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 atriale

TLN 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 che

Sinh 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 related

Patient 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