Overview of Congenital

Overview of Congenital
Heart
Heart
Diseases
Diseases
Dr. K. Vanderdonck
Charlotte Maxeke Johannesburg Academic Hospital
Universit of the !itwatersrand
"AHA #$%&'%#'&&

Congenital Heart Disease (CHD) =

0.8% of all live births

12% of all infants with CHD have

chromosomal abnormalities

n c!rrent era" # 8$% of all chil%ren born with

CHD sho!l% reach a%!lt life if treate%
a&&ro&riatel'

Correction ( re&air

)ole of &alliation

nterventional car%iolog'
Incidence

n )*+"

# 10 000 chil%ren born each 'ear with CHD

,0% of them are in nee% of car%iac s!rger'

CHD res&onsible for 1.2% of !n%er $

mortalit'

-n%erestimate% beca!se man' %ie

!n%iagnose%
Incidence

.ormal l!ng mat!ration

/egins at en% of &regnanc'

Contin!es for some time after birth

0ormation of alveoli = a &ostnatal event

1ith initiation of s&ontaneo!s ventilation

)emo%eling an% mat!ration of &!lmonar'

vasc!lat!re

Dro& of 2+ (&!lmonar' arter') &ress!re 3 24)

(&!lmonar' vasc!lar resistance)

2rocess com&lete b' , months of age

Heart & Lungs

Congenital heart %efects have 5 %ifferent effects on

the &!lmonar' circ!lation

ncrease% &!lmonar' bloo% flow

Decrease% &!lmonar' bloo% flow

ncrease% &!lmonar' veno!s &ress!re

6ach t'&e &ro%!ces a %ifferent &attern of

im&aire% growth an% remo%eling of the
&!lmonar' vasc!lar be%
Heart & Lungs

ncrease% &!lmonar' bloo% flow (2/0)

.ormal remo%eling of neonatal vasc!lat!re

%oes not occ!r

7ea%s to &!lmonar' h'&ertension an%

increase% &!lmonar' vasc!lar resistance (24))

Develo&ment of &!lmonar' vasc!lar

obstr!ctive %isease (248D)

24) initiall' reversible

-no&erate%" 24) becomes irreversible

6isenmenger s'n%rome
Heart & Lungs
4
6isenmenger s'n%rome9

*evere &!lmonar' vasc!lar obstr!ctive %isease

which is irreversible

Have s!&ras'stemic 2+ &ress!res an% 24)

with sh!nt reversal ()t 7t sh!nt)

ncreasing c'anosis

Death
Heart & Lungs

ncrease% &!lmonar' bloo% flow

Characteristic tria% of s'm&toms

)e&eate% chest infections

Congestive car%iac fail!re

0ail!re to thrive

2resent in all &atients with increase% 2/0

: ac'anotic an% c'anotic
Heart & Lungs

Decrease% &!lmonar' bloo% flow

+lveolar growth %evelo&ment is im&aire%

+ngiogenesis com&romise%

+ll broncho;&!lmonar' segments &resent b!t

smaller

ncrease% collateral &!lmonar' arterial bloo% flow"

&rimaril' from bronchial arteries

C'anosis
Heart & Lungs
Diagnosis

<ost infants referre% beca!se of a m!rm!r or

c'anosis

Histor'

2h'sical 6=amination

Diagnostic 2roce%!res

C>)

6C?

6CH8

8ften %iagnostic of the anatom'

<an' o&erations %one on 6cho %ata

6CH8

.ow the mainsta' of %iagnosis

Done b' a &ae%iatric car%iologist

,,% rate of ma@or %iagnostic errors when

&atient referre% from a%!lt 6cho lab

A66 gives a%%itional information abo!t

intracar%iac anatom'

ntrao& A66
Diagnosis

Car%iac catheteriBation

*till stan%ar% for9

<eas!ring intracar%iac &ress!res

C!antitating s'stemic an% &!lmonar' bloo%

flows

Calc!lating sh!nts

<eas!ring s'stemic an% &!lmonar' vasc!lar

resistances

nterventional catheter treatment

Diagnosis
Diagnosis

Car%iac CatheteriBation an% +ngiogra&h'9

Ao %efine anatom' es&eciall' in com&le= lesions

Ao assess o&erabilit'

