MOT SO NHAN XET VE DIEU TRI PHAU THUAT CAC BENH TIM B A M SINH Of NGUCTI L a N TAI VIEN TIM MACH VIET NAM TCT 2 0 1 1 DEN 2 0 1 5
TOIVITAT
Muc tieu: Nh|n xet ve dac diem benh ly va kinh nghiem phau thu$t cac binh tim bam sitih thi/cfng gap tren ngi^i \&n d '^en Tim mach Viet Nam. Boi t u ' ^ g va pht/tfng phap: ChCing t6i sd dung phu'dng phap hoi cdu, mo ta, phan tfch so li?u tren 549 benh nhan tim bam sinh b ngu'di ttddnq ttianh da di/dc dieu tri bang phu'dng phap phau thuat tai ViSn Tim" m?ch BV Bach Mai trong 5 nam tii 2011 den 2015. K ^ qua va ket lu$n: do tuoi trung binh cDa cac benh nhan ia 32
± 13 tufii, nam gi^i chiem 48%, ni? gi6i chiem 52%.
Cac benh i^ pho bien hay gap nhat la tiiong lien nhT (41,3%), thong lien that (36,6%), tir chiing Fallot (9,2%), con Iai la cac benh ly hiem gap khac. T^ le tu"
von| chu phlu Iii 1,6%. Phu'dng phap dieu tri phau thugt cho cac benh nhan tim bam sinh ngu'cfi idn co ket qua tS^ ty ih tii vong thap, dem lal hieu qua cai thien diat luWig cugc song cho benh nh§n.
Titldida: phau thuat, b|nh tim bam sinh ngu'di ldn.
SUMMARY
RESULT OF SURGICAL MANAGEMENT FOR ADULT CONGENNITAL HEART DISEASE PATIENT I N V I E T N A M HEART I N S T I T U T E
FROM 2 0 1 1 T O 2 0 1 5 Aim: To review some pathological characteristic and surgical experience treatment of adult congennital heart disease patient. Subject: 549 adult congennital heart disease patient in V'etnam Heart Institute during 5 years from 2011 to 2015. Methods: This isretrospective and analyze study. Results and conclusions: 549 adult congennital heart disease had successfully surgery in our centre. The average age was 32 ± 13 years, male was 52 % and female was 48 %. The most common malformation was ASD (41,3%), VSD (36,6%) TOF (9,2%). The mortality rate was low about 1,6%. Conclusion: surgical treatment liar adult congennital heart disease patient had hight successful rate and Improve quality of \\fe far the patient.
Key words :surglcai treatment, adult congennital
I.OATVANDI
B^nh ly tim mach bam sinh cf ngu'Si I6n la mot b?nh ly chiem mot t y le kha Ii3n va co cac net dac truTig rieng biet, d6i hoi nhulig kien thii'c
*Viin Tim m?ch Quoc gia, BV Bach Mai.
