Principle of Congenital Heart Diseases

8/20/2019 Principle of Congenital Heart Diseases

1/76

03/05/1603/05/16 11

PRINCIPLE OFPRINCIPLE OF

CONGENITAL HEARTCONGENITAL HEARTDISEASES (CHD)DISEASES (CHD)NoormantoNoormantoDepartment of PediatricDepartment of PediatricUniversity of Gadjah MadaUniversity of Gadjah Mada

Yo ya!arta/ "ardjito Genera# Yo ya!arta/ "ardjito Genera#$ospta#$ospta#


2/76

03/05/16 2


3/76

03/05/16 3


4/76

03/05/16 4

Dimensions of the cardiac cham%ersDimensions of the cardiac cham%ers& 'he 'he () is #ar er than the *)() is #ar er than the *) +() hand#es+() hand#es

55,- *) hand#es .5,55,- *) hand#es .5,& 'he 'he press repress re in thein the () is identica#() is identica# to thatto that

in thein the *)*)

2eta# cardiac o tp t2eta# cardiac o tp t&

*o comp#iance of the heart*o comp#iance of the heart

na%#e tona%#e to

increase stro!e vo# meincrease stro!e vo# me& 4f the heart rate drops4f the heart rate drops fa## cardiacfa## cardiac

o tp to tp t feta# distressfeta# distress + P 7 ") 8 $(+ P 7 ") 8 $(


5/76

03/05/16 5

9fter %irth:9fter %irth:& 4nter pted of the m%i#ica# cord4nter pted of the m%i#ica# cord

*ac! of %#ood ret rn from the p#acenta*ac! of %#ood ret rn from the p#acenta4ncrease in systemic vasc #ar resistance4ncrease in systemic vasc #ar resistance

& * n e8pansion* n e8pansion& Decrease of the P)(Decrease of the P)( fa## P9 press refa## P9 press re

increase p #monary %#ood ;oincrease

p #monary %#ood ;o 4ncrease p #monary veno s ret rn4ncrease

p #monary veno s ret rn increaseincreaseof the *9 press reof the *9

press re f nctiona# c#os re of thef nctiona# c#os re of theforamen ova#eforamen ova#e

& 4ncrease of arteria# < sat ration4ncrease of arteria# < sat ration PD9 c#osedPD9 c#osed


6/76

03/05/16 6

#os re of the d ct s arterios s#os re of the d ct s arterios s& 2 nctiona# : 10 & 15 ho rs after %irth2 nctiona# : 10 & 15 ho rs after %irth& 9natomic c#os re comp#ete < =3 ee!s9natomic c#os re comp#ete < =3 ee!s& 4ncrease of < sat ration4ncrease of < sat ration constriction of theconstriction of the

d cta# smooth m sc#ed cta# smooth m sc#e 'he responsiveness of the d cta# smooth m sc#e 'he responsiveness of the d cta# smooth m sc#edepend on estationa# a e +f ## term moredepend on estationa# a e +f ## term moreresponsiveness than pretermresponsiveness than preterm

& Decrease in PG>< #eve# after %irthDecrease in PG>< #eve# after %irth constriction ofconstriction ofthe d ctthe d ct

& 4ndomethacin +prosta #andine synthetase inhi%itor4ndomethacin +prosta #andine synthetase inhi%itor can %e sed to c#ose PD9 in preterm infantscan %e sed to c#ose PD9 in preterm infants


7/76

03/05/16 7

IVC

SVC

RA5

25/5

LA 6

110/6

AO25/15

110/80

Dia ram of norma# heart


8/7603/05/16 8

Classif a!i"# "$ CHDClassif a!i"# "$ CHD

NON C%ANOTIC&NON C%ANOTIC&& I# ' as *l+"#a', -l"". "I# ' as *l+"#a', -l"". "

ASD (A!'ial S !al D $ !)ASD (A!'ial S !al D $ !)VSD (V #!'i *la' s !al . $ !)VSD (V #!'i *la' s !al . $ !) PDA (Pa! #! D* !*s A'! 'i"s*s)PDA (Pa! #! D* !*s A'! 'i"s*s)

& N"'+al *+"#a', -l"". "N"'+al *+"#a', -l"". "P*l+"#a', s! #"sis (PS)P*l+"#a', s! #"sis (PS)

C%ANOTIC&C%ANOTIC&& D ' as *l+"#a', -l"". "D ' as *l+"#a', -l"". "

T"F (T !'al" , "$ Fall"!)T"F (T !'al" , "$ Fall"!)P*l+"#a', A!' sia (PA)P*l+"#a', A!' sia (PA)

& I# ' as *l+"#a', -l"". "I# ' as *l+"#a', -l"". "TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )


9/7603/05/16 9

A!'ial S !al D $ !A!'ial S !al D $ !