Ao assess 2+2 D 24) an% res&onse to o='gen on

&!lmonar' vasc!lat!re

24) # 8 1oo% !nits in 100% o='gen constit!tes a

contra;in%ication to s!rger'

m&ortance of foetal echocar%iogra&h' an%

&renatal %iagnosis

Ao &re&are famil' for hos&ital a%mission

an% &lan s!rgical intervention

Ao give the o&tion of terminating the

&regnanc' if the &rognosis is &oor
Diagnosis
Classification


A
n
o
m
a
l
i
e
s
C
h
a
r
a
t
e
r
i
s
t
i
c
s

Acanotic
&( )* "hunt
+ncreased ,-.
/riad0
../
Chest infections
CC.
PDA
ASD
VSD
A-V Canal
#( Obstructive
1ormal ,-.
Often asmptomatic
Coarctation
Aortic stenosis
Pulmonary stenosis

Canotic
$( Decreased ,-.
Canosis
Child well2
asmptomatic
Tetralogy
Pulmonary atresia
Tricusp atresia a,b
TGV + PS
3( +ncreased ,-.
Canosis
/riad0
.//
Chest infections
CC.
Truncus
TAPVC
Tricuspid atresia c
TGV
Treatment

<e%ical treatment

*!rger'
2alliative vers!s Corrective 2roce%!res

Corrective *!rger'9 <a@orit' %one on C2/

2alliative *!rger'9 /A sh!nt : <2+ ban%

8&en an% Close% Heart *!rger'

8&en = %one on car%io&!lmonar' b'&ass

Close% = C2/ not necessar'

nterventional car%iolog'
Ductus dependant lesions

2rostaglan%in 61 (+l&rostatil) o&ens an% maintains

&atenc' of the %!ct!s arterios!s

0or &!lmonar' bloo% flow

2!lmonar' atresia (4* : 4*D)

*evere tetralog'

*ingle ventricle

0or mi=ing

A?4

0or s'stemic bloo% flow

H7H* ( Critical +*

++ ( *evere coarctation
Palliative Procedures
Palliative Procedures

2rovi%es s'm&tomatic relief (!s!all' tem&orar')

b!t leaves the lesion !ncorrecte%

+;2 sh!nts (arterial;&!lmonar' sh!nts)9

Designe% to increase &!lmonar' bloo% flow in

a c'anotic chil% with ina%eE!ate &!lmonar'
bloo% flow

+trial se&tectom'

Designe% to increase mi=ing at atrial level

2!lmonar' arter' ban%ing

Palliative Procedures
*ight
-/
shunt
&433
)eft
-/
shunt
*ight
modified
-/ shunt
&456
Central
"hunt
Aorto7pulmonar "hunts or Arterial "hunts
!aterston shunt
&46#
,otts shunt &436
Palliative Procedures
Palliative Procedures
,ulmonar Arter
-anding

2+ ban%9

Designe% to limit
2/0 in a chil%
with e=cessive
&!lmonar' bloo%
flow
Closed Heart Surger
Patent Ductus !rteriosus
2atent D!ct!s +rterios!s
(2D+)

Comm!nication between aorta

(+o) an% &!lmonar' arter'
(2+)

n foetal life9 con%!cts

!nsat!rate% bloo% from 2+ to
+o

.ormall' closes shortl' after

birth" b!t in some infants" ma'
remain o&en es&eciall' if
h'&o=ia &resent

f large 2D+" ma' %evelo&

&!lmonar' h'&ertension
2remat!re infants9

*&ontaneo!s clos!re is common

Ar' n%omethacine first if chil%

s'm&tomatic

*!rgical 7igation
Patent Ductus !rteriosus

*!rgical Clos!re : 7igation or Division

*&ontaneo!s clos!re not common when

term infants D is !n!s!al after the first few
months of life

f s'm&tomatic9 clos!re as soon as &ossible

*'m&toms = CC0 ( 0AA ( Chest

infections
Patent Ductus !rteriosus

*!rgical Clos!re : 7igation or Division

f as'm&tomatic9 clos!re &lanne% within

ne=t 5 months

Ao &revent */6

f &resence of 2HA9 ma' have to be

catheteriBe%
Patent Ductus !rteriosus
PD! ligation " division
)eft thoracotom 3
th
+C"
)igation
Division
& #
PD! transcat#eter closure

nterventional
Car%iolog'9

m&lantation of
+m&latBer %evice to
occl!%e 2D+
Coarctation
Coarctation of the +orta
(Co+)

