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1 B GIO DC V O TO B Y T TRNG I HC Y H NI Lấ C TI NghiêncứuKHảNĂNGGắNGSứCBằNGTESTĐIBộ PHúT BệNHNHÂNTIMBẩMSINHCóHộICHứNGEISENMENGER 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, Armstrong I, Aldrighetti R, et al (2013), Understanding the impact of pulmonary arterial hypertension on patients and careers lives", Eur Respir Rev 22, 535-542 Kỹỗỹkolu MS, Bakurt M, (2010), Pulmonary hypertension: diagnosis and clinical classification, Anatol J Cardiol 10(1), 2-4 Duffels MG, Engelfriet PM, Berger RM (2007), Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry, Int J Cardiol 120(2), 198-204 Harald Kaemmerer, Siegrun Mebus, Ingram Schulze-Neick, et al (2010), The Adult Patient with Eisenmenger Syndrome: A Medical Update After Dana Point Part I: Epidemiology, Clinical Aspects and Diagnostic Options, Current Cardiology Review 6(4), 343-355 Wood Paul (1958), The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt, Brit Med J 46 Diller G.P, Konstantinos Dimopoulos, Craig S Broberg, et al (2006), Presentation, survival prospects and predictors of death in Eisenmenger syndrome: a combined retrospective and case control study, Eur Heart J 27(14), 1737-1742 Diller GP, Dimopoulos K, Okonko D, et al (2005), Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication, Circulation 112, 828-835 Can MM, Kaymaz C (2010), Clinical end-points and surrogate markers of pulmonary arterial hypertension in the light of evidence-based treatment, Anatol J Cardiol 10(1), 36-42 Poole Wilson PA (2000), The six minute walk test: A simple test with clinical applicatio, Eur Heart J 21(7), 507-508 10 Hunh Vn Minh (2004), Trc nghim i b phỳt, Thụng tin ni khoa, Bnh vin Trng i hc Y khoa Hu, 20-22 11 Rasekaba T, Lee AL, Naughton MT, et al (2009), The six-minute walk test: a useful metric for the cardiopulmonary patient, Intern Med J 39, 495-501 12 ATS Statement (2002), Guidelines for the Six-Minute Walk Test, American journal of respiratory and critical care medicine 166, 111-117 13 Galiố Nazzareno, Adam Torbicki, Robyn Barst, et al (2009), Guidelines on diagnosis and treatment of pulmonary arterial hypertension, Eur Heart J 25, 2243 - 78 14 Paul L Enright, Duane L Sherrill (1998), Reference Equations for the Six-Minute Walk in Healthy Adults, Am J Respir Crit Care Med 158, 13841387 15 Hodgev VA, Aliman OI, Marinov BI, et al (2003), Cardiovascular and dyspnea response to six-minute and shuttle walk tests in COPD patients, Folia Med 45, 26-33 16 Kempny A, Dimopoulos K, Alonso-Gonzalez R, et al (2013), Sixminute walk test distance and resting oxygen saturations but not functional class predict outcome in adult patients with Eisenmenger syndrome, Int J Cardiol 168, 4784-4789 17 Lờ Th Nguyt (2007), Nghiờn cu trc nghim i b phỳt ỏnh giỏ bnh nhõn suy tim khoa ni tim mch bnh vin trung ng Hu, Lun tt nghip bỏc s Y khoa, i hc Y Dc Hu 18 Nguyn Thnh Nhõn (2009), ỏnh giỏ bnh c tim thiu mỏu cc b bng trc nghim i b phỳt kt hp mỏy in tõm cm tay (Dailycare 8F) 19 Phm Th Minh c (2003), Sinh lý hc tun hon phi, Bi ging sinh lý hc, Trng i hc Y H Ni 20 Nguyn Lõn Vit (1994), Gúp phn nghiờn cu mt s thụng s siờu õm v ng mch phi ngi bỡnh thng v ngi cú tng ỏp ng mch phi., Lun ỏn tin s y hc, i hc Y H Ni 21 Wilson Jean D (1999), Thụng tim v chp mch chn oỏn, Cỏc nguyờn lý ni khoa Harrison, Nh xut bn Y hc, 66 - 76 22 V Minh Thc, inh Vn Ti (1983), p lc ng mch phi ca ngi Vit Nam bỡnh thng, Tp ni khoa 3, 19 - 25 23 Beghetti Maurice, Rolf Berger, Damien Bonnet et