(ASD)(ASD)Preva#encePreva#ence

&5 & 10 , a## $D5 & 10 , a## $D

&Ma#e : fema#e 7 1 :


10/76

101003/05/1603/05/16

T%PE OF ASD


11/76

03/05/16 11

(9

*9

()

*)

Pathophysio#o y


12/76

03/05/16 12

(9

()

*9

*)

9

P9

) P)


13/76

03/05/16 13

#inica# manifestations:#inica# manifestations:& Us a##y asymtomatic +infant and chi#drenUs a##y asymtomatic +infant and chi#dren& (ec rrent #o er respiratory tract infection +*('4(ec rrent #o er respiratory tract infection +*('4

& De#ayed ro th and deve#opmentDe#ayed ro th and deve#opment& 9 Bide#y sp#it and C8ed "


14/76

03/05/16 14

=ray=ray& ardiome a#yardiome a#y& Prominent P9Prominent P9& 4ncreased p #monary vasc #ar mar!in s4ncreased p #monary vasc #ar mar!in s

>chocardio raphy>chocardio raphy& 'ype- position and siEe of the defect 'ype- position and siEe of the defect& "h nt direction"h nt direction& >n#ar ement of the cham%ers>n#ar ement of the cham%ers& )a#ves)a#ves& Press rePress re


15/76

•Right atrial enlargement•Right ventricle hypertrophy•Prominence the MPAsegment• ncrease! p"lmonary

vasc"lar mar#ing

Chest X-Ray


16/76

03/05/16 16

3a#a + #!3a#a + #!

Medica#Medica# ::& con estive heart fai# re + $2con estive heart fai# re + $2 anti fai# reanti fai# re

" r ery" r ery ::& Preschoo# a ePreschoo# a e

Non s r ica# c#os reNon s r ica# c#os re ::& 'ranscatheter c#os re sin 9" +9mp#atEer "epta# 'ranscatheter c#os re sin 9" +9mp#atEer "epta#

cc# dercc# der


17/76

'ranscatheter c#os re sin 9"

A!'ial S !al DA!'ial S !al D

9" D>)4 >


18/76

03/05/16 18

A!'ial S !al DA!'ial S !al D


19/76

03/05/16 19


20/76

03/05/16 $0


21/76

03/05/16 $1



ASD (A!'ial S !al D $ !)ASD (A!'ial S !al D $ !)

VSD (V #!'i *la' s !al . $ !)VSD (V #!'i *la' s !al . $ !) PDA (Pa! #! D* !*s A'! 'i"s*s)PDA (Pa! #! D* !*s A'! 'i"s*s)

& N"'+al *+"#a', -l"". "N"'+al *+"#a', -l"". "P*l+"#a', s! #"sis (PS)P*l+"#a', s! #"sis (PS)





22/76

03/05/16 $$

)entric #ar "epta# Defect)entric #ar "epta# Defect

+)"D+)"DPrevalencePrevalence % 15 % $0 & all '()15 % $0 & all '()

AnatomyAnatomy % ** ubarterial defect ubarterial defect + ,elo- p"lmonary an! aortic+ ,elo- p"lmonary an! aortic

valvevalve % Perimem,rano"s !e.ectPerimem,rano"s !e.ect + ,elo- aortic+ ,elo- aortic valvevalve

at pars mem,rano"s sept"mat pars mem,rano"s sept"m % M"sc"lar !e.ectM"sc"lar !e.ect

V #!'i *l ' S ! lV #!'i *l ' S ! l


23/76

03/05/16 $3

V #!'i *la' S !alV #!'i *la' S !al

V #!'i *l ' S ! lV #!'i *l ' S ! l


24/76

03/05/16 24


COP 4 SV HR

(9

*9

()

*)

