DONG MACH VANH DUY NHAT TRONG PHAU THUAT
DIEU TRj BENH CHUYEN GOC DONG MACH HOAN TOAN 0 TRE SO SINH:
KINH NGHIEM 3 TRUTJNG HOP TAI BENH VIEN NHI TRUNG DONG Nguyln Ly Thinh Trirdng*, Dang Hanh Tiep**, Hoang Thanh Son*,
Tran Minh Dien***, Pham Hfru Hoa****, Nguyen Thanh Hai****, Cao Viet Tung****, Nguyen Thj Thu Ha**, Nguyen Thj Phirong Anh**, Nguyen Thanh Cong*, Nguyen Thanh Liem*.
I. OAT VAN OE
Phau thuat (lieu tri benh chuyen goc dong mach d tre so sinh ddi vdi nhung trudng hop chi cd mot ddng mach vanh duy nhat van la mot thach thirc va la yeu td nguy ca trong tien luang tu vong [5]. Da cd nhieu cai tien ve ky thuat trdng lai ddng mach vanh dugc dua ra nham nang cao kha nang chuyen ddng mach vanh thanh cdng trong qua trinh phau thuat [9],[10]. Trong nam 2009 chung tdi da tien hanh phau thuat cho 3 trudng hgp tre so sinh dugc chan doan chuyen gdc ddng mach hoan toan. Chan doan mdt ddng mach vanh duy nhat dugc tien hanh trong qua trinh phau thuat.
II. BENH NHAN VA PHUONG PHAP:
Tir thang 11 nam 2009 tdi thang 2 nam 2010, cd 5 trirdng hgp chuyen gdc ddng mach dugc tien hanh phau thuat sira chua toan bd tai Benh vien Nhi Trung Uong, trong dd cd 3 trudng hgp dugc chan doan mot ddng mach vanh duy nhat.
Tat ca cac benh nhan dugc chan doan chuyen gdc ddng mach hoan toan cd chi dinh phau thuat Jatene deu dugc chung tdi tien
* Khoa Ngoai - Benh vien Nhi Trung Uong
* * Khoa GMHS - Benh vien Nhi Trung Uong
*** Khoa Hdi sire Ngoai - Benh vien Nhi Trung Uong
**** Khoa Tim Mach - Benh vien Nhi Trung Uong
hanh phau thuat theo quy trinh thdng nhat chay may tim phdi nhan tao ha than nhiet muc dd trung binh 25°C - 28°C. Sau khi tien hanh ngirng tim vdi dung dich Custadiol, hai ddng mach chu va ddng mach phdi dugc cat rdi, thu thuat Lecompte dugc tien hanh nham chuyen ddng mach chii ra phia sau ddng mach phdi, cac ddng mach vanh dugc cat rdi khdi ddng mach chu (ddng mach phdi mdi) vdi cue ao day du va dugc trdng lai vao ddng mach phdi (ddng mach chu mdi).
Ky thuat trdng lai ddng mach vanh dugc chiing tdi su dung trong 3 trudng hgp tren la ky thuat "cua lat" [4],[9], Phan md khuyet tren ddng mach phdi mdi dugc thay the bang mang tim tuai, sau dd ddng mach chii va ddng mach phdi dugc ndi lai vdi nhau. Cac thuong tdn phdi hgp trong tim dugc tien hanh sua chua trudc hoac sau khi chuyen ddng mach vanh tuy theo tirng trudng hop cu the. Sau khi tha clamp ddng mach chii, tim dugc chay hd trg va benh nhan dugc tien hanh cai may tim phdi nhan tao.
Benh nhan thu nhat 30 ngay tudi, can nang 4kg, vao vien trong tinh trang suy tim sung huyet, tim nhe toan than. Tre dugc tien
hanh hdi siic trong vong 2 ngay va hoan thanh cac xet nghiem chan doan. Sieu am tim phat hien ton thuang chuyen goc dong mach, tuong quan hai dai dong mach trudc - sau, thong lien thlt quanh mang dudng kinh 6mm, dng dpng mach Idn shunt 2 chieu vdi dudng kinh 6 - 8mm, Id bau due md 4mm shunt phai - trai la chu yeu. Trong qua trinh phau thuat chiing tdi phat hien benh nhan cd mdt dpng mach vanh duy nhat xuat phat tu xoang Valsava ben phai (Hinh 1). Ddng mach vanh trai chay vong phia sau ddng mach phdi va chia thanh 2 nhanh dpng mach mu va ddng mach lien that trudc, ddng mach vanh phai theo dudng di binh thudng theo kieu (2R, AD, Cx) theo phan loai Leyden [5],[8], Cue ao chung ciia dpng mach vanh duy nhat dupe cat rdi va phau tich giai phdng du rdng de khi trdng lai vao ddng mach chu mdi khdng bi cang hay xoan van. Ld thdng lien that dudi van dpng mach chu dupe tien hanh va qua dudng van dpng mach chii sau khi cue ao ddng mach vanh da dupe giai phdng. Ld bau due dupe de md. Benh nhan sau phau thuat dupe tien hanh cai may tim phdi nhan tao khdng cd bat cu van de gi ve huyet ddng va dupe ddng xuong uc ngay sau md. Thdi gian benh nhan nam hoi sire la 4 ngay, tre ra vien vao ngay thu 12 sau phau thuat. Tai kham sau md cho thay van ddng ciia cac thanh tim hoan toan binh thudng, chuc nang tim tot vdi EF > 60%, khdng cd shunt tdn luu qua thdng lien that, khdng cd hep phdi, khdng cd hd van ddng mach chu.