al (2007), Applying the evidence of PAH in everyday clinical practice, Association for European Paediatric Cardiology (AEPC) Congress; Warsaw, Poland 24 Galiố N, Humbert M, Vachiery, et al (2015), ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension, Eur Heart J 25 Diller G.P MA, Gatzoulis (2007), Pulmonary vascular disease in adults with congenital heart disease, Circulation 115, 1039 - 1050 26 Simonneau G, Robbins I.M, Beghetti M, et al (2009), Updated clinical classification of pulmonary hypertension, J Am Coll Cardiol 54(1), 43-54 27 Brickner M E, L David Hillis v Richard A Lange (2000), Congenital heart disease in adults, N Engl J Med 342, 988 28 Alpert Joseph S, James E Dalen (1991), Pulmonary vascular disease in adults with congenital heart disease, Cardiology 2, 1177 - 1187 29 Julian Desmond G, Cowan J Campbell (1992), High pulmonary vascular resistance with right to left shunt through a septal defect or ductus arteriosus (Eisenmenger syndrome), Cardiology 6, 288 - 290 30 L Daliento, J Somerville, P Presbitero, et al (1998), Eisenmenger syndrome: Factors relating to deterioration and death, European Heart Journal 19, 1845-1855 31 Young D, Mark H (1971), Fate of the patient with the Eisenmenger syndrome, Am J Cardiol 28(6), 658-569 32 Kidd L, Driscoll DJ, Gersony WM, et al (1993), Second natural history study of congenital heart defects Results of treatment of patients with ventricular septal defects, Circulation 87(2), 138-151 33 Saha A, Balakrishnan KG, Jaiswal PK (1994), Prognosis for patients with Eisenmenger syndrome of various aetiology, Int J Cardiol 45(3), 199-207 34 Vongpatanasin W, Brickner ME, Hillis LD, et al (1998), The Eisenmenger syndrome in adults, Ann Intern Med 128(9), 745-55 35 G P Diller, M A Gatzoulis (2007), Pulmonary vascular disease in adults with congenital heart disease, Circulation 115(8), 1039-50 36 Park M K (2008), Pediatric cardiology for practitioners, Vol 5, Elsevier Mosby, London, 650-680 37 Brickner M E, Hillis L D, Lange R, et al (2000), Congenital heart disease in adults Second of two parts, N Engl J Med 342(5), 334-342 38 Phm Nguyn Vinh v cỏc cng s (2001), Bnh tim bm sinh ngi trng thnh, Mt s cp nht chn oỏn v iu tr bnh tim mch, Nh xut bn Y hc, 357 - 388 39 Nguyn Lõn Vit (2007), Hi chng Eisenmenger, Thc hnh bnh tim mch, Nh xut bn Y hc, 605 - 606 40 Kuida Hiroshi (1990), Primary and secondary pulmonary hypertension: pathophysiology, recognition and treatment, The Heart, 7, 1191 - 1198 41 Cooper KH (1968), A means of assessing maximal oxygen intake: correlation between field and treadmill testing, JAMA 203(3), 201-204 42 Paul L Enright MD (2003), The Six-Minute Walk Test, Respiratory Care 48(8), 783-785 43 C R McGavin, M Artvinli, H Naoe, et al (1978), Dyspnoea, disability, and distance walked: comparison of estimates of exercise performance in respiratory disease, Br Med J (6132), 241-243 44 Butland RJ, Pang J, Gross ER, eta al (1982), Two-, six-, and 12-minute walking tests in respiratory disease, British Medical Journal 284(6329), 1607-1608 45 Victor Zuniga Dourado (2011 ), Reference Equations for the 6-Minute Walk Test in Healthy Individuals, Arq Bras Cardiol 46 Amanda F, Casey, Xu Wang, et al (2012), Test-Retest Reliability of the 6-Minute Walk Test in Individuals With Down Syndrome, Arch Phys Med Rehabil Vol, November 93, 2068-2074 47 Troosters T, Vilaro J, Rabinovich R, et al (2002), Physiological responses to the 6-min walk test in patients with chronic obstructive pulmonary disease, Eur