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8/18/2019 (Drindriwanto) HCD - 19 September 2012
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C&''I*A+ HA*C&''I*A+ HA*
DISASDISAS
Anomalies of the heart structure andcirculatory function which is present since
birth due to disturbances or failure in thedevelopment of the heart during early fetallife
Incidence : 8 – 10 per 1000 live births
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O SURGICAL
A D
SURGICAL
I TERVE TIO
O SURGICAL
A D
SURGICAL
I TERVE TIO
UNDERSTANDUNDERSTAND
CARDIOVASCULAR CARDIOVASCULAR
ANATOMYANATOMY
PHYSIOLOGYPHYSIOLOGY
PATHOPHYSIOLOGYPATHOPHYSIOLOGY
HistoryHistory
Physical ExamiatioPhysical Examiatio
ECGECGCh!st "#RayCh!st "#Ray
Echocar$io%ra&hyEchocar$io%ra&hy
Car$iac Cath!t!ri'atioCar$iac Cath!t!ri'atio
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(o)l!$%! o* *!tal a$ &!riatal circ+latio is h!l&*+l i
+$!rsta$i% th! cliical mai*!statios a$ at+ral history o* CHD
CHANGES IN CIRCULATION
A,TER -IRTHShift of blood flow for gas exchange from placenta to the lungs
1. Interruption of the umbilical cord• Increase of SVR
• Closure of ductus venosus2. ung expansion
• Reduction of !VR • "unctional closure of !"#• Closure of !$%
CHANGES IN CIRCULATION
A,TER -IRTHShift of blood flow for gas exchange from placenta to the lungs
1. Interruption of the umbilical cord• Increase of SVR
• Closure of ductus venosus2. ung expansion
• Reduction of !VR • "unctional closure of !"#• Closure of !$%
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Fetal
Circulation
hunts:
1! "lacenta#! $uctus %enosus&! Foramen 'vale(! $ustusArteriosus
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)ormal
"ost*)atalCirculation
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"+,-')A./ %AC+,A. ".+.
A)$ .IA)C
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,C.'CA.$I'2.
A-
ADULT
EO ATE
I FA T
RV dominant
V dominant
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HA* A$SC$+*A*I&'
HEART SOUNDS AND MURMURS
vibrations produced b& cessation or
propulsion of blood within the heart
radiates through the thorax s'in
stethoscopeevaluation of heart disease
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3A. '+)$
• ,irst H!art So+$ .S/0 closure of the (itral and )ricuspid valves
• S!co$ H!art So+$ .S10 closure of the %ortic and !ulmonar& Valves
splitting of the S2 %2 procedes !2 splits with respiration
• Thir$ H!art So+$ .S20 earl& in diastole rapid filling phase of the ventricle
heard in normal children
• ,o+rth H!art So+$ .S30 atrial contraction rare in children
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3A. -+.-+.
• T+r4+l!t 4loo$ *lo) produces vibrations
stenosis
regurgitation shunt
• Classi*icatiotiming during cardiac c&cle
* s&stolic murmur * diastolic murmur * continuous murmur
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C3'CA.$I'2.A"3/
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CH&CADI&APHCH&CADI&APH - -
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CA.$IACCA3.I4AI')
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Congenital Heart Disease
Acyanotic/noncyanotic
cyanotic
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)on Cyanotic,eft to .ight hunt
• Atrial eptal $efect
• %entricle eptal $efect
• "atent $uctusArteriosus
Outflow tract Obstruction
•Pulmonal stenosis• Aorta stenosis
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oligaemic lungoligaemic lung plethoric lungplethoric lung
!H oF "A 5 %$ $'.% 5 " A$6%$ 5"
!H oF "A 5 %$ $'.% 5 " A$6%$ 5"
+!H A 5 " "A 5 .% hipoplasti7
+!H A 5 " "A 5 .% hipoplasti7
!H 2A * I% $'.% 5 %$ A"%$
!H 2A * I% $'.% 5 %$ A"%$
!H runcus Art 2A 5 %$
!H runcus Art 2A 5 %$
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Non Cyanotic
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+et to ig/t S/unt* sie of the defect
* compliance of .% is greater than ,% A, ! and PA enlargement
Pulmonary Hypertension* large A$ large left to right shunt
* develop in the third to fourth decades of life
* "ulmonary %ascular 'bstructive $isease
* bidirectional shunt right to left shunt sianosis
IS'"' S-'D&"
HEMODYNAMICHEMODYNAMIC
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AUSCULTATIONAUSCULTATION
9idely split and ;ed #9idely split and ;ed #< .% volume overload prolonged .% e=ection time
delays the closure of the pulmonary valve< large pulmonary venous return to .A ;ed split
ystolic e=ection murmurystolic e=ection murmur< not caused by the shunt< originates from the increased blood >ow passing
through the normal*sied pulmonary valve relative"
-id diastolic murmur-id diastolic murmur< increased blood >ow through the tricuspid valve
relative < large left to right shunt
Accentuated "#Accentuated "#< pulmonary hypertension
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• R%+ RV and !% dilatation
• prominent pulmonar& arter&segment• increased pulmonar&
vascular mar'ing ,plethora-
CHEST "#RAYCHEST "#RAY
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HEMODYNAMICHEMODYNAMIC
L!*t to Ri%ht Sh+t* sie of the defect
* level of pulmonar& vascular resistance
LA5 LV a$ PA !lar%!m!t
P+lmoary Hy&!rt!sio* large VS$ large left to right shunt
* high pulmonar& vascular resistance* !ulmonar& Vascular #bstructive $isease
