CHAN OAN TANG AP PHOI TIEN PHAT(Diagnostic approach to primary pulmonary hypertension)PGS.TS PHAM NGUYEN VINHVIEN TIM TP HO CH MINH

NH NGHATang ap phoi tien phat (TAPTP): ALMP tam thu > 35mmHg; ALMP trung bnh (mPAP) > 25mmHg luc ngh, > 30mmHg/ gang sc.Tuy nhien*:- ALMP tthu > 40mmHg/ 6% ngi bnh thng > 50 tuoi- ALMP tthu > 40mmHg/ 5% ngi co BMI > 30kg/m2

TL: Mc Quillan BM et al. Circulation 2001; 104: 2797 - 2802CHAN OAN TANG AP PHOI TIEN PHAT

TANG AP PHOI TIEN PHAT (TAMP tien phat)Benh tien trien nhanh, t vong sm.Tan suat mi mac (incidence): 1-2/ 1 000 000/ nam*N > nam (63%)

TL: * Rich S et al. Ann Intern Med 1987; 107: 216 223* Abenhaim L et al. N Engl J Med 1996; 335: 609 - 616CHAN OAN TANG AP PHOI TIEN PHAT

CAC TON THNG MACH MAU TRONG TAMP TIEN PHATCHAN OAN TANG AP PHOI TIEN PHATTL: Rubin LJ. The Heart ed by Fuster, Alexander, ORourkie. McGraw Hill 2004, 11th ed p.1580

PHAN LOAI TANG AP PHOI/ WHO 1998 (1)1. Tang ap ong mach phoi (TAMP)- TAPTP* ca ngau nhien* gia nh- Co lien quan en:* benh chat keo* BTBS: shunt* tang ap tnh mach ca* nhiem HIV* thuoc/ oc to+ chat chong beo (aminorex, fenfluramine dexfenfluramine)+ chat khac. Chac chan: dau hat cai. Rat co the: amphetamines, L-tryptophan. Co the: meta amphetamine, cocaine, thuoc hoa tr.* tang ap phoi keo dai t s sinh* nguyen nhan khac (td: benh tuyet giap, benh Gaucher, benh hemoglobine, cat la lach)TL: Rich S(ed). Primary pulmonary Hypertension WHO 1998.CHAN OAN TANG AP PHOI TIEN PHAT

PHAN LOAI TANG AP PHOI/ WHO 1998 (2)2. Tang ap tnh mach phoi- Benh nh trai hay that trai- Benh van ben trai tim.- Tac TMP do chen ep (viem trung that si hoa, hach, bu)- Benh tac TMP- Nguyen nhan khac

TL: Rich S(ed). Primary pulmonary Hypertension WHO 1998.

CHAN OAN TANG AP PHOI TIEN PHAT

PHAN LOAI TANG AP PHOI/ WHO 1998 (3)3. Tang ap phoi ket hp vi roi loan bo may ho hap va/hoac giam oxy mau.- Benh phoi man tac nghen- Benh mo ke phoi- Roi loan th luc ngu- Roi loan giam thong kh phe nang.- lau tren vung cao- Benh phoi s sinh- Loan san mao mach phe nang- Nguyen nhan khac

TL: Rich S(ed). Primary pulmonary Hypertension WHO 1998CHAN OAN TANG AP PHOI TIEN PHAT

PHAN LOAI TANG AP PHOI/ WHO 1998 (4)4. Tang ap phoi do benh huyet khoi man va/hoac benh ly thuyen tac- Huyet khoi thuyen tac phan gan MP- Tac phan xa MP* Thuyen tac phoi (huyet khoi, bu, KST, vat la)* Huyet khoi tai cho* Benh hong cau hnh liem5. Tang ap phoi ket hp vi nguyen nhan khac- Viem:* Schistosomiasis* Sarcoidosis* nguyen nhan khac- a bu mau mao mach phoiTL: Rich S(ed). Primary pulmonary Hypertension WHO 1998CHAN OAN TANG AP PHOI TIEN PHAT

BIEU HIEN LAM SANGTuoi khi phat: tre (TB = 36 + 15 tuoi)Trieu chng khong ac hieuTrieu chng au tien -> chan oan: 2.03 + 4.9 namTCCN thng gap nhat: kho th (60%)

TL: Rich S et al. Ann Int Med 1987; 107: 216 - 223CHAN OAN TANG AP PHOI TIEN PHAT