Chju trach nhiem chinh: Du'dng Qdc Hung Email: [email protected] NgaynhSn bai: 16.12.2016 Ngay phan bien khoa hoc: 8.2.2017 Ngay duyet bai: 17.2.2017
Dirtrng Dire Hung*
chuyen sau va chien lu'dc chan doan cung nhu"
xu" tri dac t h u [ l ] , [ 2 ] . Oac biet phau thuat d i i u t n cho cac benh nhan nay se co nhieu khac biet so vdi b$nh l i m bam sinh d tre e m [ 3 ] , [ 4 ] . Mo hinh benh ly tim mach bam sinh d Viet Nam co nhiJng net khac biet cc* ban v6i cac nu'dc phat trien tren the gidi. 6 cac nu'dc phat trien phan ldn cac benh nhan tim bam sinh phOte tap du'dc phat hiSn va dieu t n tu" rat sdm. Cac benh nhan tim bam sinh ngu'cfi Idn con ton tai phan Idn la cac benh nhan da du'dc phau thuat s u ^ chiJa nhuYig con de Iai mpt so ton thu'dng ton d u t 5 ] , [ 6 ] . Khac vdi cac nu'dc phat trien co kha nang cham soc su'c khoe ban dau cho nhan dan vdi chat lu'dng cao, d Viet Nam, dieu k i | n ph^t hien va dieu trj sdm cac benh tim bam sinh con nliieu han che, V i vay nhieu benh nhan tim bam sinh ctii du'dc phat hien hOac dieu trj rat muon d tu6i ngu'di ldn hoac trung nlen[7], Vien Tim mach Viet Nam la vien chuyen khoa tim mach tuyen cuoi kham va dieu trj benh tim ngu'di Idn nen co mpt so Idn benh nhan tim bam sinh ngu'di Idn du'dc dieu trj tai day. Vi vay chiing toi tien hanh nphien CLTU nham muc tieu: "Aihan xet vi dac diim benh ly va kinh nghiem phau thuat cac binh tim bam sinh thdc^g gap tren ngu'di Idn d i/ien Tim mach Viit Nam"
II. €)6l TUQWG VA PHUONG PHAP NGHIEN Cl>U _ B6[ tu'dng nghien ciJu ciia chiing toi la tat ca cac iKnh nhan cd chan doan benh Iy tim bam sinh da du'dc phau thuat tai Ddn vj Phau thuat Tim mgch benh vien Bach Mai trong khoang thdi gian td 01/2011 den 12/2015. Cac benh nhan du'dc lay vao nghien CLTU theo trinh t y thdi gian, khong phan biet tuoi, gidi, tinh trang benh ly Iiic nhap vien. Chiing toi sir dung phu'dng phap nghien cu'u hoi ciitj, phan tich mo t a : ghi nhan tat ca cac thong so: dac diem ton thu'dng, tlrih trang ap lyc dpng mach phoi, tien sii* theo doi va phat hien benh, cac thong so lien quan den phau thuat nhu* ky thuat mo, thdi gian nam hoi su'c, ty le bien^ chiJhg, t y le td vong. Quy trinh chan doan va phau thuat ciia chiing toi deu da du'dc thong qua bdi Hpi dong Khoa hoc chuyen mdn cua Benh vien Bach Mai de dani bao tfnh dao du'c cua nghien cu'u. Cac so lieu du'dc xd i<i bang phan mem SPSS 16.0,
VIETNAM MEDICAL JOURNAL N'1 - MARCH - 2017
... . . i s v „ . . i .I^,JI£M /-iVii nho tuoi nhat la 2, ldn nhat la 69 tuoi. Nam gia 111. K f r QUA NGHltN Cl/U ^^„^^ ^. ^^ '^^.^^^
Trong vong 5 "am chung toi da phau hu?t ^^ ^^ ^^^ ^ ^ ^ ^_. ^^^.^
cho 549 benh nhan sm tam ! ^ f • J " ° '™ 3 thdi ky chu phau la 1,6%.