* bidirectional shunt right to left shunt sianosis
EISENMENGER SYNDROME
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Small VS$Small VS$• normal !2 intensit&• holos&stolic murmur
produced b& left to right shunt
arge VS$arge VS$• accentuated !2 pulmonar& h&pertension• e/ection clic' ,occasionall& -• holos&stolic murmur left to right shunt
• mid diastolic murmur increased blood flow through the mitralvalve relative (S
arge VS$ with !ulmonar& Vascular #bstructive $iseasearge VS$ with !ulmonar& Vascular #bstructive $isease• loud and single S2• decreased loudness of the holos&stolic murmur ,or disappear-
AUSCULTATIONAUSCULTATIONSmall VSD
Large VSD
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CHEST "#RAYCHEST "#RAY
• %+ V and !% dilatation
• prominent pulmonar& arter&segment
• increased pulmonar&
vascular mar'ing ,plethora-
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HEMODYNAMICHEMODYNAMIC
L!*t to Ri%ht Sh+t
* sie of the ductus diameter+ length and turtuosit&* level of pulmonar& vascular resistance
LA5 LV5 asc!$i% Ao a$ PA !lar%!m!t
P+lmoary Hy&!rt!sio* large !$% large left to right shunt
* high pulmonar& vascular resistance
* !ulmonar& Vascular #bstructive $isease
* bidirectional shunt right to left shunt sianosis
EISENMENGER SYNDROME
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0ormal !2 0ormal !2 intensit&intensit&• small !$% normal !% pressure• accentuated if pulmonar& h&pertension is present
Continuous ,machiner&- murmur Continuous ,machiner&- murmur • left to right shunt occurs throughout the cardiac c&cle
significant pressure gradient between %o and !% during s&stole
and diastole %pical mid diastolic murmur %pical mid diastolic murmur
• increased blood flow through the mitral valve relative (S
arge !$% with 3isenmenger S&ndromearge !$% with 3isenmenger S&ndrome• single and loud S2 pulmonar& h&pertension• no longer continuous murmur e/ection s&stolic murmur
AUSCULTATIONAUSCULTATION
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CHEST "#RAYCHEST "#RAY
• %+ V+ ascending %o and !%
dilatation• prominent pulmonar& arter&
segment• increased pulmonar& vascular
mar'ing ,plethora-
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NONCYANOTIC CHDNONCYANOTIC CHD
OUT,LO6 TRACT O-STRUCTIONOUT,LO6 TRACT O-STRUCTION
VENTRICLE OUT,LO6 TRACT O-STRUCTIONVENTRICLE OUT,LO6 TRACT O-STRUCTION
6ITHOUT SHUNT6ITHOUT SHUNT
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Left ventricle outflow tractLeft ventricle outflow tract
obstructionobstruction
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• narrow split S2• ejection systolic click • harsh ejection systolic murmur
AUSCULTATIONAUSCULTATION
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• asymptomatic• symptomatic
depend ofseverity of lesion
myocardial function
dyspneuFeeding difficulty Failure to thriveHeart Failure
Syncope painchest
Sudden death
dyspneuFeeding difficulty Failure to thriveHeart Failure
Syncope painchest
Sudden death
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NEONATUSduct dependent systemic circulationClosed duktus arteriosus
deteriorate systemiccirculationhypoperfusion
BABY AND CHILD
• asymptomatic – mild lesion• symptomatic :headacheepitasisulsless
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Ri!t ventricle outflow tractRi!t ventricle outflow tract
obstructionobstruction
• !" pressure overload• R"H
valvar infun#ibular su$ravalvar
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NEONATUS % critical &Sduct dependent pulmonary circulation
closed duktus arteriosus severe cyanosis – acidosis
BABY an# CHILD• asymptomatic – mild lesion• symptomatic :
!ight Heart failureoedemahepatomegaly acites
C'anosis
#ila ada F$
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• S2 weak ejection systolic click
• harsh ejection systolic murmur
AUSCULTATIONAUSCULTATION
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LESI OBSTRU(TI) ALUR (ELUARLESI OBSTRU(TI) ALUR (ELUAR
"ENTRI(EL (IRI DAN (ANAN "ENTRI(EL (IRI DAN (ANANNeonatus % Duct De$en#ent %&'
sementara dipersiapkan intervensi
non(#edah ) #edah*
INTER"ENSI NON BEDAH
*ra#ien te+anan , -. % /. 00H
• +alloon ,ortic "alvyuloplasty -,S valv*
• +alloon ulmonal "alvuloplasty -S valv*
• +alloon ,ngioplasty -Co,*
./0&!"&/S. +&1,H
• "alvotomy -S ) ,S valvar*
• !eseksi otot -S ) ,S su#valvar*
• !ekonstruksi -S ) ,S Supravalvar*
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FKUI International
Cyanotic
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OligemicOligemic
cyanosis spell hypoxia squatting
Pulmonary StenosisPulmonary Stenosisor Atresiaor Atresia
++
PFO / ASD / VSDPFO / ASD / VSD( R L S!"#$ %( R L S!"#$ %
• $etralogi Fallot$etralogi Fallot
• PS + PFO / ASDPS + PFO / ASD• PA + VSDPA + VSD
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• ess than1 &ear , 2 4 5 month -
• minute * hour
Spell cyanotic
Em!r%rcyEm!r%rcy
S!rio+s com&licatioS!rio+s com&licatio
• CVDCVD• (EMATIAN(EMATIAN
&nee'chest position
Oxigen
Seasion !ia"e#am
or morfin aciosis correction $
%ic Nat
Propranolol
)$ Shunt/ surgery
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TOTAL CORRECTION
• 7 8 moth• %oo$ si'! o* PA
PALIATI, o&!ratio
-T SHUNT
• s&!ll hy&oxia
• 9 8 moth
• small PA si'!
