1 B GIO DC V O TO B Y T TRNG I HC Y H NI Lấ C TI Nghiên cứu KHả NĂNG GắNG SứC BằNG TEST ĐI Bộ PHúT BệNH NHÂN TIM BẩM SINH Có HộI CHứNG EISENMENGER Chuyờn nghnh: Tim mch Mó s: 60720140 LUN VN THC S Y HC Ngi hng dn khoa hc: PGS.TS NGUYN LN HIU H NI 2015 LI CM N Nhõn dp hon thnh lun tt nghip Thc s, em xin chõn thnh cm n Ban giỏm hiu, Phũng o to sau i hc, B mụn Tim mch, Vin Tim mch Quc gia Vit Nam, Trung tõm Tim mch Bnh vin i hc Y H Ni ó giỳp v to mi iu kin thun li cho em quỏ trỡnh hc v nghiờn cu khoa hc Vi lũng kớnh trng v bit n sõu sc, em xin chõn thnh cm n PGS.TS Nguyn Lõn Hiu - ngi thy ó dỡu dt, ch bo tn tỡnh t em mi b ng bc chõn vo ngnh Tim mch v ó truyn li cho em nhng kin thc, kinh nghim quý bỏu sut quỏ trỡnh hc cng nh nghiờn cu khoa hc hon thnh lun ny Em xin trõn trng cm n GS.TS Phm Gia Khi, GS.TS Nguyn Lõn Vit nhng ngi thy ln luụn l tm gng hc cho em Em xin trõn trng cm n GS.TS Doón Li - Vin trng Vin Tim mch Quc gia Vit Nam, Ch nhim B mụn Tim mch trng i hc Y H Ni ó to mi iu kin thun li v giỳp cho em rt nhiu quỏ trỡnh hc v thc hin lun ny Em xin trõn trng cm n PGS.TS Trng Thanh Hng, Trng khoa C5 Vin Tim mch Quc gia Vit Nam ó tn tỡnh giỳp em cụng vic v to iu kin cho em hon thnh lun ny Em xin gi li cm n ti PGS.TS Nguyn Th Bch Yn, PGS.TS Phm Mnh Hựng, PGS TS Phm Th Hng Thi, TS Nguyn Ngc Quang, TS Phm Minh Tun ó tn tỡnh giỳp v ch bo em sut quỏ trỡnh hc Em xin chõn thnh cm n cỏc thy giỏo, cụ giỏo hi ng chm lun cựng cỏc Thy Cụ phn bin c lp ó cho em nhng ý kin quý bỏu em hon thin bn lun ny Em xin gi li cm n ti th cỏc anh ch bỏc s cựng ton th th cỏc cụ chỳ, anh ch em iu dng, h lý, cỏn b cỏc phũng ban ca Vin Tim mch v Trung tõm Tim mch Bnh vin i hc Y H Ni - nhng ngi ó ch dn, quan tõm em rt nhiu thi gian hc va qua c bit, em xin cm n nhng bnh nhõn nghiờn cu ny, cng nh nhng bnh nhõn em ó tham gia iu tr - h thc s l nhng ngi thy ln, l ng lc thỳc y em khụng ngng hc tp, phn u lm trũn bn phn ca ngi thy thuc Cui cựng, em xin dnh tt c tỡnh cm yờu quý v bit n ti nhng ngi thõn gia ỡnh, nhng ngi luụn ht long vỡ em cuc sng v hc H Ni, ngy 23 thỏng 11 nm 2015 Lờ c Ti LI CAM OAN Tụi l Lờ c Ti, hc viờn cao hc khúa 22, chuyờn ngnh Tim mch, Trng i hc Y H Ni, xin cam oan: õy l lun bn thõn tụi trc tip thc hin di s hng dn ca PGS.TS Nguyn Lõn