TAN SUAT TRIEU CHNG AU TIEN VA KHI A KHAM CHUYEN KHOATrieu chng au tien a kham chuyen khoaKho th (khi gang sc)60%98%Kiet sc20%73%au ngc7%47%Gan ngat12%41%Phu cang chan3%37%Hoi hop5%33%Hoi chng Raynaud10%

TL: Rich S et al. Ann Int Med 1987; 107: 216 - 223

CHAN OAN TANG AP PHOI TIEN PHAT

TRIEU CHNG BENH TAMP/TP GIAI OAN NANGNgat khi gang scDan tnh mach he thongPhu chi di, co chngLoan nhp nhanh hoac loan nhp chamNoi khan (MP dan chen TK quat ngc)Buon non, oi, an kem ngon, hoi chng giam hap thu, suy kietCHAN OAN TANG AP PHOI TIEN PHAT

TRIEU CHNG THC THEGiai oan sm: khong bat thng, T2 Giai oan nang:- am thoi h MP, h 3 la- clc tam thu, T2 , T3- trieu chng suy tim phaiCHAN OAN TANG AP PHOI TIEN PHAT

IEN TAM OGiai oan au: 50% TAMP/TP co ECG bnh thngLn nh phai, ln that phai (+)Co tng quan trc tiep gia bien o song R/V1 hoac ty le R/S V1 vi mc o ALMPo nhay 55%, o ac hieu 70%*

TL: * Ahearn GS et al. Chest 2002; 122: 524 - 527CHAN OAN TANG AP PHOI TIEN PHAT

XQUANG NGCCung MP phong; MP nho dan nhanh ve ngoai vi Ln nh phai, ln that phai

TL: Rubin LJ. The Heart ed by Fuster, Alexander, ORourkie. McGraw Hill 2004, 11th ed p.1580CHAN OAN TANG AP PHOI TIEN PHAT

SIEU AM TIMMc o ap lc MP: dong h 3 laChan oan phan biet vi TAMP th cap- Benh tim bam sinh- Benh van tim- Tim 3 buong nh- U nhay nh traiCHAN OAN TANG AP PHOI TIEN PHAT

SIEU AM DOPPLER GIUP O ALMPCHAN OAN TANG AP PHOI TIEN PHATHnh anh dong h van 3 la Pho Doppler lien tuc dong h van 3 la- Ap lc ong Mach Phoi tam thu la 95 mmHg.TL: Pham Nguyen Vinh. Atlas sieu am tim 2D & Doppler mau. NXB Y hoc 2000, p 79

TIM 3 BUONG NH: mot nguyen nhan TAMP th capCHAN OAN TANG AP PHOI TIEN PHATTL: Pham Nguyen Vinh. Atlas sieu am tim 2D & Doppler mau. NXB Y hoc 2000, p 65

U NHAY NH TRAI: mot nguyen nhan TAMP th capCHAN OAN TANG AP PHOI TIEN PHATTL: Pham Nguyen Vinh. Atlas sieu am tim 2D & Doppler mau. NXB Y hoc 2000, p 105

KHAO SAT CHC NANG PHOIKhao sat FE V1, FVC, TLC, VAGiam the tch song (vital capacity)/ TAMPGiup loai tr hoac xac nh nguyen nhan benh nhu mo phoi, benh ng th trong TAMP

FEV1: forced expiratory volume/ 1 secondFVC: forced vital capacityTLC: total lung capacityVA: alveolar volumeCHAN OAN TANG AP PHOI TIEN PHAT

XA KY TI MAU THONG KH PHOIRat can thiet, giup chan oan ung TAMP/NPTi mau co the bnh thng hoac co bat thng lan toa, tng mangGiup phan biet vi TAMP do huyet khoi thuyen tac.CHAN OAN TANG AP PHOI TIEN PHAT

XA KY TI MAU PHOI B/N TANG AP PHOICHAN OAN TANG AP PHOI TIEN PHATTL: Rich S. In Heart Disease, ed by Braunwald, Zipes, Libby; WB Saunders 2001, 6th edp. 1920

MSCT PHOI CO CAN QUANGng knh MP dan tai goc: y niem ve ap lc MP*Ton thng nhu mo phoiTang ap phoi do huyet khoi thuyen tac: thay oi nhieu kch thc mach mau phoi (co the thay the xa ky ti mau phoi)**

TL: * Tan RT et al. Chest 113: 1250 1256, 1998** Rich S. In Heart Disease, ed by Braunwald, Zipes, Libby; WB Saunders 2001, 6th ed p. 1921CHAN OAN TANG AP PHOI TIEN PHAT