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PlethoraPlethora
*eeing i**iculty Failure to $hrie reccurence R$ in*ection ,!Fpulmonary !ypertention
Increase Pulmonary blood flow
TGATGA
COMMON MI"INGCOMMON MI"ING
TGATGA
COMMON MI"INGCOMMON MI"ING
P+lmoary
:asc+lar r!sist!c!
P+lmoary
:asc+lar r!sist!c!
,ommon -ixing. $APVD "nientricular ,onnection $run&us Arteriosus
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Paral!l circ+latioParal!l circ+latio
atrial . PFO ASDatrial . PFO ASD
entricle . VSDentricle . VSD
0eart o* Arteries. PDA0eart o* Arteries. PDA
www&sc'nei!erc'il!ren'os#ital&org
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Inter(ension non surgery
For Congenital Heart Diseases
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FKUI International
'cclusion of Intracardiac and %ascular
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'cclusion of Intracardiac and %ascularhuntsCoil emboliation of "$A
)eft* to# Cat'eter crosses t'e PDA
from t'e aortic si!e an! !eli(ers a coil&
)eft* bottom +it'!rawal of cat'eter*
lea(ing coil in PDA
'cclusion of Intracardiac and %ascular
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'cclusion of Intracardiac and %ascularhuntsAmplater $uctal 'ccluders
Am#lat"er !uctal occlu!er
Illustration courtesy A,A -e!ical ,rou# Aorta angiogram wit' !e(ice
occlusion of PDA* lateral (iew
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Amplater $uct 'ccluder
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Amplater $uct 'ccluder
'cclusion of Intracardiac and %ascular
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'cc us o o aca d ac a d ascu ahuntsAmplater occlusion of atrial septal defect
,loc&1ise *rom a2oe.
$ranscatheter eliery o*
Amplat3er eice 1hich is
positione across theatrial septal e*ect
Le*t. Amplat3er eice in
place
'cclusion of Intracardiac and %ascular
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hunts$evices for occlusion of the "F' and A$
A2oe.
0ore !elex septal occluer
Illustration courtesy +& )& ,ore an! Associates
"pper le*t.
,arioS4AL occluer
Illustration courtesy N-. -e!ical
Lo1er le*t.
Amplat3er PFO occluer
Illustration courtesy A,A -e!ical ,rou#
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l l
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Amplater septal
occluder
'cclusion of Intracardiac and %ascular hunts
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'cclusion of Intracardiac and %ascular hunts%entricular eptal $efect 'cclusion
A2oe. 4chocariogram o* muscular VSD"pper right. Fluoro image o* ,arioS4AL
eice occlusion o* a VSD5 $ransesophageal
echo pro2e ($44% an pigtail catheter in place5
Lo1er right. Amplat3er muscular entricular
septal occluer Illustration courtesy A,A -e!ical ,rou#
' l i f I t di d % l h t
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'cclusion of Intracardiac and %ascular hunts%$ 'cclusion with CardioA, $evice
%alloon Pulmonary
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y
(al(ulo#lasty
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Angioplasty
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AngioplastyAortic Coarctation Angioplasty
Illustrations s'owing left0 uninflate! an! rig't0 inflate! angio#lasty
balloon #ositione! wit'in coarctation of t'e !escen!ing aorta
Intravascular tents
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Intravascular tentsCoarctation of the Aorta
)eft uninflate! angio#lasty balloon an! stent wit'in coarctation
-i!!le e2#ansion of balloon an! stent
3ig't !eflation of balloon lea(ing stent wi!e o#en
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