Hiu Cụng trỡnh ny khụng trựng lp vi bt k nghiờn cu no khỏc ó c cụng b ti Vit Nam Cỏc s liu v thụng tin nghiờn cu l hon ton chớnh xỏc, trung thc v khỏch quan, ó c xỏc nhn v chp thun ca c s ni nghiờn cu Tụi xin hon ton chu trỏch nhim trc phỏp lut v nhng cam kt ny H Ni, ngy 23 thỏng 11 nm 2015 Lờ c Ti DANH MC CH VIT TT 6MWT : Six minutes walk test (test i b phỳt) ALMP : p lc ng mch phi ALMPtb : p lc ng mch phi trung bỡnh ALMPtt : p lc ng mch phi tõm thu ALMPttr : p lc ng mch phi tõm trng ATS : American Thoracic Society (Hi lng ngc M) COM : Cũn ng ng mch CPET : Cardiopulmonary exercise testing (Bi tim phi) Dd : Diastolic diameter (ng kớnh cui tõm trng tht trỏi) Ds : Systolic diameter (ng kớnh cui tõm thu tht trỏi) MP : ng mch phi T : in tõm EF : Ejection fraction (Phõn sut tng mỏu) ERC : European Resuscitation Council (Hi ng hi sc Chõu u) ESC : European Society of Cardiology (Hi tim mch hc Chõu u) NYHA : New York Heart Association (Hip hi Tim mch New York) PAH : Pulmonary arterial hypertension (Tng ỏp ng mch phi) PH : Pulmonary hypertension (Tng ỏp phi) PVR : Pulmonary vascular resistance (Sc cn mch phi) TAMP : Tng ỏp ng mch phi TBS : Tim bm sinh TLN : Thụng liờn nh TLT : Thụng liờn tht WHO : World Health Organisation (T chc Y t th gii) MC LC T VN CHNG 1: TNG QUAN TI LIU 13 1.1 TNG QUAN V TNG P NG MCH PHI V HI CHNG EISENMENGER 13 1.1.1 i cng tun hon phi 13 1.1.2 Tng ỏp ng mch phi 17 1.1.3 Hi chng Eisenmenger 23 1.2 TNG QUAN V TEST I B PHT 32 1.2.1 nh ngha test i b phỳt 35 1.2.2 u im ca test i b phỳt 35 1.2.3 Tớnh an ton ca test i b phỳt 35 1.2.4 K thut tin hnh nghim phỏp i b phỳt 36 1.3 CC CễNG TRèNH NGHIấN CU NH GI KH NNG GNG SC CA BNH NHN TIM BM SINH Cể HI CHNG EISENMENGER BNG TEST I B PHT 40 1.3.1 Cỏc cụng trỡnh nghiờn cu ỏnh giỏ trờn i tng nghiờn cu khe mnh 40 1.3.2 Mt s cụng trỡnh nghiờn cu ỏnh giỏ kh nng gng sc ca bnh nhõn tim bm sinh cú hi chng Eisenmenger bng test i b phỳt 40 1.3.3 Ti Vit Nam 41 CHNG 2: I TNG V PHNG PHP NGHIấN CU 42 2.1 I TNG NGHIấN CU 42 2.1.1 Tiờu chun la chn bnh nhõn 42 2.1.2 Tiờu chun loi tr 43 2.2 PHNG PHP NGHIấN CU 43 2.2.1 Thit k nghiờn cu 43 2.2.2 a im nghiờn cu 43 2.2.3 Thi gian nghiờn cu 44 2.2.4 Quy trỡnh nghiờn cu 44 2.2.5 Thu thp s liu 45 2.2.6 Tin hnh thc hin test i b phỳt 48 