Respir J 20(3), 564-569 48 Solway S, Brooks D, Lacasse Y, et al (2001), A qualitative systematic overview of the measurement properties of functional walk tests used in the cardiorespiratory domain, Chest 119(1), 256-270 49 Richard M.Streingart (2001), Sex differences in diagnosic and treatment of CAD, Cardiology Review 18(3), 22-25 50 Roomi J, Jonhson MM, Waters K, et al (1996), Respiratory rehabilitation, exercise capacity and quality of life in chronic airways disease in old age, Age Ageing 25(1), 12-16 51 Enright PL, Mc Burnie MA, Bittner V, et al (2003), The minute walk test: a quick measure of functional status in elderly adults, Chest 123(2), 387-398 52 Deboeck G, Niset G, Vachiery JL, et al (2005), Physiological response to the six-minute walk test in pulmonary arterial hypertension, Eur Respir J 26(4), 667-672 53 Gerhard-Paul Diller, Rafael Alonso-Gonzalez, Konstatinos Dimopoulos, et al (2013), Disease targeting therapies in patients with Eisenmenger syndrome: Response to treatment and long-term efficiency, International Journal of Cardiology 167, 840-847 54 Skjodt NM, Ritz C, Vethanayagam D, (2008), The pulse oxygen saturation: inspired oxygen pressure (SpO2:PIO2) diagram: application in the ambulatory assessment of pulmonary vascular disease, Adv Exp Med Biol 605, 492-496 55 Barst R J, Mcgoon M, Torbicki A, et al (2004), Diagnosis and differential assessment of pulmonary arterial hypertension, J Am Coll Cardiol 43(12), 40S-47S 56 Lisa Bergersen, Susan Foerster, Audrey C Marshall, (2009), Congenital Heart Disease: The Catheterization Manual, Spring Science, New York, USA 57 Th Thu Trang (2012), Tỡm hiu giỏ tr tiờn lng ca ch s E/Ea trờn bnh nhõn tng ỏp ng mch phi nhiu bnh tim bm sinh cú lung thụng tim, Lun tt nghip Bỏc s Ni trỳ, i hc Y H Ni 58 Silversides CK, Granton JT, Konen E, et al (2003), Pulmonary thrombosis in adults with Eisenmenger syndrome, J Am Coll Cardiol 42(11), 1982-1987 59 Park M K, (2008), Pediatric cardiology for practitioners, 5, Elsevier Mosby, London, 650-680 60 Nguyn Th Minh Lý (2008), Nhn xột c im lõm sng v kt qu thm dũ huyt ng trờn bnh nhõn tim bm sinh cú tng ỏp lc ng mch phi nng, Lun bỏc s Y khoa, i hc Y H ni 61 Erwin Oechslin, Siegrun Mebus, Ingram Schulze-Neick, et al (2010), The Adult Patient with Eisenmenger Syndrome: A Medical Update after Dana Point Part III: Specific Management and Surgical Aspects, Current Cardiology Reviews 6(4), 363-372 62 Baptista Rui, Castro Graca, da Silva Antúnio Marinho, (2013), Longterm effect of bosentan in pulmonary hypertension associated with complex congenital heart disease, Rev Port Cardiol 32, 123-129 63 Galie N, Beghetti M, Gatzoulis MA, et al (2006), Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study, Circulation 114, 48-54 64 Iversen K, Jensen AS, Jensen TV, et al (2010), Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial, Eur Heart J 31, 1124-1131 65 Provencher S, Chemla D, Hervộ P, et al (2006), Heart rate responses during the 6-minute walk test in pulmonary arterial hypertension, Eur Respir J 27(1), 114-120 66 Omar A Minai, Ravi Gudavalli, Srinivas Mummadi, et al (2012), Heart Rate Recovery Predicts Clinical Worsening in Patients with Pulmonary Arterial Hypertension, Am J Respir Crit Care Med 185(4), 400-408 67 Miyamoto S, Nagaya N, Satoh T, et al (2000), Clinical correlates and prognostic significance of six-minute walk test in patients with primary pulmonary hypertension Comparison with cardiopulmonary exercise testing, Am J Respir Crit Care Med 2, 487-492 