Overview of Congenital Heart Diseases, Students

8/12/2019 Overview of Congenital Heart Diseases, Students

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Overview of CongenitalOverview of CongenitalHeartHeart DiseasesDiseases

Dr. K. Vanderdonck

Charlotte Maxeke Johannesburg Academic Hospital

Universit of the !itwatersrand

"AHA #$%&'%#'&&


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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 chilren born withCHD sho!l reach a!lt life if treate

a&&ro&riatel' Correction re&air

ole of &alliation

nterventional cariolog'

Incidence


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n *+"

# 10 000 chilren born each 'ear with CHD

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

CHD res&onsible for 1.2% of !ner $mortalit'

-nerestimate beca!se man' ie!niagnose

Incidence


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ormal l!ng mat!ration

/egins at en of &regnanc'

Contin!es for some time after birth

ormation of alveoli = a &ostnatal event

ith initiation of s&ontaneo!s ventilation

emoeling an mat!ration of &!lmonar'

vasc!lat!re

Dro& of + (&!lmonar' arter')&ress!re 3 4(&!lmonar' vasc!lar resistance)

rocess com&lete b' , months of age

Heart & Lungs


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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 remoeling of the&!lmonar' vasc!lar be

Heart & Lungs


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ncrease &!lmonar' bloo flow (/)ormal remoeling of neonatal vasc!lat!re

oes not occ!r

7eas to &!lmonar' h'&ertension anincrease &!lmonar' vasc!lar resistance (4)

Develo&ment of &!lmonar' vasc!lar

obstr!ctive isease (4D)

4 initiall' reversible

-no&erate" 4 becomes irreversible

6isenmenger s'nrome

Heart & Lungs


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6isenmenger s'nrome9

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

which is irreversible

Have s!&ras'stemic + &ress!res an 4

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

ncreasing c'anosis

Death

Heart & Lungs


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ncrease &!lmonar' bloo flow

Characteristic tria of s'm&toms

e&eate chest infectionsCongestive cariac fail!re

ail!re to thrive

resent in all &atients with increase /: ac'anotic an c'anotic

Heart & Lungs


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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 arteriesC'anosis

Heart & Lungs


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Diagnosis


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6CH

ow the mainsta' of iagnosis

Done b' a &aeiatric cariologist

,,% rate of ma@or iagnostic errors when

&atient referre from a!lt 6cho lab

A66 gives aitional information abo!t

intracariac anatom' ntrao& A66

Diagnosis


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Cariac catheteriBation

*till stanar for9


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Diagnosis

Cariac CatheteriBation an +ngiogra&h'9

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

Ao assess o&erabilit'

Ao assess + 4 an res&onse to o'gen on

&!lmonar' vasc!lat!re

4 # 8 oo !nits in 100% o'gen constit!tes a

contra;inication to s!rger'


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m&ortance of foetal echocariogra&h'an

&renatal iagnosis

Ao &re&are famil' for hos&ital amission

an &lan s!rgical intervention

Ao give the o&tion of terminating the

&regnanc' if the &rognosis is &oor

Diagnosis


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Classification

Anomalies

Charateristic

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


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Treatment


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Ductus dependant lesions

rostaglanin 61 (+l&rostatil) o&ens an maintains&atenc' of the !ct!s arterios!s

or &!lmonar' bloo flow

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

*evere tetralog'*ingle ventricle

or miing

A?4

or s'stemic bloo flowH7H* Critical +*

++ *evere coarctation


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Palliative Procedures


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rovies s'm&tomatic relief (!s!all' tem&orar')b!t leaves the lesion !ncorrecte

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

Designe to increase &!lmonar' bloo flow ina c'anotic chil with inaeE!ate &!lmonar'bloo flow

+trial se&tectom'

Designe to increase miing at atrial level!lmonar' arter' baning


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*ight

-/

shunt

&433

)eft

-/

shunt

*ight

modified-/ shunt

&456 Central

"hunt

Aorto7pulmonar "hunts or Arterial "hunts


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!aterston shunt

&46#

,otts shunt &436



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,ulmonar Arter

-anding

+ ban9

Designe to limit/ in a chil

with ecessive&!lmonar' blooflow


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Closed Heart Surger


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remat!re infants9

*&ontaneo!s clos!re is common

Ar' nomethacine first if chil

s'm&tomatic*!rgical 7igation

Patent Ductus !rteriosus


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*!rgical Clos!re : 7igation or Division

*&ontaneo!s clos!re not common when

term infants is !n!s!al after the first few

months of life

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

*'m&toms = CC AA Chest

infections



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*!rgical Clos!re : 7igation or Division

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

net 5 months

Ao &revent */6

f &resence of HA9 ma' have to be

catheteriBe



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PD! ligation " division

)eft thoracotom 3th+C"

)igation

Division

& #


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PD! transcat#eter closure

nterventional

Cariolog'9

m&lantation of

+m&latBer evice to

occl!e D+


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Coarctation

Coarctation of the +orta(Co+)

arrowing of the aortic l!menistal to the origin of the lefts!bclavian arter'