2.2.7 X lý s liu 50 2.2.8 o c nghiờn cu 50 CHNG 3: KT QU NGHIấN CU 51 3.1 C IM CHUNG CA CC I TNG NGHIấN CU 51 3.1.1 c im v tui 51 3.1.2 c im v gii 51 3.1.3 c im d tt bm sinh 52 3.1.4 c im lõm sng ca cỏc i tng nghiờn cu 55 3.1.5 Mt s c im cn lõm sng ca cỏc i tng nghiờn cu 56 3.2 KT QU CA 6MWT TRấN I TNG NGHIấN CU V S THAY I SPO2, MCH 60 3.2.1 Giỏ tr quóng ng i c ca 6MWT 60 3.2.2 Thay i SpO2 v mch ca bnh nhõn thc hin 6MWT 60 3.3 CC THễNG S NH HNG N 6MWT 61 3.3.1 Gii 61 3.3.2 Loi d tt 62 3.3.3 Mc khú th NYHA 63 3.4 LIấN QUAN GIA QUNG NG I C CA 6MWT VI MT S THễNG S NGHIấN CU 64 3.4.1 Liờn quan gia quóng ng i c v SpO2 66 3.4.2 Liờn quan gia quóng ng i c v ALMP tõm thu 67 3.4.3 Liờn quan gia quóng ng i c v sc cn phi (Rp) 68 3.4.4 Liờn quan gia quóng ng i c v t l Rp/Rs 69 CHNG 4: BN LUN 70 4.1 BN V C IM CHUNG CA I TNG NGHIấN CU 70 4.1.1 Tui 70 4.1.2 Gii 70 4.1.3 c im d tt 71 4.1.4 Triu chng lõm sng 72 4.1.5 Triu chng cn lõm sng 74 4.2 BN V KT QU CA 6MWT TRấN I TNG NGHIấN CU V S THAY I CC CH S NGHIấM CU 76 4.2.1 Bn v giỏ tr quóng ng i c ca 6MWT 76 4.2.2 Bn v s thay i cỏc ch s mch v Sp02 ca bnh nhõn lm 6MWT 77 4.3 BN V CC THễNG S NH HNG N 6MWT 79 4.3.1 Gii 79 4.3.2 c im d tt 79 4.3.3 Phõn loi NYHA theo WHO 80 4.3.4 Tng quan gia giỏ tr quóng ng i c v sp02 81 4.3.5 Tng quan gia giỏ tr quóng ng i c v ỏp lc ng mch phi tõm thu 81 4.3.6 Tng quan gia giỏ tr quóng ng i c v sc cn ng mch phi 82 4.3.7 Tng quan gia giỏ tr quóng ng i c v t l Rp/Rs (t l gia sc cn ng mch phi v sc cn h thng) 83 4.3.8 Mi liờn h gia cỏc yu t tui, Qp, Qp/Qs, ALMPtb v mt s ch s trờn siờu õm tim vi giỏ tr quóng ng i c ca 6MWT 85 4.4 HN CH CA NGHIấN CU 85 KT LUN 86 KIN NGH 87 TI LIU THAM KHO PH LC DANH MC BNG Bng 1.1 Tr s ỏp lc bỡnh thng ca mt s bung tim v mch mỏu 15 Bng 1.2 Phõn loi tng ỏp phi theo Dana Point, USA 18 Bng 1.3 Phõn loi tng ỏp phi theo huyt nga 19 Bng 1.4 Phõn loi TAMP liờn quan bnh tim bm sinh 20 Bng 1.5 Phõn loi cỏc bnh TBS gõy TAMP theo ESC/ERS 26 Bng 1.6 Ch nh cho test i b phỳt theo hng dn ca ATS 34 Bng 1.7 Thang im Borg 39 Bang 3.1 Bng 3.2 Phõn b cỏc i tng theo loi d tt bm sinh 52 Bang 3.3 Bang 3.4 Phõn b cỏc loi d tt theo gii 53 Bng 3.5 c im chiu lung thụng 54 Bng 3.6 Cỏc triu chng c nng 55 Bng 3.7 Triu chng khú th theo NYHA 55 Bng 3.8 