68 Berger RM, Beghetti M, Galiố N, et al (2010), Atrial septal defects versus ventricular septal defects in BREATHE-5, a placebo-controlled study of pulmonary arterial hypertension related to Eisenmenger's syndrome: a subgroup analysis, Int J Cardiol 144(3), 373-378 69 Buber J, Rhodes J, (2014), Exercise physiology and testing in adult patients with congenital heart disease, Heart Fail Clin 10(1), 23-33 70 Sommer RJ, Hijazi ZM, Rhodes JF Jr, (2008), Pathophysiology of congenital heart disease in the adult: part I: Shunt lesions, Circulation 117(8), 1090-1099 71 Humbert M, Sitbon O, Chaouat A, et al (2006), Pulmonary arterial hypertension in France: results from a national registry, Am J Respir Crit Care Med 173, 1023-1030 72 Van De Bruaene A, De Meester P, Voigt JU, et al (2013), Worsening in oxygen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger syndrome, Int J Cardiol 168(2), 1386-1392 73 Reesink HJ, Van der Plas MN, Verhey NE, et al (2007), Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension, J Thorac Cardiovasc Surg 133(2), 510-516 74 Gungor H, Fatih Ayik M, Engin C, et al, (2014), Transthoracic echocardiographic and cardiopulmonary exercise testing parameters in Eisenmenger's syndrome Association with six-minute walk test distance, Herz 39(5), 633-637 75 Savarese G, Paolillo S, Costanzo P, et al (2012), Do changes of 6minute walk distance predict clinical events in patients with pulmonary arterial hypertension? A meta-analysis of 22 randomized trials, J Am Coll Cardiol 60(13), 1192-1201 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 chronic obstructive p ulmonary disease", Eur Respir J 20(3), tr 564-56 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 ", Heart Fa il Clin 10(1), tr 23-3 70 71 72 73 74 75 Armstrong I Guillevin L, A ldrighetti R, et al (201 3), " Understand ing the impact of pulmonary arterial hy pertension on patients and careers lives", Eur Resp ir Rev 22, tr 35-42 Ba kurt M Kỹỗỹko lu M S (2010), "Pulmonary hy pertension: diagno sis and clinical class ification ", Anatol J Cardio l 10(1), tr 2-4 Duffels MG, Engelfriet PM v Berger RM (2007), "Pulmonary arterial hy pertension in congenital heart disease: an epidemiolog ic perspective from a Dutch registry ", Int J Card iol 20(2), tr 198-20 Siegrun Mebus Harald Kae mmerer, Ingra m Sc hulze-Neic k, et al, (2010), "T he Adult Patien t with Eisenmenger Sy ndrome: A Medical Update After Dana Point Part I: Epidemiology , Clinical Aspects an d Diagnos tic Options ", Current Card iolo gy Review 6(4), tr 343-3 55 Wood P aul (1958), "The Eisenmenger sy ndrome or pulmonary hy pertension with reversed central shunt ", Br it Med J 46 Konstantinos D imopoulos Diller G.P, Craig S Broberg, et al (2006), "Presentation , survival prospects and predictors of death in E isenmenger sy ndrome: a combined retrospective and case control study ", Eu r Heart J 27(14), tr 1737-17 42 Dimo po ulo s K D iller GP, Okonko D, et al (2005), "Exercise intolerance in adult congenital heart disease: comparative severity , correlates, and prognostic implication ", Circula tion 112, tr 828-35 Kay ma z C Ca n MM (2010), "Clinical end-poin ts and s urrogate markers of pulmonary arterial hy pertension in the light of evidence -based treatment", Anatol J Card iol 0(1), tr 36-42 Poole Wilso n PA (2000), "The six ute walk tes t: A s imple test with clin ical applicatio ", Eu r Heart J 21(7), tr 507-508 Hu nh Vn Minh (200 4), "Trc nghim i b phỳt", Thụng tin n i khoa, Bnh vin Tr n g i hc Y kh oa