Have h'&ertension in the&roimal arterial tree (!&&erlimbs) an h'&otension anweaF &!lses in the lower limbs

n a!lthoo" !e to severe

s'stemic h'&ertension" will havecerebrovasc!lar accients


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+!lt t'&ecoarctation9

ftenas'm&tomatic

*!rger' inicateas soon as

iagnosis mae

Adult Coarctation or postductal

Coarctation


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eonatal coarctation9

Has a ifferent

&resentation9

Cariovasc!lar

colla&se

*evere metabolic

aciosis+t the time of !ctal

clos!re

+nfantile Coarctation orpreductal

Coarctation


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eonatal coarctation

revio!sl' associate with high mortalit'

-se of &rostaglanins has change o!tcome

*emi;!rgent s!rger' inicate once chil

res!scitate an stabilise

Coarctation


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*!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

atch angio&last'

Coarctation


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Surger Coarctation

*esection and end to endanastomosis


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Surger Coarctation

"ubclavian flap

,atch Angioplast

&

#

&

#


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nterventional Cariolog'9

/alloon ilatation an or stenting

6s&eciall' in rec!rrent Co+

*ometimes in native Co+

n oler &atients

f aortic arch is of aeE!ate siBe

Coarctation


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$pen Heart Surger& Cardiopulmonar %pass


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Cardiopulmonar %pass


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Cardiopulmonar %pass

Median sternotom

Arterial and venous

cannullas placed for

cardiopulmonar bpass


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!trial Septal Defect

+trial *e&tal Defect (+*D) Comm!nication between left

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

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

res!lting in large &!lmonar'bloo flow

ften as'm&tomatic in chilhoob!t ma' have freE!entres&irator' infections

Can evelo& &!lmonar'h'&ertension in a!lthoo(!s!all' moerate)


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


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Histor'

1stclos!re in 1G$2 (ohn 7ewis) !sing

h'&othermia inflow occl!sion

1stefect to be re&aire on C/ in 1G$5(ohn ?ibbon) !sing a &!m& o'genator

1stintracariac efect to be s!ccessf!ll' manage

with &erc!ataneo!s transcatheter techniE!es



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*!rger'

Done on C/

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

+!tologo!s &ericari!m

/ovine &ericari!m

nterventional Cariolog'

nl' +*D 2I" if small to moerate siBe" an

rim &resent



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Closure of !SD


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4entric!lar *e&tal Defect +natom'9 , t'&es

Perimembranous(B)= 80%-nerneath antero;se&talcommiss!re of the tric!s&ivalve

Muscular (D)ften m!lti&le

Infundibular / Outlet (A)+ssociate aorticreg!rgitation

Inlet (C)solate or &art of +4 canal

Ventricular Septal Defect


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*mall 4*D

ormal + &ress!re normal 4

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

4*D : close s&ontaneo!sl'


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7arge 4*D

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

*h!nt e&enent on egree of 4

Clos!re in infants9 f s'm&tomatic (CC AA Chest

infections)

f fail!re of meical treatmentf chil # 1 'ear9 nees cariac cath to

assess o&erabilit' (4 K 8 oo !nits + reactive to

2)

Ventricular Septal Defect


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+4 canal efects malaligne 4*DLs reE!ires!rgical clos!re (o not close s&ontaneo!sl')

Contra;inications to s!rger'9


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VSD Surger

&( *ight Atrium

opened

#( /ricuspid ;alve

retracted

$( ;"D exposed

3( Margins of ;"D

carefull assessed


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VSD Surger

,atch sutured in

place


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+trio;4entric!lar Canal +lso calle enocarial c!shion

efect

6ists as &artial or com&lete

+4 canal artial +4 canal9 &resenceof +*D 1I onl'

Com&lete +4 canal9&resence of +*D 1I an inlet4*D

arel' isolate inlet +4canal t'&e 4*D

!trioVentricular Canal


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+trio;4entric!lar Canal +lso have abnormal mitral an

tric!s&i valves an ma' havemitral anor tric!s&i

reg!rgitation Com&lete +4 canal !s!all' has

large 7t t sh!nt with severe&!lmonar' h'&ertension

reE!ent in DownLs s'nrome



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Com&lete +4 canal9

f &atient s'm&tomatic (CC AA Chest

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

f &atient as'm&tomatic96lective re&air one b' M months ol beca!se

of earl' evelo&mene of irreversible

&!lmonar' h'&ertension

f # M months ol" nee for cariac cath to

assess o&erabilit'


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*!rgical AechniE!e9

Com&lete correction = &roce!re of choice

Done on C/


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artial +4 canal or +*D 1I

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

ieall' before 1 'ear

Done on C/

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

(mitral valve)



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Partial !V Canal


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Complete !V canal


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'epair Complete !V canal&

#

Closure

+nlet ;"D Mitral

;alve*epair

$


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'epair Complete !V canal

/ricuspid

;alve

*epair

8

Closure

A"D &*A:

M; ); M,A

T+V Surger


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T+V Surger

Arterial "witch or Anatomical Correction


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Truncus !rteriosus


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Truncus !rteriosus

&( Closure ;"D

#( *astelli

procedure

$

3

8

Total !nomalous Pulmonar Venous


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Connection

Aotal +nomalo!s !lmonar' 4eno!sConnection (A+4C)

+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 miing 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


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Tpes of T!PVC

Total !nomalous Pulmonar Venous


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Connection

& #

$3

Interventional Cardiolog


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Interventional Cardiolog

Creation of +*D/alloon atrial se&tostom' (in A?4)

/lae atrial se&tostom'

/alloon valv!lo&last'Ao manage stenotic valves (+*;*;

Overview of Congenital Heart Diseases, Students

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