Cỏc triu chng thc th 56 Bng 3.9 c im in tõm 56 c im v tui theo nhúm d tt 52 Kớch thc ca cỏc loi d tt 54 Bng 3.10 c im X-Quang tim phi thng 57 Bng 3.11 c im v xột nghim mỏu 57 Bng 3.12 c im v siờu õm tim 58 Bng 3.13 c im v thụng tim huyt ng 59 Bng 3.14 Giỏ tr quóng ng i c 60 Bng 3.15 S thay i cỏc ch s nghiờn cu 60 Bng 3.16 c im chung ca gii nam v n 61 Bng 3.17 Quóng ng i c theo d tt 62 Bng 3.18 c im chung i tng nghiờn cu theo NYHA 63 Bng 3.19 Tng quan gia quóng ng v cỏc thụng s nghiờn cu 64 10 DANH MC BIU Biu 3.1 Phõn b v gii ca cỏc i tng nghiờn cu 51 Biu 3.2 Phõn b cỏc loi d tt theo gii 53 Biu 3.3 Giỏ tr quóng ng i c theo gii 61 Biu 3.4 Giỏ tr quóng ng i c theo NYHA 63 Biu 3.5 Tng quan gia quóng ng i c ca 6MWT v SpO2 66 Biu 3.6 Tng quan gia quóng ng i c ca 6MWT v ỏp lc MP tõm thu 67 Biu 3.7 Tng quan gia quóng ng i c ca 6MWT v sc cn MP 68 Biu 3.8 Tng quan gia quóng ng i c ca 6MWT v t l Rp/Rs 69 Biu 4.1 Thay i mch v SpO2 lm 6MWT 77 87 KIN NGH T kt qu nghiờn cu, chỳng tụi kin ngh nờn s dng test i b phỳt nh l mt test thng quy ỏnh kh nng gng sc nhng bnh nhõn tim bm sinh cú hi chng Eisenmenger v cỏc yu t nh hng, chunhs tụi nhn thy t l Rp/Rs l mt ch s ỏng tin cy ỏnh giỏ kh nng gng sc nhng nhúm bnh nhõn ny TI LIU THAM KHO Guillevin L, 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A meta -analy sis of 22 randomized trials ", J Am Coll Card iol 0(13), tr 119 2-201 BNH N NGHIấN CU I HNH CHNH: 1.1 H v tờn: Tui Gii: Nam N 1.2 Ngh nghip: 1.3 a ch: 1.4 a ch liờn lc: 1.5 Ngy vo vin: Ngy vin: 1.6 Khoa: Mó bnh nhõn: 1.7 Bnh vin: 1.8 Chn oỏn: S in thoi: II TIN S: 2.1 Phỏt hin bt thng tim mch ln u: 2.2 Lớ i khỏm ln u: 2.3 Nm chn oỏn Eisenmenger: 2.4 Cú iu tr khụng? Cú 2.5 Bnh ng mch vnh 2.6 Bnh phi hn ch hoc bnh tc nghn phi Khụng III LM SNG: 3.1 3.2 Triu chng c nng: Khú th (NYHA: ) Hi hp, trng ngc au ngc Ngt Ho mỏu Mt mi Triu chng thc th: 3.2.1 Ton thõn: í thc: Tnh, tip xỳc tt Ngt Cõn nng:.(Kg) Tnh, mt Chiu cao: (cm) BMI: Th trng: Gy Trung bỡnh Bộo Mch: nhp/phỳt Huyt ỏp:/ mmHg Nhit : oC Nhp th: ln/phỳt Tớm trung ng: Phự hai chi d i: Ngún tay dựi trng: 3.2.2 Khỏm tim mch: Lng ngc bin dng: So thnh ngc: Lớ do: SpO2: % Mm tim p KLS: Din p: Rung miu: V trớ: Nghe tim: Tn s ck/phỳt Nhp: u NTT Ting tim: T2 mnh van MP T2 tỏch ụi c nh