binh trong nhom nghien cuU la 32,02 ± 13,003, •• i i- Benh ly tim bam sinh
l.Thonglien that (TLT):
TLT ndn thuan TUT + hep du'dng ra that phai TLT + hfl van dong mach chu TLT + vB phinh xoang Valsalva
TLT + tiiong iien nhT TLT + hd van hai la TLT + con onq donq mach
2. Thong lien nhi(TLN):
TLN ddn thuan TLN + Hd van hai la TLN the xoang tlnh mach TLN + hep van dong mach phoi TLN + hep van donq mach chu
3.T(f chdnq Fallot 4. Kenh nhl-thSt ban phan (A VSD)
5. Hep eo dong mach chu 6. Hep dddng ra that phai 7. Hep dW'ing ra that trai 8. Con ong dong mach (ODM)
(XM + Viem noi tam mac ODM + Tan huyet sau can thiSp 9. Do dong mach vanh vao buong tim
10. Pliinh xoanq Valsalva ddn thuan ll.Bat thu'dng trd ve tinh mach phdi toan phan
12. Tim 3 buong nhl (Cor tritriatum) 13. Benh ly bat thddng van ba ia (Ebstein) 14. Di tatph&c tap (hep eo dong mach chit
+OBM + TLT) tSng
Nam 117 75
5 13
2 1 70 54 15 1
22 6 10
3 1
1 1 2 2 1 1
230 (42%)
I^Q' 84 8
1 157 133 21 3 2 1 27 21 5 6 3 2 1 2 1 1 1 1 319 (58%)
Tong so (ty le % ) 201 (36,6)
227 (41,3) 187 36
4 2 1 49(9,2)
27 15 9 4 4 3 2 3 2 2 1 549
Sotij' vong
J 3 1 1 'i 1 '
2 1 , 1 1
1
2
9 (1,6%)J
IV. BAN L U A N
Benh nhan tim bam sinh ngu'di Idn du'dc dinh nghTa nhu' la ngu'di cd benh tim bam sinh du ta chu^ dutfc mo suTa chffa hay da tdng du'dc mo nhu'ng da tru'dng thanh den tren 21 tuoi, Tren thut te qua trinh chuyen doi tu* tre con len ngu'di Idn da bat dau tu* nam 12 tuoi.
Phan loai cac hinh thai tim bam sinh gap d ngu'di Idn: Tru'dng hdp benh tim bam sinh nguyen phat d ngu'di Idn bao gom cac BN chu^
tuYig du'dc dieu tn gi ca, 6 nu'dc ta nhdm benh nay chiem t / le cao do nguySn nhan kham va phat hien mupn, khac vdi d cac nu'dc phat trien.
Viec chua du'dc dieu tri cd the den td 3 ly do: 1.
Mdi phat hien du'dc do benh it gay trieu chiJng
hoac khong cd di^u kien kham chan doan, 2. C tat tuy tru'dc day da cd chan doan nhuTig ben!
kha lanh ti'nh, 3. Benh trydc day cho la khong phai thuat du'dc nhuYig bay gid thi cd the. Phan Idii ca benh nhan trong nghien cdu ciia chiing toi tiiuo ve hinh thai chu^ tyng du'dc dieu tn nay.
Tru'dng hdp benh tim bam sinh thii" phat I benh nhanda tyng du'dc dieu tri phau thuat sfl chUa, Ioal nay chiem ty Ie Idn d phu'dng 'Tai Chiing toi cd khong nhieu benh nhan thuc nhom nay.
Cac benh nhan tru'dc mo du'dc danh gia ky v cac trieu chUng cd nang tru'dc kia va hien ta kham ky cac trieu chiiTig thyc the, du'dc chup >
tim phoi va dien tam do. Sieu am Doppier'
TAP CHf Y HOC VigT NAM TAP 452 - THAHG 3 - S 0 1 - 2017 ham do hang dau de khao sat he thong cac iKit
hu'dng giai phau, danh gia mu'c dp anh hu'dng :ua benh len cau true cac buong tim va van tim :ung nhu* sd bp u'dc t'nh mu'c dp nang cua cho lep, idu iddng shunt va tinh trang t§ng ap phoi.
Jhffng tru'dng hdp can danh gia chfnh xac hdn i c thu'dng ton giai phau se du'dc chup cong lu'dng t y (MRI) hoac chup cat Idp dau do da day MSCr)[4].
Thong tim va chup buong tim dutfc chi djnh :ho nhffng tru'dng hdp di tat phdc tap, tang ap 3hoi nang, danh gia sde can va kha nang con Jap lirtig (vasoreacHvity) cua dpng mach phoi, loac khi con nhffng van de ma tham do khong :hay mau cdn chffa chac chan [4]
Tuyet dai da so cac d; tat trong tim dffdc Dhau thueit vdi tuan hoan ngoai cd the, dang ihiet hoac ha the nhiet, phffdng phap bao ve cd tim dung mau (blood cardioplegia).
