defect is remote from both semilunar valvesPersistent truncus arteriosus

SINH LY BENH HOC

Yeu to:Kch thc lo thongTy le khang lcHe thong tuan hoan phoi / He thong

Phan loai huyet ong hoc:The benh (Typ)I ab - II ab - III - IV

THONG LIEN THATBANG PHAN LOAI HUYET ONG--------------------------------------------------------------------------------------The benhKch thcPAP/PsRP/RSQP/QSGr VD/AP---------------------------------------------------------------------------------------Ianho< 0,3< 0,31 - 1,50Ibnho< 0,3< 0,31,5 - 20---------------------------------------------------------------------------------------IIaln0,3 - 0,7< 0,5 > 2< 25IIbln0,7 - 1< 0,8> 2< 25---------------------------------------------------------------------------------------III ln> 1> 1< 1< 25---------------------------------------------------------------------------------------IVln +< 0,7< 0,5> 2> 25hep pheu MP

BENH CANH LAM SANG1. TLT lo nho - Benh Roger2. TLT trung bnh3. TLT vi tang ap MP4. TLT vi hep MP

TIEN TRIEN VA TIEN LNGVAI TRO CUA SIEU AM TRONG BENH TLTCH NH PHAU THUAT - NGUY C VA KET QUA

MUC CH TRONG SIEU AM THONG LIEN THAT (1)I.CHAN OANKch thcV tr khuyet tat:tren- Phan quanh mang (lan trc)sau- Vach lien that phan nhan- Vach lien that phan c be- Vach lien that phan pheuChieu hng luong thong

MUC CH TRONG SIEU AM THONG LIEN THAT (2)II. LNG GIA HUYET ONG HOC- that T, nh T, that P, MP- QP - QS- ALMP tam thu (PAPS)- ALMP trung bnh (PAPM)*Dong mau h 3 la, h MP* Dong mau luong thong* Hnh dang vach lien that- P TT/TP = 4V2 (van toc dong mau luong thong)- ALTP tam thu = HA tam thu - P (neu khong co hep ong mach phoi)

MUC CH TRONG SIEU AM THONG LIEN THATIII. PHAT HIEN BIEN CHNG- Hoi chng EISENMENGER- H van MC- Hep di van MC- Hep pheu MP- Viem noi tam mac...IV. THEO DOI HAU PHAU- Shunt ton tai- AL ong mach phoi

QUY TRNH SIEU AM (1) Mat cat canh c truc doc- Hnh anh lo thong (2D), hnh anh dong chay xoay manh mau xanh luc lap the chay qua lo thong (Doppler mau).- Kch thc cac buong tim. Chc nang tam thu cua tim.- Khao sat van MC: day, sa van, h van.

QUY TRNH SIEU AM (2)Mat cat canh c truc ngang - ngang van MC- V tr lo thong- o kch thc lo thong- Khao sat chieu luong thong- Khao sat Doppler lien tuc dong mau qua lo thonglien that- Ap lc MP tam trng va trung bnh - theo dong h van MP- Tm hep van MP phoi hp- o kch thc lo van MP, than MP

QUY TRNH SIEU AM (3)

Mat cat 4 buong t mom- Khao sat tong quat 4 buong tim- V tr lo thong, kch thc lo thong- Dong h 3 la, ap lc MP tam thu (dong h van 3 la)

QUY TRNH SIEU AM (4)

Mat cat 5 buong t momKhao sat ton thng van MC i kem

Mat cat di snKhao sat thong lien that, ac biet thong lien that phan c be

Mat cat canh c truc doc giup thay hnh anh lo TLT co dong mau xoay chay ngang. o chenh ap lc that trai/that phai la 78mmHg (A-B). Mat cat canh c truc ngang-ngang van MC : TLT di ai ong mach . TLT vung pheu MP (D)

Mat cat canh c truc ngang ngang van MC cho thay TLT phan mang

Parasternal long-axis scan with slight medial tlt of the transducer. A large membranous ventricular septal defect (arrows) is visualized just beneath the aortic valve (AV).A : anteriorLA : left atriumLV : left ventricleP : posteriorRV : right ventricleVS : ventricular septum

Parasternal short-axis scan at the level of the left ventricular outflow tract (LVO) shows a ventricular septal defect that is subtricuspid (arrows) and partially closed by septal leaflet of the tricuspid valve (arrowheads)

Subcostal four-chamber scan at aortic valve (AV level demonstrating an aneurysm of the membranous septum (small arrows). A large membranous ventricular septal defect is vusualised (large arrow). This patient had aa very small left to right shunt.

Mat cat 4 buong t mom : TLT buong nhan co kch thc ln (d=14mm) (A). Mat cat 5 buong t mom cho thay TLT phan mang co tui phnh vach mang (B).

Mat cat 4 buong canh c : TLT phan mang co kem thong noi that trai-nh phai. Dong mau xoay mau xanh luc lap the chay qua lo TLT hng ve pha nh phai (A). Pho Doppler dong mau ngang qua lo TLT co van toc = 4m/s (B)

CH NH PHAU THUATTuoiCh nh phau thuatDi 6 thangNeu khong chong che csuy tim6 - 24 thangNeu co cao ap MP hoac co trieu chng lam sangTren 2 tuoiNeu QP/QS > 2Chong ch nhTuyet oiKhi KLMP/KLMHT > 1/1Tng oiNeu KLMP/KLMHT > 0,75/1

Tam mach o can quang that phai. Tre 1 ngay tuoi cho thay xoang mach trong c tim (intramyocardial sinusoids) (mui ten nho). H van 3 la nang (mui ten am)Xquang phim thang (trc sau) cung trng hp o : Phe trng trong va b nh phai dan ln