KHOÂNG LOÃ VAN 3 LAÙ 1- 3% caùc beänh TBS - 1/10000 - 20000 sô sinh Tam saéc theå 21 - Hoäi chöùng “Maét meøo” BS. Ñaøo Höõu Trung
KHONG LO VAN 3 LA
1- 3% cac benh TBS - 1/10000 - 20000 s sinh
Tam sac the 21 - Hoi chng Mat meo
BS. ao Hu Trung
NH NGHA - NH DANH - PHAN LOAI
- Tim mot that
- Tam that chnh Trai
- Khong noi tiep NT - phai - Situs Solitus
- Phan loai :
IIaIbIcKhong co CV M
IIIIaIIbIIcCV M Type D
IIICV M Type L
Giai phau benh ly
Van 3 la - Khong phat trien - X hoa - MangNh phai - Day - 10% juxtaposition atrialeTLN nhieu typ
That trai - Buong that chnh - Day dan tuy thuoc lng mau len phoiThat phai - That phu 2 thanh phanThong lien that thng la han cheSinh ly benh hoc
3 yeu to- Kch thc lo TLN
- Co hay khong hep MP + Ch/v ?
- Kch thc lo thong TLT
Thong thng co 2 the- Tang lng mau len phoi - Hiem
- Giam lng mau len phoi - Nhieu hn
Anatomic classification of tricuspid atresia. In about 70% of cases the great arteries are normally related, and there is a small ventricular septal defect (VDS) with associated hypoplasia of the pulmonary artery (PA). When the great arteries are transposed, the VSD is usually large, and the Pas are large with increased pulmonary blood flow. AS, aortic stenosis ; D-TGA, complete TGA ; L-TGA, congenitally corrected TGA ; PA, pulmonary atresia ; PS, pulmonary stenosis ; Sub AS, subaortic stenosis ; Sub PS, subpulmonary stenosis ; TGA, transposition of the great arteries ; VSD, ventricular septal defect. (Data from Keith JD, Rowe RD, Vlad P : Heart Disease in Infancy and Childhood, 3rd ed. New York, Macmillan, 1978)
Classification by associated lesions. Type I, normally related great arteries ; II, D-transposed great arteries : a. pulmonary atresia; b. subpulmonary or pulmonary valvular stenosis (reduced pulmonary blood flow); c. no pulmonary stenosis (normal or increased pulmonary blood flow), and III, L-transposed great arteries.
(Adopted from Edwards JE, Burchell HB; congenital tricuspid atresia : A classification. Med Clin North Am 33 : 1177, 1949)
Factors that determine physiology :
VSD : if tiny, then the RV is severely hypoplastic and VR related great artery receives no direct flow. If large, then the RV can even be of normal sizeStenosis of semilunar valve arising from the RV ; this determines the volume of flow into its great arteryGreat vessel relation : TGA or normally relatedPatient to left has : Tricuspid atresia + small/moderate VSD ; PS ; normally related great arteries.
Restrictive VSD makes RV small ; small RV and PS make MPA small ; Two above factors make the Qp inadequate
Patient to left has : Tricuspid atresia + normally related great arteries. There is no VSD and therefore no RV ; without trans-RV flow in utero there is pulmonary atresia
Pulmonary blood flow is ductal-dependent ; therefore, the patient needs a shunt
Patient to left has : Tricuspid atresia + a small/moderate VSD ; AORTIC stenosis and TGA (Ao from RV and MPA from LV)
The small VSD has reduced flow in utero, causing hypoplasia of the RV, aortic valve, and Aao
FUNCTIONALLY, this child has hypoplastic left heart syndrome
LAM SANG
2 the pho bien nhat
- Khong CVMTuy thuoc hep MP
Tm sm
- Co CVMThng kem tang lu lng mau len phoi
Suy tim sm
CAN LAM SANG
ECGTruc lech trai - Ph NP - Ph TT
(SV1V2 > RV5,V6)
Song P thay oi 80% ca D1, D2, aVF, V1
Xq TP
- Dang hep MP:Tim khong ln
Hnh qua trng
Tuan hoan phoi giam
- Dang khong co hep MP:Tim ln
Tuan hoan phoi tang
CAN LAM SANG
Sieu am- Muc ch:Cach noi tiep tang NT - Xac nh ton thng
Dang - Chc nang van NT, That
V tr that, v tr M, kch thc
Ton thng phoi hp - TLT - TLN - OM...
