NGOAI KHOA - SO 2/2011
PHAU THUAT 1 THI SLfA TOAN BO BENH LY CHUYEN GOC DONG MACH - GIAN DOAN QUAI DONG MACH CHO:
NHAN 1 TRLfONG HOP
Nguyen Ly Thjnh Trudng*, Nguyin Tuan Mai*, Nguyin Dinh Chien**, Nguyin Thi Phuang Anh**, Nguyin Thj Thu Ha **, Nguyin Thanh Liem*
TOM TAT
Phau thuat 1 thi sira toan bg cac thUdng ton trong va ngoai tim ddi vdi mgt trUdng hdp chan doan chuyen gdc dgng mach-gian doan quai dgng mach chii tip A-thdng lien that da du'gc tien hanh tai benh vien Nhi Tmng L/dng vdi ket qua tdt.
ABSTRACT:
Report of one case complex congenital heart disease with transposition of the great arteries- interrupted aortic arch type A-ventricular septal defect underwent single stage total correction in National Hospital of Pediatrics Hanoi, Viet Nam with good short-term result.
I. DAT VAN DE:
Benh ly chuyin gdc ddng mach cd kem theo thdng lien thdt (TGA-VSD) cd mgt ty le nhat dinh phdi hgp vdi thuang tdn hep eo ddng mach chu [1]. Nhumg trudng hgp chuyen goc ddng mach phdi hgp vdi gian doan quai dgng m^ch chu la benh ly hilm gap. 1 trudng hgp dugc chan doan chuyen gdc dgng mach, thdng lien that va gian doan quai dgng mach chii tip I da dugc tien hanh phau thuat sua toan bd trong 1 thi thanh cdng tai Benh vien Nhi Trung LTomg.
* Khoa Ngo^i - B$nh vi$n Nhi Tnmg uvng
* * Khoa GMHS - B$nh vi$n Nhi Tnmg WOng
I I . BENH NHAN VA P H U O N G PHAP:
Benh nhan la tre gai 1 thang tudi, nhap vien trong tinh trang suy tim sung huylt kem theo suy hd hap. Tre dugc tien hanh ho trg hd hap vdi may thd va dugc diing cac thudc trg tim va loi tieu kem theo. Sieu am tim phat hien benh ly chuyen gdc ddng mach, thdng lien that quanh mang Idn (dudng kinh 8mm) kem theo gian doan quai ddng mach chu tip I, nii'a dudi co the dugc tudi mau qua ong dgng mach rat Idn (dudng kinh 6mm). Thdng tim chan doan dugc tien hanh xac dinh chdn doan tuong tu ket qua sieu am, ddng thdi xac dinh khdng cd tinh trang hep dudng ra thdt trai.
Sau 10 ngay dieu trj hdi siic, benh nhan dugc tien hanh phau thuat sira toan bd. Toan bg cac thuong tdn dugc bdc Id qua dudng giiia xuong uc. Ddng mach chu xuat phat tir that phai va dgng mach phdi xuat phat tir thdt trai, dgng mach chu nam phia trudc va ben phai so vdi dgng mach phdi. Ddng mach vanh phai bat ngudn tir xoang Valsava ben phai, ddng mach vanh trai xuat phat tir xoang Valsava trai chia thanh 2 nhanh mu va lien that trudc, tuomg irng vdi phan loai 1 LCx- 2 R theo phan loai giai phau ddng mach vanh
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NGOAI KHOA - SO 2/2011
trong chuyin gdc ddng mach ciia Leiden [2].
Dudng kinh ciia dgng mach phdi Idn gdp 2 lan so vdi ddng mach chu. Ddng mach chu len, quai ddng mach chu, cac nhanh than tay ddu, dgng mach canh gdc trai va dudi ddn trai, eo ddng mach chu va phdn ddu cua dgng mach chu xudng dugc giai phdng rdng rai (Hinh 1). Tudn hoan ngoai ca thi dugc tiln hanh vdi hai canuyl dgng mach chii va hai canuyl tinh mach. Mdt canuyl dgng mach
Hinh 1: Chuyen gdc ddng mach - gian doan quai dgng mach chu chu tudi mau qua than tay dau phai thdng qua mgt doan Gore- tex 3.5, canuyl dgng mach chu cdn lai tudi mau
cho niia dudi ca the bang each dat true tiep vao dng ddng mach (Hinh 2).