Hu, tr 20-22 Lee AL Rase kaba T, Na ug hton MT, et al (20 09), "The s ix-minute walk tes t: a useful metric for the cardiopulmonary patient", Intern Med J 39, tr 49 5-501 ATS Stateme nt (2002), Guidelines for the S ix-Minute Walk Test, AM ERICAN JOURNA L OF RE SPIRATORY AND CRITICAL CARE MEDICINE VOL 166 200 Galiố Nazzareno v cỏc cng s (20 09), "Gu idelines o n diagno sis and treatment of pulmonary arterial hy pertension", Eur Hear t J 25, tr 243 - 78 PAUL L ENRIGHT v DUAN E L SHER RILL (1998), "Reference Equations for the Six-M inute Walk in Healthy Adults", AM J RES PIR CRI T CARE MED 158, tr 1384 1387 Aliman OI Ho dgev VA, Marinov BI, et al (200 3), "Cardiovascular and dy spnea response to six-minu te and shuttle walk tes ts in COPD patien ts", Folia Med 45, tr 26-33 Kempny A, Dimo po ulos K v Alo nso-Gonzale z R (2013), "Six-minute walk tes t dis tance and resting oxy gen saturation s but not functional class predict outcome in adult patients with Eise nmenger sy ndrome", Int J Cardiol 168, tr 478 4-4789 Lờ Th Ng uyt (2007), Nghiờn cu trc ng him i b phỳ t tr ong ỏn h giỏ bnh nhõn suy tim khoa n i tim mch bnh vin trung ng Hu, Lun t t ngh ip bỏc s Y khoa, i hc Y Dc Hu Nguyn Thnh N hõ n (2009), ỏnh g iỏ bnh c tim thiu mỏu cc b bng trc nghim i b phỳt k t hp mỏy in tõm cm tay (Daily care 8F), ch biờn Ph m Th Minh c (2003), "Sinh lý hc tun h on phi", Bi gin g sinh lý hc, Trng i h c Y H Ni Nguyn Lõ n Vit (1994), Gúp phn n ghiờn cu mt s thụn g s s iờu õm v ng mch phi ng i bỡnh thng v n gi cú t ng ỏp ng mch ph i., Lun ỏn tin s y hc, i hc Y H Ni Wilso n Jea n D (1999), "Thụng tim v chp mch chn oỏn", Cỏc n guyờn lý ni khoa Harrison, Nh x ut bn Y hc, tr 6 - 76 V Minh Thc v inh V n Ti (1983), "p l c ng mch phi ca ng i Vit Nam bỡnh thng ", Tp n i khoa 3, tr 19 - 25 Beghetti Maurice, Rolf Berger v Damien Bo nnet (2007), Ap plying the evidence of PAH in everyday clinical practice , Ass ociation for Euro pean Paediatric Cardiology (AEPC) Congress; Warsaw, Poland Galiố N v cỏc cng s (20 15 Aug 9), "E SC/ERS Guidelines for the d iagnosis and treatment of pulmonary hy pertension", Eur Heart J Diller G.P, MA v Gatzo ulis (2007), "Pu lmonary vascular disease in adults with congenital heart d isease", Circula tion 115, tr 039 - 105 Simo nnea u G v cỏc cng s (2009), "Updated clin ical classification of pulmonary hy pertension", J Am Coll Cardio l 54(1), tr 43-54 Brickner M Eliza beth, L David Hillis v Richar d A Lange (2000), "Congenital heart disease in adu lts ", N E ngl J Med 342 , tr 988 Alpert Joseph S v Ja mes E Dale n (1991), "Pulmonary vascular disease in adults with congenital heart disease ", Cardiology 2, tr 1177 - 87 Julian Des mo nd G v Cowa n J Ca mpbell (1992), "High pulmonary vascular resistance with right to left sh unt through a septal defect or ductus arteriosus (Eisenmenger sy ndrome).", Cardiology 6, tr 288 - 90 L Daliento v cỏc cng s (1998), "Eisenmenger sy ndrome: Factors relating to deterioration and death ", Eur opean Heart Jou rnal 19, tr 18 45-1855 Young D v Mar k H (19 71), "Fate of the patient with the Eisenmenger sy ndrome", Am J Cardio l 28(6), tr 658-69 Kidd L v cỏc cng s (1993), "Second natural his tory study of congenital heart defects Results of treatment of patients with ventricular septal defects", Circu lation 87(2), tr 138-51 Saha A, Bala kr is hna n KG v Jais wal P K (1994), "Prognosis for patients w ith E isenmenger sy ndrome of various aetiology ", Int J Card iol 5(3), tr 199-20 Vong patanasin W v cỏc cng s (19 98), "The E