Y H p c T P . H b C h i M i n h * P h u B a n T a p 19 * S o 5 * 2 0 1 5 N g h i e n c u u Y h p c
BAO CAO MQT CA: PHINH NHI PHAI BAM SINH
Nguyen Do Trong*, Cliiu Kin iTdu*, lid Vdn Anh Dung*, Ton Tlii Anh Tu*
TOMTAT
Tre trai 7 tudi dugc chuyen viin vd nhgp khoa tim mach vi suy ho hap, phdt hien bong tim rdi to tren X Qtwng plioi thdng vd nhi plidi phinh to tren sieu am tim. Binh nlu sau do dugc pltdu thugt cdt nho bdt nha phdi dudi chgy tuan hodn ngodi ca the, nhdm ngdn ngita biai chihtg thuyin tdc phdi vd hg tliiqj nguy ca rot logn nhjp idti.
KM qud mo hoc dw thay thdnli nhi dan mong nhu ta^m/, teo vd thodi hoa kem tdng sinh mo sgi Idnli thii.
Tit kltoa: Phinh dgi nhi phdi, siiu am tim, thuyin tac, roi logn nhip tim.
A B S T T ^ C T
IDIOPATHIC GL^NTRIGHT ANEUSYSUM, A CASE REPORT
N g u y e n D o Trpng, C h i u Kin H a u , H o Van A n h D u n g , Ton Thi A n h T u
* Y H o c TP. H o Chi M i n h * S u p p l e m e n t of Vol. 19 - N o 5 - 2015:185 - 188 A 7- year- old boy was sait to Cardiology departmait zoith symptom of shortness of breath, fiirther investigations slwioed a massive cardiomegaly finding on diest X-ray and a giant right atrial aneurysm on edwcardiography. The patiait thai underwait surgical resection of the right atnum under cardiopulmonaiy bypass to prevent thromboembolic complications and to lower the risk of atrial arrthymias
Tlie histology result was paper-thin wall atrium, focal endocardia fibrosis consistent and dcgena-ation Keywords: Idiopathic giant right aneuiysm
G I O I T H I E U
P h i n h dgi n h i p h a i la m o t b e n h tim b a m sinh cue h i e m cd the g a p d bat cii Iiia tuoi n a o t u s o sinh d e n n g u d i ldn. Benh k h d n g k e m t h e o cac b | n h ly khac n h u : b ? n h v a n b a la, b e n h tim b a m sinh, cao a p d p n g m a c h p h o i h a y n h i m g p h a n u n g viem m a c p h a i t r o n g co tim.
Da so cac t r u d n g h p p k h o n g bieu hien trieu chiing, m p t vai b e n h n h a n bieu hien roi loan n h i p n h i h a y cd bieh chiing t h u y e n tac. Vi d a y la mpt b e n h h i e m g a p , p h i n h d a i n h i p h a i co the d e d a n g n h a m lan vdi cac chan d o a n khac, cac b e n h d a n d e n ldn n h i phiii, n h u la Ebstern's, hoac t r a n dich m a n g tim, n h u t r u d n g h p p cua c h i i n g toi khi t h a m k h a m b a n d a u .
C h i i n g tdi b a o cao m d t t r u d n g h p p b e trai bieu hien trieu c h i i n g s u y h d h a p va d u p c p h a u
t h u a t cat b o tui p h i n h nhi p h a i t h a n h cong.
P h i n h n h i p h a i xuat t h a n h t u d o cua t a m nhi, t h u d n g n h a t 6 tieu nhi.
B A O C A O M O T C A L A M S A N G Be trai 7 tudi, bieu hien trieu chiing suy h o h a p , n h a p khoa tim m a c h vi c h u p X q u a n g n g u c t h a n g p h a t h i e n b d n g tim to ( H l n h 1), n h u n g h i a n h o a n phdi b i n h t h u d n g . Be d u g c sieu a m tai k h o a c a p ciiu va co c h a n d o a n tran dich m a n g tim, n h u n g khi k i e m tra lai tren sieu a m tai khoa tim mach, d a y la t r u d n g h o p p h i n h dai n h i phai.
Ket q u a sieu a m tim cho t h a y co m d t khoi d a m d d dich, kfch t h u d c r a t ldn (d> 15 cm) n g h i d o p h i n h tieii n h i p h a i / n h i phai. Echo contrast {+) (mau t u T M C tren -> mass). N h i trai, that trai , t h a t p h a i cd t h u d c h o i n h d d o khoi n a y chen ep.
