Tâm thất độc nhất dị tật tim

UNIVENTRICULAR ATRIOVENTRICULAR CONNECTIONS Double Inlet Single Inlet Common Inlet univentricular connection: double inlet, single inlet, and common inlet... When the right AV annulu

TÂM THẤT ĐỘC NHẤT

TS LỄ KIM TUYẾN VIÊN TIM TPHCM

DEFINITION VAN PRAAGH /ANDERSON

¢ Single ventricle

¢ Common Ventricle

¢ Single ventricle with small outlet chamber

¢ Heart with two chambers

¢ HOLMES heart

¢ Primitive ventricle

¢ Bilocular heart

¢ Trilocular heart

¢ Biatrial trilocular heart

¢ Left ventricle double inlet

-Tâm thất độc nhất (đúng nghĩa) :

Hai van NT vào 1 buông thất

Một van NT chung trong KNT

Bao gồm : Không lỗ van 2 lá hoặc 3 lá

Thiéu sản tim trai

Không lỗ van ĐMP với

thiểu sản thất phải

HỘI NGHỊ TIM MẠCH _ -.® ` T0ÀNUỐC 20G *> < :

BTBS CÓ BIEU HIEN DANG TAM

THAT BOC NHAT/ SATT

Hội chteng thiéu san tim trai Không lô van DMP voi VLT kin Kênh nhĩ thât (không cân đổi) Tâm thât độc nhât/ HC đông dang Bát tương hợp đôi kèm không van 3 lá

UNIVENTRICULAR ATRIOVENTRICULAR CONNECTIONS

Double Inlet Single Inlet Common Inlet

univentricular connection: double inlet, single inlet, and common inlet

OVERRIDING ATRIOVENTRICULAR VALVES Effect on Atrioventricular Connections

overriding overriding Overriding Overriding

RA-RV Connection RA-LV Connection

tricular (AV) connection based on the 50% rule Note the associated effect of increasing atrial and

ventricular septal malalignment produced by increasing annular override When the right AV annulus

overrides more than 50%, there is a predominant right atrium (RA) to left ventricle (LV) connection LA,

left atrium; RV, right ventricle

Schematic representation to show the possible segmental combinations that can result in a functionally univentricular heart Any of the four types

of atrial arrangement can be present There are then two major divistons, those with univentricular atrioventricular connections, double inlet,

or absent connection, or else those with biventricular connections which can be concordant, discordant or ambiguous when the atrial appendages are isomeric Any of these types of atrioventricular connection can potentially co-exist with either a dominant left, right or indeterminate ven- tricle Likewtse any form of ventriculo-arterial connection 1s posstble and associated malformations need also to be documented separately

iology in the Young: Supplement 3 (2005) December 2005 i

e Assess for the morphology of the larger ventricle

Identify the atrioventricular valve connections to

the left ventricle, and assess size and degree of

insufficiency, if present

Identify the atrial septum and direction of shunt- ing across

lf the left atrioventricular valve is small, assess

left-to-right gradient across the atrial septum and interrogate the pulmonary veins for Doppler flow

pattern in order to determine whether there is

obstruction to left atrial egress

Identify the position of the small right ventricle as

either anterior and rightward, anterior and left- ward, or superior

Locate and identify the size of the ventricular

septal defect communication between the large

left ventricle and the smaller right ventricle

s ldentify the great arterial positon and origins

as either from large left or from small right ventricle

e Determine the nature of the pathway patency from the large left ventricle to the pulmonary artery and to the aorta

e Assess direction of flow in the ductus arteriosus,

antegrade from pulmonary artery to aorta or retrograde from aorta to pulmonary artery

e Assess the size of the ascending aorta and aortic arch when the aorta arises from a small hypo- plastic right ventricle and the ventricular septal defect Is restrictive

e Identify the direction of flow in the transverse aorta when there is aortic outflow obstruction

e Assess the size of the branch pulmonary arteries when there is pulmonic outflow obstruction

e Look for irregularity in heart rhythm, which may

reflect heart block

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The Univen†ricular Hear†

All will end up with one

ventricle repair (Fontan)

Neonatal surgery varies

From 0% (no surgery necessary) to

30% mor†ali†y

2"4 s†age: Glenn (SVC to RPA)

3r4 s†aqe: condui† TVC †o RPA

Cumula†ive mor†ality 10-50% by 5 years

The Univen†ricular Hear†

What's wrong with only one ventricle?