isenmenger sy ndrome in adults", Ann In tern Med 128(9), tr 745-55 G P Diller v M A Gatzo ulis (2007), "Pulmonary vascular disease in adults with congenital heart disease", Circulation 115(8), tr 039-50 Par k M K (20 08), Pediatric cardiology fo r practitio ners, Tp 5, E lsevier Mo sby , London, tr.6 50-680 Hillis L D & Lange R Brickner M E (200 0), "Congenital heart disease in adults Second of two parts", N Eng l J Med 342(5), tr 33 4-342 Ph m Ng uyn V inh v cỏc c ng s (200 1), "Bnh tim bm sinh ng i trng thnh ", Mt s cp nh t chn oỏn v iu tr bnh tim mch, Nh xut bn Y hc, tr 357 - 8 Nguyn Lõ n Vit (2007), "Hi chng Eisenmenger", Thc h nh bnh tim mch, Nh xut bn Y hc, tr 05 - 606 Kuida Hiros hi (19 90), "Primary and secondary pulmonary hy pertension: pathophy sio logy , recognition and treatment", The Hear t, 7, tr 1191 - 98 Cooper KH (1968), "A means of assessing maximal oxy gen intake: correlation between field and treadmill testing ", JAMA 20 3(3), tr 201-20 Paul L Enrig ht MD (2003), "The Six-Min ute Walk Test ", RESPIRA TOR Y CARE 48(8), tr 78 3-785 C R McGavin v cỏc cng s (1978), "Dy spnoea, disability , and distance walked: comparison of estimates of exercise performance in respiratory disease ", Br Med J 2((6132), tr 241-243 Butland RJ v cỏc c ng s (198 2), "Two-, s ix-, and 12-minu te walking tests in respiratory disease", British Med ical Journa l 284(6 329), tr 16 07-8 Victor Zuniga D oura (2011 ), "Reference Equations for the 6-M inute Walk Tes t in Healthy Individuals ", Arq Bras Car diol Ama nda F v cỏc cng s (2012), "Test- Retest Reliability of the 6-Minu te Walk Test in Indiv iduals With Down Sy ndrome ", Arch Phys Med Rehabil Vol, November 93, tr 2068-2 074 Troosters T v cỏc c ng s (200 2), "Phy siolog ical responses to the 6-min walk tes t in patients w ith Solway S v cỏc cng s (2 001), "A q ualitative sy stematic overview of the measurement properties of functional walk tests u sed in the cardiorespiratory domain", Chest 19(1), tr 256-70 Richard M Streingart (2001), "Sex d ifferences in diagnosic and treatment of CAD ", Cardio logy Review 18(3), tr 22-25 Roomi J v cỏc c ng s (199 6), "Res piratory rehabilitation, exercise capacity and quality of life in chronic airway s disease in old age", Age Ageing 25(1), tr 12-16 Enrig ht P L v cỏc cng s (2003), "The minute walk test: a qu ic k measure of functional status in elderly adults ", Chest 12 3(2), tr 387-398 Deboec k G1 v cỏc cng s (2005), "Phy siolo gical response to the six-minute walk test in pu lmonary arterial hy pertension", Eur Resp ir J 26(4), tr 667-72 Gerhard-Paul Diller v cỏc cng s (2013), "D isease targeting therapies in patien ts with Eisenmenger sy ndrome: Response to treatment and long-term efficiency ", Internation al Journ al of Car diology 167, tr 840-847 Ritz C Skjo dt NM1, Vetha naya ga m D, (2008), "The pulse oxy gen saturation: inspired o xy gen pressure (SpO2:PIO2) diagram: application in the ambulatory assessment of pulmonary vascular disease", Adv Exp Med Biol 605, tr 492-6 Mcgoon M Barst R J., Torbic ki A., Sit bon O ), (200 4), "Diagno sis and differential assessment of pulmonary arterial hy pertension", J Am Coll Ca rdio l 43(12), tr 40S-4 7S Susan F oerster Lisa Bergersen, Audrey C Mars hall, (2009), Congen ital Hea rt Disea se: The Ca theteriz atio n Manua l, Spring Science, New York, U SA Th Thu Tra ng (201 2), Tỡm hiu giỏ tr tiờn lng ca ch s E/Ea trờn bnh nhõn tng ỏp ng mch p hi n hiu bnh tim b m sin h cú lun g thụn g tro ng tim, Lun tt ngh ip Bỏc s Ni