THONG TIM CHUP MACH MAU PHOI CO CAN QUANGGiup loai tr TAMP th phatTAMP/ TP- khong tang ap lc bt mao mach phoi (ALBMMP)- giai oan cuoi: ALBMMP , khong qua 16mmHgThong tim: chan oan xac nh TAMP/TPCHAN OAN TANG AP PHOI TIEN PHAT

LNG NH KHA NANG GANG SCTrac nghiem i bo trong 6 phut (6 min walk test)Trac nghiem gang sc tim phoi kem o trao oi khCHAN OAN TANG AP PHOI TIEN PHAT

HAI IEM CHNH TRONG CHAN OAN TAMP/TPPhat hien TAMPLoai tr cac nguyen nhan TAMPchu y: * benh huyet khoi thuyen tac* benh chat keo* yeu to gia nhCHAN OAN TANG AP PHOI TIEN PHAT

HNG DAN CHAN OAN VA LNG NH TANG AP PHOITrieu chng c nang Kham thng qui Phat hien tnh cKham thc the xquang ngc ECG Sieu am timNghi TAMPTrac nghiem thiet yeuChc nang phoiXa ky phoi V/QTm benh chat keoHIVHuyet o, + tieu cauChc nang ganKhang the khang phospholipidsLng nh kha nang gang scThong tim phaiTrac nghiem phu thuoc nguyen nhanSATQTQCT xoan ocChup mach mauTrac nghiem ong mauSaO2, uric acid, BNP, TroponinTrac nghiem ve giac ngu (polysomnography)Sinh thiet phoiPhat hien benh(1)(2)(1)+ (2): xac nh va ac iem benhTL: Barst RJ et al. J Am Coll Cardiol 2004; vol 43, N012. Suppl S p 41SCHAN OAN TANG AP PHOI TIEN PHAT

DAU HIEU TIEN LNG XAU B/N TAPTP: LAM SANGTuoi < 14 hoac > 65Phan o NYHA bien oi: III, IVo I, II: song con 6 nam (trung bnh)o III: 2,5 namo IV: 0,5 namKhong gang sc cNgatKhai huyetTrieu chng suy that phai

TL: * Tan RT et al. Chest 113: 1250 1256, 1998CHAN OAN TANG AP PHOI TIEN PHAT

DAU HIEU TIEN LNG XAU B/N TAPTP: HUYET ONGo bao hoa oxy MP < 63%> 63%: 53% song con 3 nam< 63%: 17% song con 3 namCh so tim < 2,1l/phut/m2< 2,1: song con 17 thang (trung bnh)Ap lc nh phai > 10mmHg< 10mmHg: song con 4 nam (trung bnh)> 20mmHg: 1 thangKhong ap ng vi trac nghiem dan mach cap

TL: * Tan RT et al. Chest 113: 1250 1256, 1998CHAN OAN TANG AP PHOI TIEN PHAT

TIEN LNG B/N TAPTP C IEU TRNam t khiTat ca b/n:NYHA III, IV.NYHA III, IVchan oansong conieu tr thong thngieu tr eprostenolsong con (%)song con (%)1 687787252733 4841634305 342754ieu tr thong thng: khang vit K, digoxin, li tieu.

TL: Gibb JSR et al. Heart 2001; 86 (supple I) i1 i 13CHAN OAN TANG AP PHOI TIEN PHAT

NGUYEN TAC IEU TR TAPTP (1)CHAN OAN TANG AP PHOI TIEN PHATAcute vasodilator testResponderSvO2 > 63%, Cl > 2.1NYHA III, IVSvO2 < 63%, Cl < 2.1Non - responderNYHA I/II, SvO2 > 63%, Cl > 2.1Calcium antagonistWarfarin + diureticsDigoxin, oxygenFailed treatmentProstaglandin treatment + lung transplantTL: Gibbs JSR et al. Heart 2001; 86 (Suppl I): i1 i13

NGUYEN TAC IEU TR TAPTP (2)CHAN OAN TANG AP PHOI TIEN PHATNYHA IIIProstaglandinNYHA IVLung transplant listImprovement to NYHA IINo improvementSuspend from listDeteriorationTL: Gibbs JSR et al. Heart 2001; 86 (Suppl I): i1 i13

KET LUANTien lng nang, tien trien nhanh: can phat hien smLam sang: kho th cha ro nguyen nhan +++Tang ap MP: sieu am tim DopplerTang ap MP tien phat: phan tch loai tr cac nguyen nhanCHAN OAN TANG AP PHOI TIEN PHAT