van MP TTT ( van lỏ) LNHT Cỏc ting bt thng khỏc: 3.2.3 Cỏc triu chng ngoi tim: Khỏm phi Gan to cm di b sn C trng: Phn hi gan tnh mch c: IV CN LM SNG: 4.1 X quang tim phi: Ch s tim ngc :% Rn phi m, m, rng: Cung MP phng : Dy tht phi, nh phi: Cỏc bt thng khỏc: 4.2 in tõm : Tn s: nhp/phỳt Nhp: Trc in tim: Dy nh phi: Dy tht phi: Cỏc bt thng khỏc: 4.3 Xột nghim mỏu: Hng cu:ì109 HST : .g/l HCT : % MCV : fl BC: ì106 TC: ì106 4.4 Húa sinh mỏu: AST: UI/L ALT: UI/L Bilirubin ton phn: àmol/L Bilirubin trc tip: àmol/L 4.5 Siờu õm tim: V trớ l thụng: ng kớnh l thụng: NT MC Dd Ds Vd Chiu lung thụng: Hai chiu u th shunt: PT Thụng s Vs %D EF TP T P Giỏ tr p lc ng mch phi (mmHg) ng kớnh gc ng mch phi (mm) ng kớnh thõn ng mch phi (mm) Nhn xột khỏc: 4.6 Thụng tim: Din tớch da (BSA): m2 Nhp tim : ck/phỳt Th tớch O2 tiờu th: ml/ph/m2 Thụng s Giỏ tr Thụng s p lc nh phi (mmHg) p lc MP (mmHg) p lc MC(mmHg) p lc nh trỏi (mmHg) Bóo hũa O2 TMCT Bóo hũa O2 MP Bóo hũa O2 nh trỏi Bóo hũa O2 MC Lu lng phi Qp (l/ph) Lu lng ch Qs (l/ph) Sc cn phi Rp (Woods) Sc cn ch Rs (Woods) Qp/Qs Rp/Rs Giỏ tr Nhn xột khỏc: V Test i b phỳt (6MWT): Cỏc thụng s trc thc hin 6MWT: - Ngy lm test: - Thi gian lm test: - Mch: ck/p; - Huyt ỏp: / mmHg - Sp02: % Sau thc hin trc nghim: - Bnh nhõn cú phi ngng 6MWT trc phỳt khụng? Cú: Khú th: Lý c bit khỏc: Khụng: - Cú phi s dng liu phỏp Oxy quỏ trỡnh trc nghim khụng? Cú: Khụng: Tc : .mL/p - Cỏc thụng s sau thc hin trc nghim: + Ngay sau hon thnh (Thi im phỳt) Thi gian: Sp02: % Mch: ck/p + phỳt sau test i b Thi gian: Sp02: % Mch: ck/p + phỳt sau test i b Thi gian: Sp02: % Mch: ck/p - Ch s khú th Borg: - Tng quóng ng i c: .m - Nhn xột khỏc: ... sức test phút bệnh nhân tim bẩm sinh có hội chứng Eisenmenger với mục tiêu: Bước đầu tìm hiểu khả gắng sức test phút bệnh nhân tim bẩm sinh có hội chứng Eisenmenger Các yếu tố ảnh hưởng đến test. .. khả gắng sức bệnh nhân bệnh phổi mãn tính đối 13 tượng bệnh nhân tim bẩm sinh có hội chứng Eisenmenger [14],[15],[ 16] Tại Việt Nam có công trình nghiên cứu test phút đối tượng bệnh nhân suy tim, ... đoạn gọi hội chứng Eisenmenger [5] 1.1.3 Hội chứng Eisenmenger Từ kỷ trước người ta ý nghiên cứu nhiều đến giai đoạn cuối bệnh TAĐMP bệnh nhân có bệnh tim bẩm sinh hay gọi hội chứng Eisenmenger