trỳ, i hc Y H Ni Silversides C K1 v cỏc cng s (2003), "Pulmonary thrombosis in adults with Eisenmenger sy ndrome", J Am Coll Car diol 42(11), tr 982-7 Par k M K (20 08), Pediatric cardiology f or practitioners, 5, ed, Elsevier Mo sby , London, tr.650-680 Nguyn Th Minh Lý (20 08), Nh n xột c im lõm sng v kt qu th m dũ huyt ng trờn bnh nhõn tim b m sin h cú tng ỏp l c ng mch phi n ng, Lun bỏc s Y khoa, i h c Y H ni Erwin Oechslin v cỏc cng s (2010), "T he Adult Patien t with Eisenmenger Sy ndrome: A Medical Update after Dana Point Part III: Specific Management and Sur gical Aspects", Cur rent Cardiology Reviews 6(4), tr 363-372 Castro Graỗa Baptista Rui, da Silva A ntúnio Marinho, (20 13), "Lo ng-term effect of bosentan in p ulmonary hy pertension associated with complex congenital heart d isease ", Rev Port Card iol 32, tr 12 3-9 Beghetti M Galie N, Gatzoulis MA, et al (2006), "Bo sentan therapy in patients with E isenmenger sy ndrome: a multicenter, double -blind, randomized, placebo-controlled s tudy ", Circulation 14, tr 8-54 Jensen A S Iversen K, Je nsen TV, et al (201 0), " Combinatio n therapy with bosentan and sildenafil in Eisenmenger sy ndrome: a random ized, placebo-controlled, dou ble-blinded trial ", Eur Hear t J 31, tr 124-31 Chemla D Provenc her S1, Hervộ P, et al (2006), "Heart rate responses durin g the 6-minute walk test in pulmonary arterial hy pertension", Eu r Respir J 27(1), tr 114-2 Omar A Minai v cỏc c ng s (201 2), "Heart Rate Recovery Predicts Clinical Worsening in Patien ts with Pulmonary Arterial Hy pertension", Am J Resp ir Crit Care Med 18 5(4), tr 400-40 Nagaya N Miy amoto S1 , Sato h T, et al (2000), "Clinical correlates and progno stic s ignificance of six-minute walk test in patients with primary pulmonary hy pertension Comparison w ith cardiopu lmonary exercise testing", Am J Respir Crit Care Med 2, tr 48 7-92 Beghetti M Berger RM1, Galiố N, (2010), "Atrial septal defects versus ventricular septal defects in BREATHE-5, a placebo-controlled study of pulmonary arterial hy pertension related to Eisenmenger's sy ndrome: a subgroup analy sis", Int J Card iol 44(3), tr 373-8 Rhodes J Buber J1 (2014), "E xercise phy siology and testing in adult patien ts with congenital heart disease Hijazi ZM So mmer RJ1, R hodes JF Jr (2008), "Pathophy siolo gy of congenital heart disease in the adult: part I: Sh unt les ions ", Circula tion 117(8), tr 090-9 Sitbon O Humbert M, Chaouat A, et al, (2006), "Pulmonary arterial hy pertension in France: results from a national registry ", Am J Respir Cr it Care Med 173, tr 1023-30 De Meester P Van De Bruaene A1, Voigt JU, et al (2013), "Worsening in oxy gen saturation and exercise capacity predict adverse outcome in patients with Eisenmenger sy ndrome ", Int J Card iol 68(2), tr 138 6-92 van der Plas MN Reesink HJ1, Verhey N E, et al (200 7), "Six-minute walk distance as parameter of functional outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hy pertension", J Thorac Card iovasc Surg 133(2), tr 10-6 Fatih Ay ik M Gungor H1, Eng in C, Yagdi T, Atay Y, Ozbaran M, Nalba ntgil S, (2014 ), "Transth oracic echocardiographic and cardiopulmonary exercise testing parameters in Eisenmenger's sy ndrome Association with s ix-minute walk tes t dis tance", Herz 39(5), tr 633-7 Paolillo S Sa varese G1, Costanzo P, D 'A more C, (2012), "Do changes of 6-minute walk distance predict clinical events in patien ts with pu lmonary arterial hy pertension? 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