Tv" le tff vong chung trong nghien cffu ciia diung tdi la 1,6%, cd the so sanh du'dc ty Ie 1,5% trang nghien cffu ciia Putman vdi 830 BN [Yeii td nguy cd chi'nh lien quan den ty le tff TOng cd le la tinh trang tang ap Iffc ddng mach phoi trffdc mo [4],
Benh ly thong lien nhT chiem ty Ie Idn nhat (41,3%) trong nghien cffu ciia chiing toi, giong nhff nhieu tac gia khac. Dieu nay cd the dutfc giai thich nhd dac diem tffdng doi lanh tinh ciia dj tat nay vi phan dong benh nhan khong cd trieu chffng luc cdn be ma van Idn len binh thffdng. Lull Iffdng shunt cua thong lien nhT phu thupc vao chenh ap giffa nhT trai va nhT phai. Khi benh nhan cang nhieu tuoi thi nhffng van de cua tim trai lam giam dp thff gian ciia that trai nhff tang huyet ap, benli mach vanh, benh van hai la... se lam t^ng ap lu'c nhT trai va lam tang Iffu iddng shunt qua thong lien nhT, khi do benh nhan mdi cd bieu.hien trieu chiiYig.
Mpi hinh thai giai phau ciia thong lien nhT gap d tre em deu cd the thay d ngffdi Idn. Chan doan chii yeu dffa vao sieu am Doppler qua thanh ngu'c va thii'c quan de danh gia thffdng ton benh va mffc dp rdi loan sinh ly benh. Chup cong hu'dng tff cd gia trj trong danh^ gia bat thffdng trd ve cua tinh mach phoi d the sinus veinosus.
Thong tim dffdc chi djnh trong trffdng hdp cd tang ap phoi nang (>56% ap Iffc d^i tuan hoan) de danh gia sffc can dpng mach phoi va Iffu Iffdng shunt.
Chi dinh mo hoac can thiep can thiet cho cac benh nhan thong lien nhT ma shunt dii Idn, cd dau hieu tang ganh the t'ch that phai, tuy nhien chffa tang ap Iffc dpng mach phoi cd djnh. Khong
cd chi djnh mo khi benh da den giai dpan tang sffc can phoi co ^nh (Eisenmenger). Vdi trffdng hdp tang ap phoi nang ranh gidi, chi djnh mo hay khong du^ vao Iffu Iffdng shunt, sffc can phoi va kha nang con dap ffng vdi thuoc gian madi [3].
Ve ky thuat mo, cac lo thong ki'ch thffde Idn dffdc ddng bang pateh mang tim hoac vat lieu nhan tao. Mpt sd lo thdng cd gd chac, to chffe khi keo Iai khdng bi cang duWe ddng bang khau dde tiep hai Iffdt. Kgt qua sau mo eua nhdm nay khong khac biet vdi nhdm dutfc ddng bang pateh.
Ty le tff vong cua phau thuat thong lien nhT eua ehung toi dffdi 1%, tffdng dffdng vdi ket qua d cac trung tam khac tren the gidi.
Trai Iai, d nffdc ta, trong nghien cffu ciia chiing toi cung nhff cac nghien cffu khac d Viet Nam, cd rat nhieu benh nhan thong lien that bj
"bd quen" lan dau tSen dffdc phat hien d tuoi trffdng thanh va trung nien, tham chi 50-60 tuoi.
Benh nhan Idn tuoi nhat eua ehiing toi dddc piiau thuat la 61. Khdng ft benh nhan cd lo thong tdi 25mm, tang ap Iffc dpng mach phoi nang mdi dffde phat hien va phau thuat.