Benh nhan thir hai la mdt tre nam 5 ngay tudi, can nang 3.6 kg, vao vien vdi chan doan benh tim bam sinh tim sdm. Benh nhan dupe sieu am tim va chan doan mac benh chuyen gdc dpng mach hoan toan - thdng lien
that Idn - cdn dng ddng mach, tuang quan hai dai ddng mach trudc - sau, kich thudc cua dpng mach phdi gin tuang duang vdi dpng mach chii. Qua trinh phiu thuat phat hien thay benh nhan cd hai lo mach vanh nam sat canh nhau kieu "ndng sung san", hai ld dpng mach vanh nam dudi mep van td chim giira la vanh phai va la vanh trai. Tir ld ddng mach vanh nam phia ben phai tach ra dpng mach vanh phai va dpng mach vanh trai. Ddng mach vanh trai chay vong phia sau dpng mach phdi va chia thanh hai ddng mach mii va dpng mach lien that trudc. Tu ld ddng mach vanh nam phia ben trai mdt nhanh ddng mach vanh nhd chay trong thanh ciia dpng mach chu kieu intramural, cap mau cho phan pheu ciia that phai. Chung tdi tien hanh tach rdi mep cua hai la van "td chim" khdi thanh ddng mach chu, rdi tien hanh cat rdi cue ao chung cua ca hai ddng mach vanh sau dd tien hanh chia ddi cue ao chung cd chua hai ld dpng mach vanh thanh hai cue ao rieng biet. Sau dd tren cue ao ddng mach vanh ben trai cd hinh thai intramural dupe chung tdi tien hanh md rdng ld vao ciia ddng mach vanh tdi sat nguyen uy cua ddng mach nay (Hinh 2). Hai cue ao ddng mach vanh dupe giai phdng rpng rai va trdng lai vao ddng mach chii mdi. Cac thucmg tdn phdi hpp dupe tien hanh sira chua ciing mdt thi. Sau khi tha clamp ddng mach chu, chung tdi phat hien chay mau tai vi tri cua cue ao ddng mach vanh nam ben trai. Benh nhan dupe tien hanh ngimg tim trd lai va mieng ndi ciia ddng mach vanh nam ben trai duac lam lai.
Sau khi tha cap ddng mach chu benh nhan dupe tien hanh cai may tim phdi nhan tao khdng cd gi khd khan, khdng cd chay mau sau md. Benh nhan dupe de hd xuong uc sau
mo va chuyen ra hoi siic vai cac lieu thuoc van mach a muc toi thieu. Sau 36h sau mo, benh nhan dugc dong xuong ire va tien hanh cai va rut may tha sau 72h sau mo. Thai gian nam hoi sire sau mo la 10 ngay va tre ra vien
vao ngay thu 117 sau phau thuat. Tai kham khong phat hien bat cir vein de nao nghiem trong. chuc nang tim tot, EF > 60%, khong co roi loan van dong thanh that, khong hep duong ra that phai.
Hinh 1: Dong mach vanh duy nhat xuat phat tir xoang Valsava phai
Hinh 2: Chia tach dia chung dpng mach vanh thanh 2 cue ao dong mach vanh rieng biet.