.arrowing of the aortic l!men

%istal to the origin of the left
s!bclavian arter'

Have h'&ertension in the

&ro=imal arterial tree (!&&er
limbs) an% h'&otension an%
weaF &!lses in the lower limbs

n a%!lthoo%" %!e to severe

s'stemic h'&ertension" will have
cerebrovasc!lar acci%ents

+%!lt t'&e
coarctation9

8ften
as'm&tomatic

*!rger' in%icate%
as soon as
%iagnosis ma%e
Adult Coarctation or postductal
Coarctation

.eonatal coarctation9

Has a %ifferent
&resentation9

Car%iovasc!lar
colla&se

*evere metabolic
aci%osis

+t the time of %!ctal

clos!re
+nfantile Coarctation or
preductal
Coarctation

.eonatal coarctation

2revio!sl' associate% with high mortalit'

-se of &rostaglan%ins has change% o!tcome

*emi;!rgent s!rger' in%icate% once chil%

res!scitate% an% stabilise%
Coarctation

*!rgical AechniE!es9

6n%;to;en% anastomosis
)esection of coarctation an% %!ctal tiss!e

*!bclavian fla&
7eft s!bclavian arter' !se% to &atch the
%efect

2atch angio&last'
Coarctation
Surger Coarctation
*esection and end to end
anastomosis
Surger Coarctation
"ubclavian flap
,atch Angioplast
&
#
&
#

nterventional Car%iolog'9

/alloon %ilatation an% ( or stenting

6s&eciall' in rec!rrent Co+

*ometimes in native Co+

n ol%er &atients

f aortic arch is of a%eE!ate siBe

Coarctation
$pen Heart Surger
& Cardiopulmonar %pass
Cardiopulmonar %pass
+nitiall surger done using
controlled cross circulation
&483 9)illehei:
Cardiopulmonar %pass
Car%io&!lmonar' b'&ass =
C2/

Heart an% l!ngs e=cl!%e% from

circ!lation

4eno!s bloo% with%rawn b'

gravit' thro!gh cann!las in
*4C an% 4C

4eno!s bloo% %raine% into

o='genator where loses C82 D
gains 82

8='genate% bloo% &!m&e%

bacF into aorta via an arterial
cann!la

)ole of car%io&legia
Cardiopulmonar %pass
Cardiopulmonar %pass
Median sternotom
Arterial and venous
cannullas placed for
cardiopulmonar bpass
!trial Septal Defect
+trial *e&tal Defect (+*D)

Comm!nication between left

atri!m (7+) an% right atri!m
()+)

0low is !s!all' from 7+ to )+

res!lting in large &!lmonar'
bloo% flow

8ften as'm&tomatic in chil%hoo%

b!t ma' have freE!ent
res&irator' infections

Can %evelo& &!lmonar'

h'&ertension in a%!lthoo%
(!s!all' mo%erate)
!trial Septal Defect

+natom'9 5 t'&es
O
*ec!n%!m = 80%
O
*in!s 4enos!s
O
8sti!m 2rim!m

Histor'

1
st
clos!re in 1G$2 (Hohn 7ewis) !sing
h'&othermia D inflow occl!sion

1
st
%efect to be re&aire% on C2/ in 1G$5
(Hohn ?ibbon) !sing a &!m& o='genator

1
st
intracar%iac %efect to be s!ccessf!ll' manage%
with &erc!ataneo!s transcatheter techniE!es
!trial Septal Defect

n%ications for s!rger'