Mpt each kinh dien, thong liSn that cd Iffu Iffdng shunt Idn hdn 1,5 (Qp/Qs) mdi cd ehi dinh can tJiiep[3],[4],[5]. Tuy nhien ddi vdi cac thong lien that nhd Qp/Qs dffdi 1,5, gan day quan diem cd xu hffdng md rpng ehi dinh hdn [1], [4], Dieu dd la do trong ky nguyen hien nay, phau thuat thong lien that ngay cang ft bien chutig va tff vong hdn, trong khi dd cae nguy ed IMen chffng va tff vong ciia thong lien that de dien bien tff nhien la khdng the col thffdng dffdc.
Vdi nhffng trffdng hdp tang ap Iffe dpng mach phoi nang rat can ttiong tim danh gia cac chi so lutj Iffdng shunt, ap Iffe cuoi tam trffdng that trai, ap Iffc dpng mach phoi, sffe can dpng mach phoi va kha nang cdn dap ffng vdi thudc gian maeh ciia giu^g dpng mach phoi de quyet dinh xem benh nhan cdn phau thuat dutfc nu^ Idiong, Tuy theo vj tn ciia lo thdng li^n that ma ehiing tdi ddng qua dffdng nhT phai hay md dpng mach phoi.
_Chiing toi cd 2 trffdng hdp tff vong (1%) sau phau thuat thdng lien that, mdt do shock nhiem khuan va mpt do rdi loan dien giai.
Vdi cac benh nhan kenh nhT that ban phan chung toi da phau thuat sffa chUa cho 27 benh nhan. Ranh xe giu^ 2 thanh phan trffdc van nhT that trai gay hd van dffde ddng Iai. Vdng van neu gian nhieu gdp phan lam hd van se dffdc tao hinh thu nhd Iai tren phan vdng van la sau. I^ieng va lo thong lien nhT se di ben trai hoac phai xoang vanh tuy theo vi tri eiia lo xoang vanh.
VIETNAM MEDICAL JOURNAL N't • MARCH • 2017
Chung toi khong ed tff vong quanh^ phau thuat. Cd mot trffdng hdp bj block nhT-th§it phai dat may tao'nhjp vTnh vien. Mot trffdng hdp tien tri^n hd van nhT that trai nang sau 3^nam phai mo Iai thay van nhan tao vdi ket qua tdt.
Mdt loai benh ly hay gap nij^ la tff chffng Fallot, a cac riffdc phat tri^n, cac benh nhan tff chuhg Fallot d ngffdi Idn tuyet dai da so da tffng dffdc phau thuat sffa chffa tff be. D^n tuoi trffdng thanh, cac benh nhan nay cd the^phai xem xet ehuyen mo Iai vi cac van de edn ton tai hoac tien tri^n nhff hep toji dff dffdng ra^ that phai, lid van dpng mach phoi, thdng lien that ton dff, hd van ba la, hd van dpng maeh chii.^, Trong tutfng Iai ch§c ehan phau thuat tim bam sinh
ngffdi ldn d Viet Nam cung se phai doi mat nga cang nhieu vdi nhffng van de tren vi sd Iffdn benh nhan FaIlot4 da dffdc mo sffa khong ngffn tang thSm.
Chiing tdi cd 49 benh nhan, tuoi trung binh I, 26,94, Idn nhat la 42, [an dau tien dffdc phai thuat sffa ehiJa toan bp. Nhffng loat benh nhai Fallot4 dd6c mo sffa lln dau d tuoi ngffdi Idi kieu nhff tlie nay thffdng chi thay trong bao ca(
d cac nffdc phat trien tff thdi nhffng nam 60-7(
the ky trffdc, va ngay nay d eae nffdc dang phi trien nhff Viet Nam [3],[4],[7]. De so sanh, cai sd lieu va ket qua dieu trj ciia cac nghien^ cijt trffdc do ve phau thuat lan dau Fallot4 d ngi/cl idn dddc ehiing tdi tap hdp trong bang 2.