110/F


25/76

03/05/16 25


P)( 7 p #monary vasc #ar resistant()P 7 ri ht ventric #ar press re*)$/()$ 7 #eft/ri ht ventric #ar press re


26/76

03/05/16 $6

#inica# manifestation#inica# manifestation& 9symtomatic9symtomatic symtomaticsymtomatic&

(ec rrent *('4(ec rrent *('4& De#eyed ro th and deve#opmentDe#eyed ro th and deve#opment& "i n of $2"i n of $2& Pansysto#ic m rm rPansysto#ic m rm r

>#ectrocardio raphy>#ectrocardio raphy& *)$- com%ine *)$ and ()$*)$- com%ine *)$ and ()$


27/76

03/05/16 $

=ray=ray& ardiome a#yardiome a#y& Prominent P9Prominent P9& 4ncrease p #monary vasc #ar mar!in4ncrease p #monary vasc #ar mar!in

>chocardio raphy>chocardio raphy& 'ype- position and siEe of the defect 'ype- position and siEe of the defect& "h nt direction"h nt direction

& >n#ar ement of the cham%ers>n#ar ement of the cham%ers& Press re ca#c #ationPress re ca#c #ation



28/76

03/05/16 $8

1 Ca'.i"+ al,2 A ." # a'.

7 P'"+i# # *l+"#a',a'! ', s + #! I# ' as . *l+"#a', 9as

+a':i#

V #! i *la S !alV #! i *la S !al

3

.

<

a

%c

ardiome a#y if '( 0 5

'( 7 aH%/c


29/76

03/05/16 $9

3a#a + #!3a#a + #!

Me!icalMe!ical % '('( anti.ail"reanti.ail"re

)e.initive)e.initive % *) clos"re*) clos"re

*"rgery*"rgery2ranscatheter clos"re2ranscatheter clos"re


30/76

03/05/16 30

Transcatheter closure of Perimembranous VSD


31/76

03/05/16 31



ASD (A!'ial S !al D $ !)ASD (A!'ial S !al D $ !)VSD (V #!'i *la' s !al . $ !)VSD (V #!'i *la' s !al . $ !)

PDA (Pa! #! D* !*s A'! 'i"s*s)PDA (Pa! #! D* !*s A'! 'i"s*s)& N"'+al *+"#a', -l"". "N"'+al *+"#a', -l"". "

P*l+"#a', s! #"sis (PS)P*l+"#a', s! #"sis (PS)


T"F (T !'al" , "$ Fall"!)T"F (T !'al" , "$ Fall"!)P*l+"#a', A!' sia (PA)P*l+"#a', A!' sia (PA)& I# ' as *l+"#a', -l"". "I# ' as *l+"#a', -l"". "

TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )


32/76

03/05/16 3$

Patent D ct s 9rterios sPatent D ct s 9rterios s

+PD9+PD9Patho#o yPatho#o y& Persistent patency of d ct s arterios sPersistent patency of d ct s arterios s

+a norma# feta# str ct re %et een the+a norma# feta# str ct re %et een the*P9 and the descendin aorta*P9 and the descendin aorta

(is! 2actor(is! 2actor& Premat rePremat re

& 9sphy8ia9sphy8ia& * n diseases* n diseases& 4nf sion of PG>14nf sion of PG>1

Pa! #! D* !*s A'!Pa! #! D* !*s A'!


33/76

03/05/16 33

COP 4 SV HR

Pa! #! D .! s A !Pa! #! D .! s A !

(9

*9

()

*)

110/F0


34/76

03/05/16 34

)

(9

()

*9

P)

*)

9

P9PD9


35/76

03/05/16 35

Preva#encePreva#ence& 5 & 10, a## $D5 & 10, a## $D& Ma#e : fema#e 7 1 : 3Ma#e : fema#e 7 1 : 3

#inica# manifestations#inica# manifestations& 9symtomatic = symtomatic9symtomatic = symtomatic& (ec rrent *('4(ec rrent *('4& Poor ei ht ainPoor ei ht ain

& "i n of $2"i n of $2& Io ndin periphera# p #ses +pisto# shoot si nIo ndin periphera# p #ses +pisto# shoot si n& ontino r m rm rontino r m rm r