V a n 2 la, v a n d p n g m a c h chu va v a n d p n g m a c h
• Benh vi?n Nhi Dong 2.
Tdc gid lien he: Bs CKI Nguyin D6 Trong DT: 0S3S227453
Chuyen De Ngoai Nhi
Email: [email protected]
185
Nghien curu Y hpc Y Hpc TP. Ho Chi Mmh * Phu Ban Tap 19 * So 5 * 2015
phdi binh thudng. Van ba la hd nhe PAPs = 25 mm Hg. Dudng vao that T va dudng thoat 2 that khong hep. Tinh mach chii tren va dudi khong
dan. (Hinh 2). i Dien tam do cho thay nhip xoang deu, song
P binh thudng.
Be duoc hpi chah phau thugt cat bdt tui phinh nhi, nghi la nguyen nhan gay suy hd hap d tre, ngan ngua huyet khoi nhi phai, giam nguy CO loan nhip nhi.
Phlu thuat dupc thuc hien dudi tuan hoan ngoai CO the. Khi mdnguc, nhi phai phinh rat to, chiem nhu toan bp trung that, thanh tam nhi dan deu mdng nhu td giay, khong phan biet dupc giiia mo binh thudng va mo benh ly. Vdng van ba la dan. Cac day chang van binh thudng.
Khdng ghi nhan cac sang thuong tren dgi ttie.
(Hlnh 3).
Phlu thuat bao gom cat bd phan tui phinh to nhi phai. Tao hinh vdng van ba la bang manh gap mang ngoai tim.
Kiem tra sieu am va X quang nguc sau md cho thay kich thudc nhi phai giam dang ke (Hinh 1). Khdng tran djch mang tim. Khong con hd van ba la dang ke. Ket qua md hpc cho thay thanh nhi dan mong nhu td giay, teo va thoai hda kem tang sinh mo spi lanh tinh.{Hinh 4) Tai kham sau 1 nam cho tiiay be khdng bieu hien them trieu chiing gi.
Hinh 3: Trong mo. 1/ Ben trdi: nhi phdi phinh rdi to klii nhgn dgng dgi tliema nguc. 2/ Phdu thugt tgo hinh nhiphdi difdi chay tudn hodn ngodi ca the, cat bdt mo nhiphdi dan
Hinh 1. Hmh X quang ngifc thdng 1/ ben trdi: bong tim rai to vd phi dgi nhi phdi, tiian hodn plioi binh thif^ig. 2/ bin phdi: kich thuac tim binh thumig sau phdu thugt.
Hinh 4 Mo thanh nliTphai duac cat i a rai mong tren dai the Ca hm co vung teo va thoai hoa kem tang sinh mo sgi ldnh tinh. Mo ca vdn v&i nhdn te'bdo ca to vd mo lien kei sgi
Chuyen De Ngo^l Nhi
Y Hpc TP. Hb Chi Minh * Phu Ban Tap 19 * Sb 5 * 2015 Nghien cuu Y hpc
BAN L U 4 N
Phinh nhi phai la benh hiem gap. Nguyen nhan thudng khdng ro, nhung cd the la do bain sinh. Cac bao cao rieng le cho thay phinh nhi phai cd the xay ra d moi Itia tuoi tii so sinh d&i ngudi ldn. Bailey va cpng su*^' mo ta benh nay Kn dau tien nam 1955, nhieu ca lam sang dupc bao cao rieng le sau do'-''^-*^'''""*. Bieu hien lam sang hi khong cd t r i ^ chiing, den nhip nhanh tren that va / hoac suy tim. Benh nhan ciing cd nguy CO thuyen tac phoi khi cd huyet khoi hinh thanh trong that phai.
Mpt nghien cuu<5i ve tat ca cac tgt b&n smh ciia nhi phai va xoang vanh trong y van thu tiiap tir nam 1955 den 1998, cd tdng cpng 105 trudng hpp. Trong sd do, phinh nhi phai chiem tdng cpng 60 ca bam sinh. Tudi bieu hien benh co the gap tir 32 tuan thai den 75 tudi. 48% ca khdng bi§'u hi#n trieu chiing. Cdn lai bieu hi^n lam sang vdi cac trieu chiing rdi logn nhip, tim nhanh, dau nguc, kho tiid va met moi. Nhip xoang gap trong 53% cac ca. Cac rdi logn nhip nhi bao gdm nmg nhi, cudng nhi chi§'m 28%. Roi logn dan truyen khac cd the gap bao gom nhip sdm, nhip bd noi, block nhi that, va nhjp nhanh tren that.
u cac benh nhi khdng bieu hien trieu chiing, da sd phat hien finh cd khi chup Xquang nguc thang. Cd the Iam lan chan doan ban dau vdi benh nay nhu: bat thudng Ebstein's, tran dich mang tim, ken mang ngoai tim, u tim"^'^.