Benh nhan con lai la mot truong hop tre nam 12 ngay tudi, 3.2 kg, vao vien vi kho tha va tim tai. Sieu am chan doan phat hien chuyen goc dong mach - con ong dong mach - 16 bau due han che. Benh nhan dugc tien hanh pha vach lien nhi bang bong va hoi sue noi khoa. Phau thuat dugc tien hanh luc tre dugc 21 ngay tuoi. Trong mo phat hien bat thuang mot dong mach vanh, dong mach vanh duy nhat xuat phat tir xoang Valsava ben phai, dong mach vanh trai chay vong phia sau dong mach phoi chia thanh 2 nhanh mu va lien that trudc kieu (2R,AD,Cx) theo phan loai Leyden [5],[8], Cue ao dpng mach vanh dugc giai phong rpng rai va trong lai vao dpng mach chii mai, phan vach lien nhT dugc va lai bang mieng mang tim tuai. Sau phau thuat benh nhan dugc de ha xuong ire trong vong 48h. Sau khi dong xuong uc, benh nhan dugc cai va rut may tha vao ngay thu 5 sau phau thuat. Sieu am sau mo cho thay chuc nang tim va van dpng thanh that tot, khong hep duong ra that phai, van dpng mach chu ha rat nhe. Benh nhan ra vien sau 35 ngay sau phau thuat.
III. BAN LUAN
Chuyen goc dpng mach kem theo bat thuang mach vanh duy nhat van la mot thach thuc ve mat ky thuat va la mot trong cac yeu td nguy ca tien luang tir vong trong qua trinh phau thuat [2],[5]. Ngoai ra, mdt sd nghien cuu ve cac yeu td tien lugng va nguy ca tu vong hien nay cho thay yeu td thdng lien that, can nang thap, thdi gian cap ddng mach chii dai, va xuong ire de hd sau phau thuat la nhirng yeu td nguy co dpc lap cd lien quan mat thiet tdi ty le tir vong sau phau thuat [2],[6],[7],[8].
Tai Benh vien Nhi Trung Uong, phau thuat sua chua chuyen gdc ddng mach d tre so sinh da tien hanh tu nam 2009 va van tiep tuc trong qua trinh hgc hdi va nang cao kinh nghiem. Ddi vdi phiu thuat chuyen gdc, budc quan trgng nhat la trdng lai dpng mach vanh. Cd nhieu ky thuat trong lai dpng mach vanh duy nhat vao ddng mach chii mdi da dugc md ta vdi nhirng ket qua khac nhau.
Parry va cdng su md ta ky thuat "mang tim phu" nham duy tri hinh thai hoc cua ddng mach vanh va nhan manh sir duy tri hinh thai hgc cua dpng mach vanh sau khi chuyen vi tri cd anh hudng quyet dinh tdi ket qua lau dai sau phau thuat [4], Hp da thanh cdng khi sir dung ky thuat nay cho 4 trong 5 benh nhan phai tien hanh sua lai ddng mach vanh.
Tuy nhien vdi nguy ca canxi hda cua mang tim, nguy ca hinh thanh huyet khdi trong xoang mach vanh mdi, va nguy ca xoang dpng mach vanh mdi bi de ep bdi ddng mach phoi mdi van la nhirng lo ngai cdn tdn tai khi tien hanh ky thuat nay. Mot nhdm tac gia tir An Dp ciing sir dung ky thuat chuyen ddng mach vanh tai chd nham tranh nguy co xoah van ddng mach vanh khi tien hanh chuyen ddng mach vanh [3]. Tuy ket qua ngan han va trung han cho thay khdng cd hep dudng ra cua hai that hoac hd van ddng mach chu hay ddng mach phdi kem theo tudi mau mach vanh dn dinh, van tdn tai nhirng nghi ngai ve anh hudng lau dai ciia ky thuat nay ddi vdi hinh thai ciia dudng ra that cd the gay hd van ddng mach chii sau md va hep dudng ra that phai. Vdi kinh nghiem cdn han che ciia chiing tdi, ca 3 trudng hgp ddng mach vanh duy nhat deu cd the tien hanh chuyen vi tri ma khdng gap phai bat ky van de khd khan nao. Ky thuat "cira lat" dugc chung tdi ap
dung cho ca 3 truong hop tren trong qua trinh trong lai dpng mach vanh vao dpng mach chu mai. Ket qua ngin han cho thay tuai mau mach vanh hoan toan on dinh va khong co bat cu ghi nhan nao ve kha nang hep duong ra ciia that. Doi vai truong hpp co ha van dpng mach chii nhe sau mo, sieu am tim trudc phau thuat ciing da ghi nhan co ha nhe van dpng mach phoi (van dpng mach chii mai) trudc khi tien hanh phau thuat.
Chuyen vi dpng mach vanh ciia dpng mach vanh duy nhat vai hai 16 mach vanh rieng biet dupe ghi nhan la phuc tap han nhieu so vai chuyen vi dpng mach vanh binh thuang. Ve ca ban co hai ky thuat dupe mo ta va ap dung doi vdi trudng hop nay. Mdt ky thuat duy tri hinh thai ciia ddng mach vanh va tudi mau ciia ddng mach vanh qua mdt xoang cd phii mang tim sat canh dpng mach chii mdi. Ky thuat cdn lai cd gang chia tach dpng mach vanh thanh 2 ciic ao vanh rieng biet. Mdi mot ky thuat bao ham trong nd nhirng uu diem va nhupc diem rieng biet.