6arl' s!rger' if ca!ses s'm&toms9

CC0 ( 0AA ( Chest infections

*'m&toms more freE!ent when associate%

2+24C

f C&(Cs (7t)t sh!nt) # 1.$" b!t

controversial if as'm&tomatic

f as'm&tomatic : elective clos!re at ,;$

'ears
!trial Septal Defect

*!rger'

Done on C2/

/' %irect clos!re or &atch clos!re9

+!tologo!s &ericar%i!m

/ovine &ericar%i!m

nterventional Car%iolog'

8nl' +*D 2I" if small to mo%erate siBe" an%

rim &resent
!trial Septal Defect
Closure of !SD
!trial Septal Defect
4entric!lar *e&tal Defect
(4*D)

Comm!nication between left

ventricle (74) an% right
ventricle ()4)

1hen the sh!nt is large" there is

&!lmonar' h'&ertension which
ma' &ro%!ce %amage to the
&!lmonar' vasc!lar be% with
%evelo&ment of &!lmonar'
vasc!lar obstr!ctive %isease an%
sometimes 6isenmenger
s'n%rome
Ventricular Septal Defect
4entric!lar *e&tal Defect
+natom'9 , t'&es
OPerimembranous (B)
= 80%
-n%erneath antero;se&tal
commiss!re of the tric!s&i%
valve
OMuscular (D)
8ften m!lti&le
OInfundibular / Outlet (A)
+ssociate% aortic
reg!rgitation
OInlet (C)
solate% or &art of +4 canal
Ventricular Septal Defect

*mall 4*D

.ormal 2+ &ress!re ( normal 24)

.o s!rger' if &erimembrano!s or m!sc!lar

4*D : close s&ontaneo!sl'

<o%erate 4*D

2+ &ress!re = J s'stemic
6lective clos!re when 2 'ears of age

6arlier if s'm&tomatic (CC0 ( 0AA ( Chest

infections)
Ventricular Septal Defect

7arge 4*D

2+ &ress!re = s'stemic &ress!re

*h!nt %e&en%ent on %egree of 24)

Clos!re in infants9

f s'm&tomatic (CC0 ( 0AA ( Chest

infections)

f fail!re of me%ical treatment

f chil% # 1 'ear9 nee%s car%iac cath to

assess o&erabilit' (24) K 8 1oo% !nits D
2+2 reactive to 8
2
)
Ventricular Septal Defect

+4 canal %efects D malaligne% 4*DLs reE!ire

s!rgical clos!re (%o not close s&ontaneo!sl')

Contra;in%ications to s!rger'9

<arFe% fi=e% 24) # 8 1oo% !nits in 82

*!rgical AechniE!e

+lwa's &atch clos!re

Done on C2/

2+ ban%ing (rare)9

n *wiss cheese 4*DLs

*icF infants (&ne!monia)

Ventricular Septal Defect
VSD & Surger
&( *ight Atrium
opened
#( /ricuspid ;alve
retracted
$( ;"D exposed
3( Margins of ;"D
carefull assessed
VSD & Surger
,atch sutured in
place
+trio;4entric!lar Canal

+lso calle% en%ocar%ial c!shion

%efect

6=ists as &artial or com&lete

+4 canal
+
2artial +4 canal9 &resence
of +*D 1I onl'
+
Com&lete +4 canal9
&resence of +*D 1I an% inlet
4*D
+
)arel' isolate% inlet +4
canal t'&e 4*D
!trio&Ventricular Canal
+trio;4entric!lar Canal
+lso have abnormal mitral an%
tric!s&i% valves an% ma' have
mitral an%(or tric!s&i%
reg!rgitation
Com&lete +4 canal !s!all' has
large 7t )t sh!nt with severe
&!lmonar' h'&ertension
0reE!ent in DownLs s'n%rome
!trio&Ventricular Canal
!trio&Ventricular Canal

Com&lete +4 canal9

f &atient s'm&tomatic (CC0 ( 0AA ( Chest

infections)" s!rger' as soon as &ossible

f &atient as'm&tomatic9

6lective re&air %one b' M months ol% beca!se

of earl' %evelo&mene of irreversible
&!lmonar' h'&ertension

f # M months ol%" nee% for car%iac cath to

assess o&erabilit'