Bang 2: Mdtsdnahien cdu ve phiu thuat ian dSu Faliot4 d ngddi Idn Tac gia
Attenhofer va cs.
Allzadeh va cs.
Atil< va cs.
Lu va cs.
Horer va cs.
Rammohan va cs.
Nguyen Van i^ao va cs.
Chiing tdi
Thdi gian 1970-2007 1995-2005 1982-2001 1990-2004 1974-2003 1991-1996 2007-2010 2011-2015
SoBN 52 51 39 57 52 100 62 49
Tuoi trung binh
50 22 27 25 29 20 21,6
27
.
Ty 1$ tu* vong sdm {%) ,S5,8 '!
2,0 i.
5,1 7,0 A 15,0 •%
4,0 '%
1,9 • 2,0 Nghien CUOJ cua chung toi cung nhu* phan I6n
cac tac gia khac cho thay cac benh nhan Fallot 4 duoc phiu thuat lan dau d tuoi ngu'di idn co ty le tir vong quanh mo thap[3],[4],[5];[6]. Cac nghien ciru danh gia ket qua dieu trj lau dai ciia nhom benh nhan nay cho thay su* cai thien tot tinh trang cd nang va chat lu'dng cuoc song, mac dil tuoi tho nhom nay khong bang khoi dSn so chung va nhom Fallot4 du'dc sio chuS sdm tir nho [3].
Vdi cac Vl do tren, dac biet la ty le tif vong thap trong nghien diu ciia ciiiing toi va cac tac gia khac, chiing ta van can chi djnh phau thuat suS &iia cho cac benh nhan Fallot 4 ngudi Idn 6 Viet Nam, cho dii phat hien 6 glal doan mupn.
V. KFT LUAN
Tren 500 BN benh tim bam sinh ngUSi ldn da du'dc dieu tri phau thuat tai Vien tim mach - Benh Vien Bach l«lai trong 5 nam qua vdi kgt qua tot va ty le tiT vong thap. Phiu thuat da dem Iai l;li (ch cho BN nham cai thien trieu chihig, nang cao chat Wdngi^cuoc song, phong ngim cac bien chu'ng nguy hiem.
TA! LlfU THAIVI KHAO
1. Kirklin/Barratt - Boyes - Cardiac Sur^ery^
Fourtli edition (2013) -Congenital Heart Diss^
in the Adult.
2. H Baumgartner (2010) "ESC Guidelines Ibr till management of grown-up congenital heart dis^sf (new version 2010):European Heart Joumai'il, 291S-2957
3. Karamalou T et al (2010) "Adult or big kids what is the ideal environment for management o grown-up patient with congenital heart disease^
Ann TfioracSui^, Au0O{2):S73-9. iMU 4. Atik Fernando A., Atik Edmar, Cunha Giaii^w
et ai.(2004) , 'Long-term results of c d r ^ 3 | tetralogy of Fallot in adulthood", European jB^^
Cardia-thoracSurgery, 25, pp. 250-255. ' ^ 5. Rammohan M., Airan Balram, Bhan Anil e t i
(1998)., "Total correction of tetralogy of FallotJ adults-surgical experience", Intemationai Joiim ofCardioiogyi'i.pp, 121-128, 'Ja 6. Kogon BE, Plattner C (2009) " Adult congfnlB
heart surgery :adult or pediatric facility? Adult o peditrlc surgeon?" Ann Thorac Surg :2009fl8l
87(3)j833-40. J B 7. Nguyin Van Mao; Binh Tien DDngiSi
Quang Vinh et ai.(2011):"Nh?n xet diftB phau thuat sira chua triet de tir chuTig F a l ^ ngua ldn" -Tap cht Hoi PhSu thuat Tm'W.
&l6ng ngi/c Vtet Nam sdlQI2.<lll P67-69. ! •
