NOI LIEN BAT THNG TNH MACH PHOI(Anomaly of pulmonary venous connection)PGS.TS PHAM NGUYEN VINHVIEN TIM TP HO CH MINH

Noi lien bat thng TMP ban phanNoi lien bat thng TMP toan phanNoi lien bat thng TMP toan phan a chiem 2% tong so benh tim bam sinhNoi 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

Cac the giai phau benh NLBT TMP/ ban phanNLBT TMP vao TMC tren. The nay thng co TLN kieu xoang tnh machNLBT TMP vao TMC di. Phoi phai thng c dan mau bang 1 TMP bat thng.Thng co bat thng nhu mo phoi phai. Vach lien nh thng nguyen ven

Cac the giai phau benh NLBT TMP/ ban phanC. NLBT TMP vao TM vo danh qua TM thang ngD. NLBT TMP vao xoang vanh (CS)

Lo TMP phai gan thong lien nh hn TMP trai, do o mau t TMP phai o vao nh phaiPhan 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 phaiSVC: TMC trenRUPV: TMP tren phaiRLPV: TMP di phaiLUPV: TMP tren traiLLPV: TMP dii trai

Cac the giai phau benh NLBT TMP/ toan phanNLBT TMP / toan phan vao TM vo danhNLBT TMP / toan phan vao xoang vanh

Cac the giai phau benh NLBT TMP/toan phanC. NLBT TMP/ toan phan vao nh phaiD. NLBT TMP/ toan phan vao TM caPV: TM caSMV: TM mac treo trenSV: TM lachRH: TM gan phai

NLBT TMP toan phan the di tim: de nghen macha. 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 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 splanchniqueMang mach mau phoi khong o vao tim ma o vao mang splanchniqueMang 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 vitellineBon TMP noi vi nh trai qua trung gian TMP chung bon TMP se gan thang vao nh trai

Phat trien bnh thng he thong TMPA. 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 vitellineB. TMP chung bat nguon t nh trai, thong thng vi mang splanchnic. Do o mau TMP thong thng vi nh traiC. S noi lien gia mang mach mau phoi vi TM cardinal va TM umbilico- vitelline khong can na nen bien matD. S phat trien cua nh trai, giup TMP chung hoa nhap vao. Do o tng TMP gan vao nh trai LV: that traiSA: vung xoang nhV: that

Can ban phoi thai hoc cua noi lien bat thng TMPTrai: 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 NLBT TMP/ hoan toanDi phai: Bt lo nhanh chnh cua TMP chung gay ra NLBT TMP/ ban phan

Giai phau benh (1)Noi lien bat thng TMP ban phan:

TMP trai se o bat thng vao TMC tren trai vaxoang vanh, TMP phai se o vao TMC tren phai, TMC di va nh phaiTan suat bat thng cua TMP phai gap oi TMP traiCac bat thng t gap: TMP phai o vao xoang vanh hay tnh mach azygos

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 azygosNoi lien TMP vao tim (25%): 4/5 o vao xoang vanh, 1/5 o vao nh phaiNoi lien TMP di tim (20%): o vao tnh mach ca hoac 1 trong cac nhanh cua tnh mach caNoi lien TMP hon hp (5%): th du 3 TMP o vao xoang vanh, 1 TMP con lai o vao TMC tren traiac 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

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 nhBenh thng nang, 75 90% khong song en 1 tuoi

Trieu chng c nang/ NLBT TMP toan phan:

Kho th, ngay cang nangKho nuoi anNhiem trung phoi thng xuyenTm tng luc hay tm lien tuc, thng la tm nhe. Mc o tm tuy thuoc mc o tac nghen TMPT vong thng xay ra trong benh canh phu phoi va suy tim phai

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 cheCo 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

ECGECG 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 phai khong hoan toan

ECGECG 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 song S sau V6

ECGECG 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

ECGECG 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

XquangNoi lien TMP toan phan vao xoang vanh, co hnh anh Xquang giong thong lien nh lo th 2Noi lien bat thng TMP toan phan vao TMC tren phai: bong tim co dang so 8 hoacngi tuyetKhi 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

Sieu am tim

1. Muc tieu chan oanXac nh situsnh danh cac buong timKhao sat s tng hp nh that, van nh that va s tng hp that ai ong machXac nh v tr cac TMPKhao sat xoang vanh, than vo danh (tronc innomine) va TMC tren trai (neu co)Khao sat o thong cua TMP va ap lc MPKhao sat ton thng phoi hp: thong lien nh, thong lien that...

Sieu am tim2. Cac mat cat hu chTMP 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 caoTMC tren trai (hay TM thang ng veine verticale)

Sieu am timMat 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 tren phai vave nh phai (B)

Sieu am timMat 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 kieu xoang TM (B)

Sieu am timMat 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 mau t ong thu thap o vao TMC tren

PHU LUC VE PHOI THAI HOC VA CAC DANG GIAI PHAU HOC BENH NLBT TMPPhan loai theo phoi thai cac bat thng TMPI. Bt lo (Atresia) TMP chung (sm) trong khi con thong thng TMP vi TM he thongA. NLBT TMP/ ban phanB. NLBT TMP/ toan phan:* co tac nghen TMP* khong tac nghen TMPII. Bt lo TMP chung (cham) sau khi khong con thong thng TMP vi TM he thongA. Bt lo TMP chungIII. Hep TMP chung (common pulmonary vein stenosis)A. Tim 3 buong nhIV. Hoa nhap bat thng TMP chung vao nh traiA. Hep tng TMP n ocB. So lng TMP bat thng

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 matB. Bt lo TMP chung vao luc nay, se khong con ng dan mau TMPC. Hau qua la benh bt lo TMP chung, 4 TMP o vao 1 tui cung

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 matE. Neu hep xay ra, mau TMP vao nh trai qua cho hepF. TMP chung dan ln, tao thanh buong nh th 3

Cac the tim 3 buong nhA, B, C: buong nh phu thong nh traiA. The co ien, buong nh phu nhan cac TMPB. The co thong thng gia buong nh phu vi nh phaiC. Tim 3 buong nh kem NLBT TMP

Cac the tim 3 buong nhD, E: buong nh phu khong thong vi nh traiD. Buong nh phu thong vi nh traiE. Buong nh phu thong vi TMP chung, thong vi TM caMau se ti nh trai nh TLN

Cac the tim 3 buong nhF, G, H: Tim 3 buong nh ban phanF. Buong nh phu nhan mau TMP phaiG. NLBT TMP trai vao TM vo danhH. Buong nh phu nhan mau TMP phai thong vi nh phai