i
Hinh 2: Ky thuat tudi mau chgn lgc qua dng Gore-tex va qua PDA
Benh nhan dugc tien hanh ha than nhiet xudng 20 do C. Khi bat dau tiln hanh chay may tim phdi nhan tao, dng ddng mach dUgc that va trong qua trinh ha nhiet do, doan ddng mach chu xudng dugc giai phdng rgng rai, thdn kinh thanh quan quat ngugc dugc baio tdn. Chiing tdi tien hanh tao hinh quai va eo ddng mach chu bang ky thuat cat ndi tan-tan md rgng va toan bg doan dau ciia dgng mach chu xudng, quai ddng mach chu va ddng mach chii len dugc md rdng bang mieng mang tim tu than cd xvr ly Gluteraldehyd 0.6%) trdng khi tim vin dugc tudi mau nham muc dich dat dugc kich thudc ciia dgng mach chii tuong duong vdi ddng mach phdi (Hinh 3).
t".
Hinh 3: Hoan tat tao hinh toan bd ddng mach chu bang mang tim
Khi qua trinh tao hinh ddng mach chu hoan tat, dung dich liet tim dugc bam xudi ddng. Phau thuat Switch dugc tien hanh vdi ky thuat chuyen dgng mach vanh "trap door".
Id thdng lien that phan quanh mang do thuang tdn lech hang ve phia sau cua vach ndn dugc va bang mieng va mang tim vdi chi Prolene 6.0 miii rdi. Khi qua trinh sura chiia trong tim hoan tat, clamp ddng mach chu dugc tha, tim dugc tudi mau deu, khdng cd hien tugng thieu mau ca tim tren lam sang va tren dien tam do (Hinh 4).
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Hinh 4: Phiu thuat Switch
Qua trinh cai may tim phoi nhan tao dien ra thuan Igi, khdng cd biln chung. Benh nhan dugc de md xuomg iic va xuomg lic dugc ddng tai khoa hdi siic sau md vad ngay thir 2 sau md. Sau 5 ngay thd may, benh nhan dugc cai may thd va ra vien sau 2 tudn vdi ket qua sieu am sau md khdng cd hien tugng h?p eo ddng mach chu, chenh ap qua eo ddng mach chu la 15mmHg, chiic nang tim tdt vdi EF>60%, van ddng cac thanh tim binh thudng, khde to, cac phan xa cua tudi nhii nhi binh thudng.
I I I . BAN LUAN:
Thuong tdn gian doan quai dgng mach chu trong benh ly chuyen gdc dgng mach la ton thuang hiem gap. Phiu thuat sira toan bg thuang ton cd the tiln hanh trong 1 thi mo hoac qua nhieu thi, tuy theo miic do thuang tdn trong tim va tuy thudc chien luge dieu tri cua timg trung tam [3,4,5,6]. Hien nay, xu hudng tien hanh phau thuat sua toan bg cac thuang tdn trong tim va ngoai tim phdi hgp trong ciing 1 thi md la phd bien tren the gidi vdi sy phat trien cua ky thuat tudi mau nao chgn lgc [3,4]. Tai Benh vien Nhi Trung l/cmg, chiing tdi da tien hanh phau thuat sira chijra cac thuang tdn cua quai-eo ddng mach chu phoi hgp vdi cac thuang tdn trong tim
trong ciing mdt thi mo vdi ket qua ban dau tuong ddi kha quan.
Trong benh ly gian doan quai ddng mach chu kem theo chuyin goc ddng mach va thdng lien thdt, phiu thuat sira chira mdt thi da dugc chung td cd nhilu Igi diem hon han so vdi phiu thuat nhilu thi [4]. Thii nhat la phau thuat mgt thi cd kha nang tien hanh sira chiia tat ca cac thuong tdn trong cung 1 thi md, tranh dugc giai doan chuyen tiep giira hai cugc md, giup cho qua trinh hdi sire de dang han so vdi khi phai ddi mat vdi tinh trang tre sau phau thuat tam thdi van cdn cac thuong tdn trong tim kem theo. Ddi vdi benh ly gian doan quai dgng mach chu, vdi tinh trang thieu san quai dgng mach chii la rd rang, phau thuat tam thdi qua dudng nguc ben la rat khd de cd the can thiep va giai quyet toan bg thuong tdn ciia quai dong mach chu. Dac biet hon, trong benh ly chuyen goc dong mach kem theo thdng lien that va gian doan quai dgng mach chii, su khac biet ve kich thudc giira hai dgng mach chu va dgng mach phdi la rat rd ret vdi dudng kinh ciia dgng mach phdi thudng gap 2 hoac gap 3 lan so vdi ddng mach chu. Phiu thuat 1 thi cho phep tien hanh tao hinh quai dgng mach chii, ddng thdi cd the md rdng toan bg ddng mach chu, ke tir ddng mach chu xudng, quai dgng mach chu, va ddng mach chu len, tranh hien tugng tai hep sau md.