36/76

03/05/16 36

>#ectrocardio raphy 7 )"D>#ectrocardio raphy 7 )"Dhest =ray 7 )"Dhest =ray 7 )"D

>chocardio raphy>chocardio raphy& 'ype- position and siEe of the defect 'ype- position and siEe of the defect& "h nt direction"h nt direction& >n#ar ement of the cham%ers>n#ar ement of the cham%ers& )a#ves)a#ves& Press rePress re


37/76

03/05/16 3

Mana ementMana ement

4ndomethacin in premat re infant4ndomethacin in premat re infant 'ranscatheter c#os re/ non s r ica# 'ranscatheter c#os re/ non s r ica#

c#os re:c#os re:& oi# em%o#iEationoi# em%o#iEation& 9mp#atEer d cta# occ# der +9D9mp#atEer d cta# occ# der +9D

" r ica# c#os re" r ica# c#os re


38/76

03/05/16 38

T'a#s a! ! ' PDAT'a#s a! ! ' PDA

" l*si"#s" l*si"#s(ash!ind m%re##a device(ash!ind m%re##a device"tain#ess stee# coi#s"tain#ess stee# coi#s

9mp#atEed d cta# occ# der9mp#atEed d cta# occ# der


39/76

03/05/16 39


40/76

03/05/16 40

Pa! #! D*.!*s A'!Pa! #! D*.!*s A'!


41/76

03/05/16 41

Gia#!*' " "ilsGia#!*' " "ils

Pa! #! D*.!*s A'!Pa! #! D*.!*s A'!


42/76

03/05/16 4$

A+ la!; ' D* !alA+ la!; ' D* !al

O l*. ' (ADO)O l*. ' (ADO)

Pa! #! D*.!*s A'!Pa! #! D*.!*s A'!


43/76

03/05/16 43

arge P)A


44/76

03/05/16 44


45/76

03/05/16 45


46/76


47/76

03/05/16 4





P*l+"#a', s! #"sis (PS)P*l+"#a', s! #"sis (PS)C%ANOTIC&C%ANOTIC&& D ' as *l+"#a', -l"". "D ' as *l+"#a', -l"". "

T"F (T !'al" , "$ Fall"!)T"F (T !'al" , "$ Fall"!)P*l+"#a', A!' sia (PA)P*l+"#a', A!' sia (PA)& I# ' as *l+"#a', -l"". "I# ' as *l+"#a', -l"". "

TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )


48/76

03/05/16 48

P*l+"#a', S! #"sisP*l+"#a', S! #"sis

4ncidence : F=10,4ncidence : F=10,

9natomy:9natomy:P #monary stenosis va#v #ar :P #monary stenosis va#v #ar :& Iic spid p #monary va#veIic spid p #monary va#ve& )a#ve #ea;et thic!enin and adhession)a#ve #ea;et thic!enin and adhession

P #monary stenosis inf ndi% #arP #monary stenosis inf ndi% #ar

$yperthropy inf ndi% # m$yperthropy inf ndi% # m


49/76

03/05/16 49

#inica# Cndin s#inica# Cndin s)a#v #ar s)a#v #ar s tenosistenosis Mi#d :Mi#d : >jection systo#ic>jection systo#ic m rm rm rm rBide jection c#ic! + = >jection c#ic! + = 11 stst $" norma#-


50/76

03/05/16 50

Mil! + e ection systolic m"rm"r

$ n! (* -i!e splite ection clic#

Mo!erate+ e ection systolic m"rm"r early e ection clic#*evere + e ection systolic m"rm"r clic# e ection 7

P*l+"#a', S!P*l+"#a', S!


51/76

03/05/16 51

KNorma# or mi#dcardiome a#yKMar!ed

p #monary va#vepost stenoticdi#atationKNorma#p #monaryvasc #arity


52/76

03/05/16 5$

> G : (9D> G : (9D - ()$- ()$>chocardio rahhy : conCrmation dia nosis>chocardio rahhy : conCrmation dia nosis

atheteriEation: increased () press reatheteriEation: increased () press reitho t increased o8y en sat rationitho t increased o8y en sat ration


53/76

03/05/16 53


Medicamentosa : se#essMedicamentosa : se#essMi#d stenosis: intervention +=Mi#d stenosis: intervention +=Moderate stenosis: o%servationModerate stenosis: o%servation"evere stenosis: %a##oon va#v #op#asty"evere stenosis: %a##oon va#v #op#asty

P*l+"#a', S!P*l+"#a', S!