*!rgical AechniE!e9

Com&lete correction = &roce%!re of choice

Done on C2/

<2+ ban%" rarel' %one an% onl' if9

ntractable CC0 D active res&irator'

infection

-ncorrectable associate% lesions

4entric!lar imbalance
!trio&Ventricular Canal

2artial +4 canal or +*D 1I

7ess s'm&tomatic : re&air %one later b!t

i%eall' before 1 'ear

Done on C2/

+lwa's &atch clos!re D re&air left +4 valve

(mitral valve)
!trio&Ventricular Canal
Partial !V Canal
Closure Partial !V canal
Complete !V canal
'epair Complete !V canal
&
#
Closure
+nlet ;"D
Mitral
;alve
*epair
$
'epair Complete !V canal
/ricuspid
;alve
*epair
8
Closure
A"D &<
3
Congenital +ortic *tenosis

Congenital obstr!ction to the

flow from the left ventricle

Develo& 74 h'&ertro&h'

<a' %evelo& s'nco&e D chest

&ain of angina t'&e if stenosis
severe

+t later stage" 74 will fail with

%evelo&ment of CC0
Congenital !ortic Stenosis
Congenital !ortic Stenosis
Congenital +ortic *tenosis
+natomical t'&es9

+t valvar level9
abnormal or bic!s&i% aortic
valve

/elow the valve9

%iscrete s!baortic stenosis
(D*+*) = fibro!s membrane
with narrow orifice

+bove the valve

s!&ravalvar stenosis
Isolated Pulmonar Stenosis
2!lmonar' *tenosis

+bnormal &!lmonar' valve

&ro%!cing an obstr!ction to the
flow from the right ventricle

8bstr!ction can also e=ist

+
nf!n%ib!lar level
+
+rterial level9 <2+ stenosis
+
7evel of &eri&heral
&!lmonar' arteries

Develo& )4 h'&ertro&h'

)4 fail!re with increase% )+

&ress!re"
if &resence of 2089
)t 7t sh!nt ma' occ!r with
c'anosis
Pulmonar Stenosis
*;O/ patch
Canotic Congenital Heart
Defects
Tetralog of (allot
Aetralog' of 0allot (A80)

Definition of Aetralog' incl!%es

, elements9
O
7arge ventric!lar se&tal
%efect
O
+ortic overri%e
O
)ight ventric!lar o!tflow
tract obstr!ction
O
)4 h'&ertro&h'

Ahe )48A obstr!ction ma' be

inf!n%ib!lar" valvar or
s!&ravalvar
Aetralog' of 0allot
2atho&h'siolog'9
+Decrease% &!lmonar'
bloo% flow
+)t 7t sh!nting
+2ol'c'themia
+C'anotic s&ells
Contrast in=ected
in *; opacifies Ao
as well as M,A
Tetralog of (allot
Tetralog ) Clinical Signs
Canosis
Clubbing

/asic %efect = h'&o&lasia of )ight 4entric!lar

8!tflow Aract ()48A)

ncl!%es a wi%e s&ectr!m of )48A obstr!ction

nf!n%ib!lar stenosis onl'

+ssociate% stenosis of the &!lmonar' valve

*evere h'&o&lasia of )48A

H'&o&lasia of &!lmonar' ann!l!s an%

&!lmonar' tr!nF
Tetralog of (allot

Ahe e=treme form of A80 is N&!lmonar'

atresia with 4*DO
( = %!ctal %e&en%ant lesion)

Ahe more severe the )48A obstr!ction" the

earlier the &atient is s'm&tomatic" an% the
more severe the c'anosis
Tetralog of (allot

*!rgical techniE!es

Com&lete re&air i%eall' an% as earl' as

&ossible

Consists of clos!re of 4*D

1i%ening of )48A

2romotes alveologenesis an% angiogenesis

*!ccessf!l o!tcome %etermine% b' effective

relief of )48A obstr!ction
Tetralog of (allot

1i%ening of )48A

nf!n%ib!lar stenosis9 )48A &atch

nf!n%ib!lar stenosis D valvar stenosis9

)48A &atch D &!lmonar' valvotom'