Trudng hpp ciia chiing tdi, chiing tdi lam lan chah doan ban dau la tran dich mang tim, nhung khi kiem tra lai qua sieu am tim, cho ket qua la phinh nhi.
Cac tgt tim kem theo nhu thdng lien that, thong lien nhi, hii phinh mgch vanh thudng khdng nhieu. Thong thudng cd mdt khiem khuyet bain sinh cua thanh tam nhi lam tam nhi de bi phinh ra, dudi ap luc tim phai thudng thap hon tim trai, Thoai hoa md (lipomatous degeneration) va giam cac yeu to cua co tam nhi trong tlii phinh dong duoc bao cao. Co bao cao ciing cho rang mot trong nhiing nguyen nhan
hiem d tre em la benh co tim md bao (histiocytoid cardiomyopathy)"^'.
Phinh nhi mac phai thudng lien quan den chan thuong trudc dd.
Tren X quang nguc tre thudng cd bong tim rat to, dien tam do cd the binh thudng hay nhip nhanh tren that. Sieu am tim la phuong phap chah doan thudng gap nhat de loai trir cac benh khac, gjiip do kich thuoc va hinh dang nhi phai.
Ddi khi neu kho phan biet giiia pMnh nhi phai vdi u tim hay cac cau tnic khac. CT Scan hay MRI gjiip ich cho chan doanP'".
Trong y van co nhieu phuong phap dieu tri benh nay nhu theo doi bao ton, phau thugt cit tlii thua dudi tuan hoan ngoai co the va sua chiia cac tat tim kem thed^'^.
Dieu tri bao ton khdng nhirng khdng ngan chan dan nhi tien trien ma cdn co cac nguy co khac nhu huyet khdi, rdi loan nhip ac tinh, hd ba la va dot tli.
Phau thuat cat bo tiii phinh giiip dieu tri roi loan nhip hieu qua. Kopf va cpng su*"'' ap dung ky thuat Maze kha thanh cong trong cac benh tim bam sinh ben phai, cho rang day la mpt trong nhiing each dieu tii hieu qua trong dieu tri rdi loan nhip, dac biet nhip vao lai.
Cd bao cao thuc hien tren tre so sinh co phinh nhi phai, khong kem cac tat bam sinh khac, khong chay tuan hoan ngoai co the. Tac gia diing nhieu clamp mach mau mem, chua lai tieu nhi phai, cho la giiip bao tdn yeu td lpi tieii Natri nhi (atnal natriuretic factor). O benh nhi nay cd ket qua mo hpc la phan ly te bao co (myocytolysis) vdi thua thdt te bao co d thanh tam nhi dupc cat bo lam tam nhi it co dan va xu hudng to ra dan's.
Theo y van, do benh hiem nen khong the tien lupng benh nhan neu khdng dieu tri hay tien lupng tai phat dan nhi phai sau md'-^-'"'^
Trong trudng hpp ciia chiing toi benh nhan dap ling tot sau can thiep phau thugt. Do vgy, de giam thieu nguy co xay ra cac bien chiing de dpa mang song cua benh nhan (nhip
Chuyen D e Ngoai Nhi
Nghien cuu Y hpc Y Hpc TP. Hb Chi Minh * Phu Ban Tap 19 * Sb 5 * 2015
nhanh, suy tim sung huyet, thuyen tac phoi), chiing tdi de nghi nen chi dinh phau thuat cat bo phinh tam nhi ngay ca d nhiing benh nhi khdng bieu hien trieu chiing. Nguyen tac chung cho phau thuat bao gom chay tuan hoan ngoai co the, c^t bo phinh dan nhi va tao hinh nhi phai . PhSu thuat nay cd nguy co thap va thich hpp cho dieu tri tat benh nay.