Ddi vdi trudng hpp thir 2 ciia chiing tdi, ky thuat chia ddi ddng mach vanh chung thanh 2 cue ao rieng biet da dupe ap dung thanh cdng. Tuy nhien sau khi chuyen vi dpng mach vanh, cue ao ciia dpng mach vanh trai bi rach ddi hdi mieng ndi dpng mach vanh phai lam lai, chiing tdi quyet dinh lam ngimg tim trd lai, mieng ndi dpng mach vanh ben trai dupe thao rdi ra va lam lai. Benh nhan sau phau thuat khdng gap phai van de ve chay mau, tuy nhien chiing tdi bat budc phai de hd xuong ire cho benh nhan do tinh trang phii ne ca tim sau md. Sau phau thuat 36h benh nhan dupe ddng xuong uc ma khdng cd bat cir anh hudng nao tdi huyet dpng. Tir kinh nghiem ddi vdi trudng hpp nay, chiing
tdi thay rang khdng nen do dir ma nen tien hanh kiem tra va neu can thiet thi phai tien hanh ngimg tim va lam lai cac mieng ndi trong bat ky trudng hpp nao cd tinh trang chay mau khd kiem soat sau md ddi vdi benh nhan chuyen gdc dpng mach so sinh.
De ket luan, ket qua budc dau dieu tri nhung trudng hpp chuyen gdc ddng mach hoan toan cd mot ddng mach vanh duy nhat tai Benh vien Nhi Trung (Jong da dat dupe la kha quan vdi ky thuat "cira lat". Mdt nghien ciru trong tuang lai vdi sd lupng benh nhan nhieu hon, thdi gian theo ddi dai hon la rat can thiet de danh gia chinh xac hieu qua ciia dieu tri phau thuat ddi vdi hinh thai tim bam sinh phuc tap nay.
TAI LIEU THAM KHAO
1. Chang YH, Sung SC, Lee HD, Kim S, Woo JS, Lee YS. Coronary reimplantation after neoaortic reconstruction can yield better result in arterial switch operation:
comparison with open trap door technique.
Ann Thorac Surg 2005;80:1634-1640.
2. Daebritz SH, Nollert G, Sachweh JS, Engelhardt W, von Bernuth G, Messmer BJ.
Anatomical risk factors for mortality and cardiac morbidity after arterial switch operation. Ann Thorac Surg 2000;69:1880-1886.
3. Murthy KS, Coelho R, Kulkarni S, Ninan B, Cherian KM. Arterial switch operation with in situ coronary reallocation for transposition of great arteries with single coronary artery Eur J Cardiothorac Surg 2004;25:246-249.
4. Parry AJ, Thurm M, Hanley FL. The use of 'pericardial hoods' for maintaining exact coronary geometry in the arterial switch
operation with complex coronary anatomy.
Eur J Cardiothorac Surg 1999;15:159-165.
5. Pasquali SK, Hasselblad V, Li JS, Kong DF, Sanders SP. Coronary artery pattern and outcome of arterial switch operation for transposition of the great arteries: a meta- analysis Circulation 2002;106:2575-2580.
6. Qamar ZA, Goldberg CS, Devaney EJ, Bove EL, and Ohye RG. Current Risk Factors and Outcomes for the Arterial Switch Operation.
Ann Thorac Surg 2007;84(3):871-879.
7. Sarris GE, Chatzis AC, Giannopoulos NM, et al. The arterial switch operation in Europe for transposition of the great arteries:
a multi-institutional study from the European Congenital Heart Surgeons Association. J
Thorac Cardiovasc Surg 2006;132:633-639.
8. Scheule AM, Zurakowski D, Blume ED, et al. Arterial switch operation with a single coronary artery. J Thorac Cardiovasc Surg 2002;123:1164-1172.
9. Shukla V, Freedom RM, Black, MD Single coronary artery and complete transposition of the great arteries: A technical challenge resolved? Ann Thorac Surg 2000;69:568-571.
10. Si Chan Sung, Yun Hee Chang, Hyoung Doo Lee, Siho Kim, Jong Soo Woo, and Young Seok Lee. Arterial Switch Operation for Transposition of the Great Arteries With Coronary Arteries From a Single Aortic Sinus. Ann Thorac Surg 2005;80:636-641.