H'&o&lastic &!lmonar' ann!l!s ( &!lmonar'

tr!nF9

Aransann!lar &atch

<onoc!s&

4alve% con%!it
Tetralog of (allot

2alliative *!rger'

+orto;&!lmonar' sh!nt in%icate%

Po!ng age9 !& to , months of life

H'&o&lastic 24 : <2+ : 2+ branches

Aransann!lar &atch onl' is other
alternative

+nomalo!s 7+D from )C+ crossing

)48A
Tetralog of (allot
Tetralog & Surger
*;O/ patch
+nfundibular stenosis onl
,ulmonar ;alvotom
;alvar stenosis
/ransannular patch
+nfundibular2 valvar and supravalvar stenosis
Tetralog & Surger
Tetralog & Surger
Hpoplastic *; outflow tract
*eplacement of *;O/ with
conduit 9> *astelli procedure:
Homograft
Contegra > bovine =ugular vein
Artificial conduit
Pulmonar !tresia
2!lmonar' +tresia

+bsence of &!lmonar' valve9

there is no o!tlet to )4

<a' or ma' not have a 4*D

Can onl' s!rvive if a 2D+

&ersists or if aorto;&!lmonar'
collaterals are &resent

+re severel' c'anose%

.ee% &alliative s!rger' earl' in

life" which consists of creation
of a s'stemic &!lmonar' sh!nt
(/A sh!nt)
Q 2/0
C'anosis
)eE!ire s'stemic;
&!lmonar' sh!nt
7ater reconstr!ction
of )4 o!tflow
Pulmonar !tresia
Pulmonar !tresia * VSD
Ductus dependant lesion
&( -/ shunt earl in life
#( Closure ;"D ? *astelli at
later stage

0!nctional single ventricle

1 well %evelo&e% ventricle

1 !n%er%evelo&e% ( h'&o&lastic ventricle

*ingle ventricle

<or&hological left

<or&hological right

H'&o&lastic ventricle often lacFs an

atrioventric!lar connection

6arl' %eath witho!t s!rger'

Single Ventricle

2atho&h'siolog' %e&en%s on

2resence or absence of obstr!ction to

&!lmonar' circ!lation

2resence or absence of obstr!ction to

s'stemic circ!lation

2resent in car%iogenic shocF

nfl!ence of &atent %!ct!s arterios!s

Single Ventricle

*&ectr!m of severit' of obstr!ction to

&!lmonar' bloo% flow

*evere obstr!ction Q 2/0

C'anosis : severit' of c'anosis %e&en%ant

of severit' of &!lmonar' o!tflow
obstr!ction

6=treme obstr!ction = &!lmonar' atresia

7ittle or no obstr!ction to &!lmonar' bloo%

flow 2/0
Single Ventricle

nitial management

Awo &resentations9

Decrease% &!lmonar' bloo% flow

K M months9 arterial;&!lmonar' sh!nt

# M months9 ?lenn

ncrease% &!lmonar' bloo% flow

<2+ ban%

0!rther management

?lenn (bi%irectional cavo;&!lmonar'

connection)

0ontan &roce%!re or mo%ification

Single Ventricle
Single Ventricle
Aric!s&i% +tresia

+bsence of tric!s&i% valve"

there is no &assage from )+ to
)4

)4 is h'&o&lastic onl'
f!nctional 74

8bligator' +*D" bloo% &asses

from )+ to 7+ an% then into
74

0rom 74" the bloo% goes into

the aorta" an% via a 4*D into
&!lmonar' arter'

<i=e% bloo% in 74 coming

from 7+ an% )+ chil% is
c'anose%
Aric!s&i% +tresia
n R$% c'anosis com&o!n%e%
b'9
+2resence of a restrictive
4*D
+2* ( &!lmonar' atresia
which marFe%l' %ecreases the
bloo% s!&&l' to the l!ngs
+.ee% &alliative s!rger'
earl' (/A sh!nt) beca!se of
severe c'anosis an% c'anotic
s&ells
Single Ventricle
Aric!s&i% +tresia

n 2$%" have large 4*D an% no 2*

+2resent with increase% &!lmonar' bloo% flow
an% &!lmonar' h'&ertension
+
.ee% &!lmonar' arter' ban%ing to &rotect the
l!ngs
Single Ventricle