TAI LIEU THAM KHAO
1 Asayama ], Matsuura T, Endp N, Malsukubo H, Furukawa K {19771 Idiopathic enlargonert of the right atnum. Am ] Cardwh^^pp. 620-3-
' Bailey C (1995) Surgery of Ihe heart Philaddphia: Lea &
Febiger. pp 403-20.
3 Beder SD, NihUl MR, McNamara DG (1382). IdiopalWc dilabon of the nght atrium in a d u l d /m Hmt /,1D3' pp-134-7 4 Bdsissy SM. Kon)-a L (1984). Idiopathic dilatabon d flie nght
atrium. A case report. Saaid J Thorac Cardiovasc Surg 18 p p 191-2.
-) Binder TM, Rosenhdt R Frank H, Gwechenberger M Mairer G, Baumgartner H. (2000). Congenital malformations of the right atrium and the coronarj' sdnus" An analysis based on 103 cases reported In the literature and two additional cases.
Chest;:i7:pp. 1740-8.
6 Chatrafl* R, Tuiek O, Quivers ES, Clnscoll DJ, Edwards WD.
Danielson GK. (2001). AsjTnptomatic giant nght atnal aneurysm. Tex Heart Inst ].2S-:pp. 301-3.
7 Da Siva AM, MTitsembuig H Hzenza X. Stewart P (1992) Idiopathic dilatation of the nght atnum diagnosed m utero Rev Port Cardiol.;llpp, 161-3.
8 Joshi P, Pohlner P ((2005). Right atnal aneuiysm treated with atiioplasty without u s n g cardiopulmonaiy bypass in an mfant Eur J of Canii thorac Sur 28 - pp. 343-345.
9 Kun YI, Kim H, Choi JY (1995) Right atnal aneurysm Caniiol Y o u n g 5 p p 354-356
10. Kopf GS, Mdlo DM, Kennev KM, Moltedo J, Rollin.son NR.
SnydCT CS (2002), Intraoperative radiofrequency ablation of the atrium: effectiveness for treatment of supra\'entncular tachycardia in cOT^erutal heart surgerv. Ann Thorac Surg;74(3)pp. 797-804.
11. Kozdj M, Angdsla R, Pavenik D, Zonnan D (1998) Miopattuc enlargement(rfthenghtatriuHL PedCardiol,19:pp 420421.
12. Lee Y, Cho J, Kim G, Lee S. Hyun M, Kim Y (2011). Surreal repair of g a n t ri^ atrial aneurysm in a neonate. Korean G r c J.,41:pp. 331-3.
13 Maione S, Giunta A, Betocchi S, Ferro G, Vigonto C, Chianetlo M (1983). Two dunensional echocardiography m idiopathic enlargement of the nght atrium. Reliability and knitations Cardiok^yrTlkRS 216-22.
14. M o n c w AG, Behrendt DM (1968) Congenital aneun'sm (diverticulum) of the right atrium. Clinical manifstalions and results of operative treatment Grculation;3S-pp 124-8 15. Papagiannis ], Chatzis A, Sanis G (2008). Giant nght atnal
aneurysm; A case report. Int J CardioL,-pp. 129 e5-6- l e Pastor BH, Forte AL (1961). Idiopathic enlargement of the
right atnum. A m J Cardiol;8:pp 513-8.
17 Reinhardt-Owlya L, Sekarski N, Huini M, Laurini R, Payot M.
(1998). Idiopathic dilatation of the right atnum amulating Ebstdn's anomaly. Apropos of a case diagnosed m utero [in French]. Arch Mai Ci«ur Vaiss;9Ipp. 645-9.
IS. Rufledge J, Robertson MA, Kantodi M,-Dyck ] (1997) Idiopathic dilabon of the nght atnum; case rqxirt and survey of the literahire. Can J C a r d i o t l S f ^ . 855-7.
19 Sheldon W C Jdmson CD, Favaloro RG (1969)- Idiopathic e t J a r g a n e i t of the ri^t atrium. Report of four cases. Am } CardJol;23:pp 278-84.
20 Zedwegts CJ, Hensens AG, Lacquet LK (1997). Asymptomatc right atnal aneuiysnt Fortuitous 6nding and n Cardiothorac Surg.;ll:pp. 591-3.
Ngdy nhdn bdi bdo: 24/08/2015.
Ngdy phdn bicn nhgn xet bdi bdo: 25/08/2015.
Ngdy bdi bdo dugc dang: 01/10/2015