54/76

03/05/16 54

P*l+"#a', S!P*l+"#a', S!


55/76

03/05/16 55

Before ballooning

D ring b llooningP*l+"#a', S!P*l+"#a', S!


56/76

03/05/16 56

During ballooning


57/76

03/05/16 5










58/76

03/05/16 58

Tetralogy of FallotTetralogy of Fallot

K. Baumgartner,Freiburg 1839

A boy with congenitalheart disease

T !'al" , "$ FT !'al" , "$ F


59/76

03/05/16 59

(9

*9

()

*)

I#si. #I#si. #

5=F, from a## $D5=F, from a## $D

A#a!"+,A#a!"+,+-',"l" ,&+-',"l" ,&

a se: *eft=anteriordeviation ofinf ndi% #ar sept m

Si#.'"+a "#sis! "$Si#.'"+a "#sis! "$ i! +s& i! +s&

11 p #monary stenosisp #monary stenosis


60/76


61/76

03/05/16 61

#inica##y :#inica##y :yanosisyanosis

Dyspnea on e8ertionDyspnea on e8ertion"L attin"L attin$istory of hyper=cyanotic spe##$istory of hyper=cyanotic spe##

# %%in Cn er# %%in Cn er

"in #e


62/76

03/05/16 6$

•*ingle $ n! (*• ection systolic m"rm"r

T !'al" , "$ FT !'al" , "$ F


63/76

03/05/16 63

( :( :Ioot=shapedIoot=shaped

oncaveoncave

p #monaryp #monaryse mentse ment9pe8 pt rned9pe8 pt rnedDecreasedDecreasedp #monaryp #monary%#ood ;o%#ood ;o


64/76

03/05/16 64

>#ectrocardio raphy>#ectrocardio raphy& (9D(9D& ()$()$

>chocardio raphy>chocardio raphy& 'o conCrm dia nosis 'o conCrm dia nosis& Prepare to s r ery +e8chan e toPrepare to s r ery +e8chan e to

catheteriEationcatheteriEationP #monary stenosisP #monary stenosis)"D)"D9ortic over=ridin9ortic over=ridin()$()$


65/76

03/05/16 65


Pa#iative treatmentPa#iative treatment&I#a#oc!='a ssi sh ntI#a#oc!='a ssi sh nt

DeCnitiveDeCnitive&tota# correction- the %asis oftota# correction- the %asis of

morta#ity and physio#o ica#morta#ity and physio#o ica#

o tcomeo tcome optima# a e for repairoptima# a e for repair3=11 months3=11 months

)an 9rsde## G" et a#-


66/76

03/05/16 66


67/76


68/76

03/05/16 68








& I# ' as *l+"#a', -l"". "I# ' as *l+"#a', -l"". "TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s)TGA (T'a#s "si!i"# "$ ! G' a! A'! 'i s) TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )TAPVD (T"!al A#"+al"*s P*l+"#a', V #"*s D'ai#a )

f


69/76

03/05/16 69

Transposition of TheTransposition of The

Great ArteryGreat ArteryA#a!"+,A#a!"+,9%norma#ity of formation of tr n!a#9%norma#ity of formation of tr n!a#

sept m that ca se aorta arisin fromsept m that ca se aorta arisin from() and P9 arisin from() and P9 arisin from LVLV

T'a#s "si!i"# "$ T G' aT'a#s "si!i"# "$ T G' a


70/76

03/05/16 0

Bith )"D Bitho t )"D +"imp#e 'G9


71/76

03/05/16 1

Hemodynamic normal Hemodynamic of TGA“series” “parallel”

P2


72/76

03/05/16 $

TGA without !"

#n ade$uate %i&in' Ade$uate %i&in'

T'a#s "si!i"# "$ T G' aT'a#s "si!i"# "$ T G' a


73/76

03/05/16 3

':R +• 'ar!iomegaly• gg7on7si!e

heart• ncrease!

p"lmonaryvasc"lar

mar#ing

MM


74/76

03/05/16 4



75/76

03/05/16 5


76/76

Principle of Congenital Heart Diseases

Documents