_ Incidence 4-40%, increasing over time

_ Influenced by Fontan technique

Univentricular hearts are dasstfied on the basis of

insertion and appendages morphology), type of atrioventricular (AV) connection (absent right,

double-iniet or absent left), ventricular morphology (dorninant left ventricle, dorninant right ventride or

indeterminate single ventricle) and ventriculo-

of transposed great arteries) IVC, inferior vena cava;

LV, left ventricle; RV, right ventnide

Echocardiography in Pediatric and Congenital Heart Disease: From Fetus to Adult

Arterial segment

¢

great vessels i greatvessels Edited by Wyman W Lai, Luc L Mertens, Meryl S Cohen and Tal Geva

© 2009 Blackwell Publishing Ltd ISBN: 978-1-405-17401-5

DOI: 10.1002/pd.4821 PRENATAL DIAGNOSIS

ORIGINAL ARTICLE

Prenatal diagnosis of functionally univentricular heart, associations

and perinatal outcomes

Aline Wolter! *, Sina Nosbiisch', Andreea Kawecki', Jan Degenhardt', Christian Enzensberger’, Oliver Graupner?, Carina Vorisek!,

Hakan Akintiirk*, Can Yerebakan*, Markus Khalil®, Dietmar Schranz®, Jochen Ritgen®, Riidiger Stressig® and Roland Axtfliedner!

ABSTRACT

Objective Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or

systemic circulation cannot be supported independently The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH

Methods We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne We included double inlet left ventricle (DILV), tricuspid valve atresia

(TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC)

Results Of initially 155 patients, 128 were liveborn (82.6%) Ten neonates (7.8%) were lost to follow-up, in three

(2.5%) neonates, parents decided for compassionate care Overall survival after prenatal diagnosis of functionally UVH was

67.1%, and 90.4% on an intention-to-treat basis Survival after surgery reached 93.7% The majority of deaths occurred within

the group of dominant RV (10/74, 13.5%) High risk HLHS with restrictive foramen ovale was associated with the lowest

survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p< 0.05)

Conclusion These results should be explained to parents to ensure informed decisions and counselling © 2016 John Wiley & Sons, Ltd

155 fetuses with UVH 9 lost for follow up

3 compassionate care 118 with 30dđ outcome (Dom LV = 2; Dom &V = 1) < (Bom LV = 43; Bom RV =75)

+115 intention to treat (74.2%)

(Dom LV = 41; Dom RV & 74)

11 neonatal deaths with intention to treat 104 survivors (67.1%)

(7.1%) (Dom LV = 40; Dom RV = 64)

(DOM LV = 1 died Ove rail survival 104/155 (67.196)

Kêt Luận

<> Tam that 2 buông nhận là dạng thường

gặp nhất của bệnh tâm thât độc nhất

+ Biểu hiện là 2 nhĩ kết nội với 1 tam that

chung qua 2 van nhĩ thất trái và phải

“- Dạng thường gặp nhất là dạng thất trái 2

buông nhận, chiêm 80%

+ Biểu hiện trên mặt cắt 4 buông

HỘI N0HỊTIMMACH -@:` T0ÀNUỮ 20G ` yw

Kêt Luận (tt)

Thường kèm tắc nghẽn đường ra và ảnh hưởng đên mạch máu ra từ thất thiêu sản

<> Cac bat thường kết hợp: không, thiêu sản

van NT, tặc nghẽn đường ra ĐMIP, DMC

va bat thường đường dẫn truyên

+ Không nên lạm dụng từ “tâm thât độc nhất

chức năng”

+ Mô tả giải phẫu chỉ tiết theo tang giúp phát

hoạ tiên lượng gân

a vận cònxâu ¡ao HÔINGHITIIMMACOH -2@:" 7C

‘ea’ > sae