- Mat cat hu ch:4 B mom
DS vong quanh, DS doc
- Chan oan xac nh:Khong co lo van 3 la
Ch co bo may van NT trai
Kch thc TLN - TLT
ng MP - MC
Tracing from a 6-month-old girl with tricuspid atresia showing left anterior hemiblock (-30 degrees), right atrial hypertrophy, and left ventricular hypertrophy
Posteroanterior view of chest roentgenogram in an infant with rricuspid atresia with normally related great arteries. The heart is minimally enlarged. The pulmonary vascular markings are decreased, and the main pulmonary artery segment is soewhat concave
The electrocardiogram typically demonstrates left axis deviation and left ventricular enlargement ; alarge notched P-wave may be present, indicating atrial enlargement (P-tricuspidale)
Mat cat 4 buong t mom : van 3 la la 1 mang day, co TLT ln i kem (A). Mat cat 4 buong di sn (B-C) : van 3 la la 1 mang day
DIEN TIEN
- 40% t vong nam 1 tuoi
- 50% t vong t 1 - 15 tuoi - Tuy thuoc tuan hoan phoi
- Bien chng than kinh: cn ngat, abces
- Bien chng VNTM 7%
- Loan nhp (Rung nh, Cuong nh)
- Suy tim
IEU TR
ieu tr phau thuat - Ho tr
- The giam lu lng mau len phoi
S sinh 6 thang tuoi:Rashkind (neu TLN han che)
BTM - GTX neu tm nhieu
Tren 6 thang - 2 tuoi:GLENN TMC tren x MP-P
- The tang lu lng mau len phoi
Co CVM :That M luc 3 thang tuoi
Khong co CVM :Tuy trng hp that tam thi ngan han
IEU TR
ieu tr phau thuat sa cha
- Kieu FONTAN
Nguyen tac:Sa cha he tieu TH. Dan lu mau
TM chu -> MP, ngan chan pha tron mau TM - M
- Ky thuat
Co CVM:ong lo TLN - Cat than MP
Noi trc tiep tieu nh P - MP khong van
Khong co CVM: ong TLN - ong TLT
Noi trc tiep tieu nh P -
Buong TP co van hay That lo pheu
IEU TR
ieu tr phau thuat sa cha
ieu kien
- Tuoi: 2 - 4 tuoi
- Nhp xoang - TMC bnh thng
- NP kch thc co that BT
- Khang lc MP BT - PAPM < 15 mmHg than MP BT
- TT BT - Khong h van 2 la - nhanh MP
khong hep
Tieu chuan (co ien) e phau thuat Fontan c toi u
(10 ieu ran cua Fontan)
Tuoi 4-15
Nhp xoang
He thong tnh mach : bnh thng
The tch nh phai : bnh thng
Ap lc MP trung bnh < 15mmHg
Khang lc MP < 4 v Woods/m2
T le ng knh MP/MC > 0,75
Phan suat tong mau tam thu > 0,60
Van hai la, kn
MP khong b van veo
TL : Choussat 1980
ABCDPA can be constructed as a bidirectional Glenn shunt in which the superior vena cava is anastomosed to the confluent pulmonary arteries. (From Castaneda AR, Jonas RA, Mayer JE, et al : Single ventricle with tricuspid atresia. In Cardiac Surgery of the Neonate and Infant. Philadelphia, WB Saunders, 1994, p.262 ; with permission)
Glenn
Hemi Fontan
Original Fontan
Atrio-pulm
Derivation atrio-pulmonaire
Le toit de loreillette droite (OD ) a ete anastomose a la face inferieure de lartere pulmonaire droite (APD) proximale. Tout le sang cave est ainsi derive a larbre arteriel pulmonaire car la communication inter-auriculaire (CIA) a ete fermee.