?lenn sh!nt = s!&erior cavo&!lmonar'

anastomosis

Ahe %ifference with a s'stemic;&!lmonar'

arterial sh!nt is that a ?lenn sh!nt increases
&!lmonar' bloo% flow an% s'stemic arterial
sat!rations witho!t increasing the vol!me
loa% on the s'stemic ventricle
+lenn s#unt
+lenn s#unt
@lenn shunt or superior
cavo7pulmonar anastomosis
";C connected to *,A
";C
*,A
*A
M,A
-C/
";C
*,A

0ontan = total cavo;&!lmonar' connection

2rinci&al %eterminants of s!ccess of a 0ontan;t'&e

connection are

-nobstr!cte% &athwa's between s'stemic veins

an% &!lmonar' arteries

2reservation of anatomic an% f!nctional integrit'

of &!lmonar' vasc!lat!re

7ow 2+2 an% 24)

.o %istortion of &!lmonar' arteries

+%eE!ate f!nction of s'stemic ventricle

(ontan
(ontan
.ontan or total cavo7
pulmonar anastomosis
Man modifications
";C
+;C
connected
to *,A
+;C
Ao
*,A
(ontan
)ateral tunnel .ontan
Axtracardiac .ontan
Transposition of t#e +reat
Vessels
Arans&osition of the ?reat
4essels (A?4)

+orta arises from )4

2!lmonar' arter' arises from 74

Ahe 2 circ!lations are sit!ate% in

&arallel incom&atible with life !nless
some mi=ing of the bloo% occ!rs at9
+
+trial level (+*D)
+
4entric!lar level (4*D)
+
?reat vessel level (2D+)

2resent with &!lmonar' h'&ertension"

c'anosis an% CC0 an% reE!ire !rgent
corrective s!rger' earl' in life
T+V & Surger
Mustard ,rocedure
Atrial "witch or
,hsiological Correction
,ulmonar venous atrium 9>)A:
/; *; Aorta
"stemic venous atrium 9>*A:
M; ); M,A
T+V & Surger
Arterial "witch or Anatomical Correction
Truncus !rteriosus
Ar!nc!s +rterios!s

Has onl' a single semi;l!nar valve

(calle% tr!ncal valve) locat% above
a large 4*D" the aorta an%
&!lmonar' valve arise from this
common tr!nF

2!lmonar' arter' &ress!re is eE!al

to aortic &ress!re" with greatl'
increase &!lmonar' bloo% flow an%
&!lmonar' h'&ertension

2resent as neonates with c'anosis

an% CC0

.ee% corrective s!rger' earl' in

life
Truncus !rteriosus
&( Closure ;"D
#( *astelli
procedure
&
# $
3
8
Total !nomalous Pulmonar Venous
Connection
Aotal +nomalo!s 2!lmonar' 4eno!s
Connection (A+24C)

+ll &!lmonar' veins connect to a common

&!lmonar' veno!s chamber" which
em&ties into *4C or )+ or 4C

.o &!lmonar' veins are connecte% to 7+

/loo% can onl' go into 7+ an% 74 via

+*D

Com&lete mi=ing of &!lmonar' an%

s'stemic veno!s ret!rn with &ress!re an%
vol!me overloa% of the right heart

Corrective s!rger' is an emergenc'

Tpes of T!PVC
Total !nomalous Pulmonar Venous
Connection
&
#
$
3
Interventional Cardiolog

Creation of +*D

/alloon atrial se&tostom' (in A?4)

/la%e atrial se&tostom'

/alloon valv!lo&last'

Ao manage stenotic valves (+*;2*;<*;A*)

/alloon angio&last' an% stents

n Coarctation"

n h'&o&lastic or stenotic &!lmonar' arteries

Device clos!re of 2D+ : +*D ; 4*D