Ddng thdi khi dgng mach chu len dugc md rgng, su khac biet ve kich thudc giiia ddng mach chii va dgng mach phdi la khdng dang kl, giiip cho qua trinh tao hinh va trdng lai dgng mach chii mdi de dang hon nhieu [4].
Ngoai ra, phiu thuat mgt thi chi cd 1 dudng md duy nhdt, tranh dugc nguy co xo ciing ngirc vdi dudng md nguc trai sau ben. Benh
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NGOAI KHOA - SO 2/2011
nhan cua chiing tdi dugc tiln hanh tao hinh toan bd tir dgng nriach chii xudng, quai ddng mach chu va dgng mach chu len vdi milng mang tim da qua xu ly Gluteraldehid 0.6%, giai phdng toan bd tdn thuang hep eo va dl dang xur ly su chenh lech vl kich thudc giiia ddng mach chu va ddng mach phdi, tao dilu kien dl dang thuc hien phiu thuat Switch.
Ky thuat tudi mau nao chgn lgc trong phiu thuat sira chiira thuang tdn hep. eo ddng mach chu hoac gian doan quai dgng mach chii phdi hgp vdi sira chiia cac tdn thuong trong tim da dugc ap dung tai nhilu trung tam tim mach tren the gidi. Mdt sd trung tam sir dung canuyl true tilp vao than ddng mach canh tay-dau. Sd cdn lai ap dung ky thuat canuyl gian tiep qua mgt doan Gore-tex dugc noi vdi than canh tay-dau [3,4]. Vdi. nhiing uu diem rd ret nhu tranh dugc kha nang tdn thuang true tiep cd kha nang gay tac mach do hinh thanh cue mau ddng hay su cd san vat lieu Gore-tex, chiing tdi thay viec ap dung ky thuat tudi mau nao chgn lgc qua dng Gore- tex la phu hgp vdi dilu kien hien tai cua benh vien Nhi Trung LTong. Canuyl ddng mach chu cdn lai dugc dat true tiep vao dng dgng mach, von cd kich thudc rdt ldm trong benh ly nay, ma khdng gap bdt cii van de nao trong qua tfnh tudi mau va ha than nhiet cho niia dudi CO thi. Trong trudng hgp kich thudc dng dgng mach khdng phu hgp de canuyl true tilp, chiing tdi khdng do du tiln hanh canuyl true tilp vao dgng mach chu xudng d ngang ca hoanh nhdm tudi mau va ha than nhiet dgng thi cho toan bg ca thi.
D I thay kit luan, qua trudng hgp lam sang ndi tren, ky thuat tudi mau nao chgn lgc trong phiu thuat sua toan bg thuang tdn gian
doan quai ddng mach chu phdi hgp vdi chuyen gdc ddng mach la hoan toan kha thi trong dilu kien cua Benh vien Nhi Trung LTong. cdn mdt nghien cuu toan dien vdi sd lugng benh nhan nhilu horn va thdi gian theo ddi sau phiu thiiat dai horn nhdm danh gia chinh xac va khach quan han niia ket qua phau thuat.
TAI LIEU THAM KHAO
1. Mitchell S C, Korones S B, and Berendes H W. Congenital Heart Disease in 56,109 Births Incidence and Natural History.
Circulation 1971,43:323-332
2. Gittenburger de Groot A, Sauer U, et al.
Coronary arterial anatomy in transposition of the great arteries; a morphologic study.
Pediatr Cardiol 1983;4(Suppl 1): 15-24.
3. Lim C, Kim WH, Kim SC, et al. Aortic arch reconstruction using regional perfusion without circulatory arrest Eur J Cardiothorac Surg 2003;23:149-155.
4. B. Alsoufi, S. Cai, W. G. Williams, et al.
Improved results with single-stage total correction of Taussig-Bing anomaly. Eur J Cardiothorac Surg 2008; 33(2): 244 - 250.
5. M. Griselli, S. P. McGuirk, C.-S. Ko, et ah Arterial switch operation in patients with Taussig-Bing anomaly influence of staged repair and coronary anatomy on outcome.
Eur J Cardiothorac Surg 2007; 31(2): 229 235.
6. M. Hakimi, S. K. Clapp, H. L. Walters III, et al. Arch Growth After Staged Repair of Interrupted Aortic Arch Using Carotid Artery Interposition. Ann. Thorac. Surg., August 1, 1997; 64(2): 503 - 507.
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