Superior caval reconstruction. The superior cava is transected and the cardiac and cephalad ends are anastomosed to the pulmonary arterial confluence. A septation patch placed within the right atrium completes cavocaval continuity and separates systemic from pulmonary venous drainage
Derivation cavo-pulmonaires partielles
Il ny a quune seule veine cave superiure (VCS) situee a droite et recevant un tronc veineux innomine (TVI). La grande veine azygos (Az) a ete sctionnee-suturee, la VCS a ete sectionnee et son bout peripherique anastromose a lartere pulmonaire droite (APD), ce qui permet une irrigation bi-directonnelle (fleches) du sang cave
Il y a deux veines caves superieures, droite (VCSD) et gauche (VCSG). On procede de la meme facon quen A mais de facon bilaterale sur les arteres pulmonaires droite (APD) et gauche (APG)
Exemples de circulation mixte
Un montage cavo-pulmonaire superieur bi-directonnel a ete confectionne comme sur la figure 2A. On peut y ajouter : une anas tomose systemico-pulmonaire gauche (BG), droite (BD) ou laisser libre la voie anerograde venctriculo-pulmonaire (VA)
Derivation cavo-pulmonaire sub-totale
Cest un montage identique a celui de la Figure 2A. Le grainage azygos (Az) a la veine cave sperieure (VCS) apporte aux arteres pulmonaires tout le sang cave inferieur mais ni le sang veineux coronaire qui reste draine a loreillette droite (OD) par le sinus coronaire (Sco), ni le sang des veines sus-hepatiques (VSH) qui debouchement directement dans lOD
Principaux types de derivation cavo-pulmonaire totale. On a commence par faire un montage cavo-pulmonaire superieur bi-directionnel
A : La CIA a ete fermee et le bout cardiaque de la VCS anastomose a lAPD. Le sinus coronaire se draine a lOD
B : LOD a ete divisee par une piece de tissu synthetique (en rouge) et le bout cardiaque de la VSC anastomose a lAPD. Le sinus coronaire se draine maintenant a loreillette gauche. Une fenetre peut etre ou ne pas etre percee dans la paroi du tunnel intra-auriculaire pour autoriser un shunt droite-gauche (fleche rouge).
C : La veine cave inferieure (VCI) a ete anastomosee a lAPD par un conduit prothetique extra-cardique (rouge) le long de la paroi externe de lOD. On peut aussi le fenestrer a loreillete droite devenue gauche
Modified Fontan
A variant of the Fontan operation in a patient with tricuspid atresia and associated transposition of the great arteries (aorta cut away to show details of operation).
A : The main pulmonary aetery is divided, and the opening is extended into the right pulmonary artery behind the superior vena cava. The superior vena cava is divided, and th e incision is extended into the base of the right atrial appendage
B : The proximal stump of the main pulmonary artery has been over-sewn, the ustream end of the superior vena cava has been anastomosed to an opening in the right pulmonary artery, and the atrial septal defect has been closed with a patch
C : The large opening in the pulmonary artery has been sewn tho the large opening in the superior vena cava and right atrium. Now, both the inferior and superior venae cavae and no communications remain between the right and left sides of the heart.
Currently pupular modified Fontan operation.
Bidirectional Glenn operation or superior vena cava (SVC)-to-right pulmonary artery anastomosis.
Cavocaval baffle-to-pulmonary artery (PA) connection, with or without fenestration. See text for description of these procedures. AO, aorta ; IVC, inferior vena cava ; LV, left ventricle ; RA, right atrium ; RV, right ventricle
The Damus-Stansel-Kaye uses the unobstructed pulmonary outflow tract to provide relief of systemic outflow obstruction. The main pulmonary artery is anastomosed to the aorta. Pulmonary blood flow is provided with a systemic to pulmonary artery shunt or a BCDPA.
TL : Laks H, Gates RN, Elami A, et al : Damus-Stansel-Kaye procedure : Technical modifications. Ann Thorac Surg 54 : 169-172, 1992 ; with permission