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Ria Nova
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Structures oftheheart
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Normal Heart
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KLASIFIKASI PJB
BERDASARKAN KEGAWATAN PADA BBL:
> PJB RISIKO RENDAH
> PJB RISIKO TINGGI
BERDASARKAN DUCT DEPENDENT:
>PJB dgn sirkulasi pulmonal: Atr pulm, atr trikuspid,TOF
> PJB dgn sirkulasi sistemik: HLHS,Coarct aorta
> PJB dgn percampuran darah: TGA
BERDASARKAN TANDA/GEJALA SIANOSIS:
>PJB NON SIANOTIK
> PJB SIANOTIK
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Atrial Septal defect (ASD )
Insidence : + 10 %
: ratio = 1,5 to 2 : 1
Anatomy :
Defect on foramen ovale : Secundum ASD
Defect at SVC and RA junction: sinus
venosus ASD Defect at ostium primum : primum ASD
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ASD
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AtrialSeptal Defect
Diagram of ASD
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Shunt physiology
Shunting through an ASD is determined by the
relative compliances of the two ventricles and notby the size of the defect, unless the defect is very
small
The direction in which blood flows through the
defect is primarily related to the differences inpressure in the right and left atria during the
cardiac cycle, and atrial pressure are determined
by the relative compliances of the ventricles.
The right ventricle generally is more compliant thanthe left, resulting in less resistence to filling in the
right atrium
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Shunt physiology
Most shunting is left to right, because left atrial
pressure exceeds right atrial pressure throughmost of the cardiac cycle
Early in infancy left to right shunting is minimal
because the right ventricle is thick and stiff and
relatively noncompliant
In the first few weeks of life the pulmonary
vascular resistence decreases, the right ventricle
becomes more compliant, and the amount of left to
right shunting increases
Pulmonary blood flow increases, often three to four
times normal; however pulmonary artery pressure
increases only slighty and pulmonary resistence
remains in the normal range
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RA
RV
LA
LV
RA
RV
LA
LV
Atrialseptal Defect
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Clinical manifestations
History
Infants and children are usually asymptomatic
Physical examination
A relatively slender body build
A widely split and fixed S2, a grade 2-3/6 systolic
ejection murmur. With a large left to right shunt,
a middiastolic rumble resulting from relative
tricuspid stenosis may be audible at the lower
left sternal border
The typical auscultatory findings may be absent
in infants, even infants who have a large defect
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AtrialSeptal Defect
Auscultation :1st HS N or loud
widely split and fixed 2nd HS
Ejection Sistolic Murmur
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Electrocardiography
Right axis deviation of + 90 to + 180
degrees and mild right ventricular
hypertrophy (RVH) or right bundlebranch block (RBBB) with an rsR
pattern in V1 are typical findings
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X-ray studies
Cardiomegaly with enlargement of
the right atrium and right ventricle
with a cardiothoracic ratio greaterthan 50%
A prominent pulmonary artery
segment and increased pulmonary
vascular markings
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Right atrial enlargement
Prominence the MPAsegment
Increased pulmonary
vascular marking
Atrial Septal DefectChest X-Ray
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Natural History
Spontaneous closure of the secundumdefect occurred in about 40% of pt in the
first 4 years of life
The defects may be decrease in size in
some pt In pt with an ASD < 3 mm in size diagnosed
before 3 mo of age, spontaneous closure
occurs in 100% of pt at 11/2 yr of age.
A large ASD ( with a diameter > 8 mm)
rarely close spontaneously
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Natural History
Most children with an ASD remain active and
asymptomatic. Rarely, congestive heartfailure (CHF) can develop in infancy
If a large defect is untreated, CHF and
pulmonary hypertension develop in adults
who are in their 20s and 30s With or without surgery, atrial arrhytmias
(flutter or fibrillation) may occur in adults
Infective endocarditis does not occur in pt
with isolated ASDs. Therefore, subacute
bacterial endocarditis (SBE) prophylaxis is
unnecessary
Cerebrovascular accident, resulting from
paradoxical embolization through a ASD, is a
rare complication
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AtrialSeptal Defect
Diagnosis Differential
Primary Atrial Septal Defect
ECG : LAD
Partial Anomalous Pulmonary Vein
Drainage
Pulmonary Stenosis Innocent Murmur
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Management
Nonsurgical closure/ catheter
closure
ASO: atrial septal occluder
Surgical closure : preschool age
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Atrialseptal defect
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Ventricular septal defect Insidence
20 % of all CHD
No sex influenced Anatomy
Subarterial defect: below pulmonary and
aortic valve
Perimembranous defect: below aortic valve at parsmembranous septum
Muscular defect
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VSD
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Physiologic effects
The primary anatomic variable thatdetermines the physiologic state of the
patient is defect size
In small or medium sized VSD, the size ofthe defect limits the left to right shunt
In large VSD, approximately the size of the
aortic orifice, the relative resistances of thesystemic and pulmonary circulations
regulate flow across the defect
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Clinical manifestation
History
With a small VSD, the patient is asymptomatic
with normal growth and development
With a moderate to large VSD, delayed growth
and development, decreased exercise tolerance,
repeated pulmonary infections, and CHF are
relatively common during infancy
With long standing pulmonary hypertension, a
history of cyanosis and a decreased level of
activity may be present
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Clinical manifestations
Infants with small VSD: well development andacyanotic
A systolic thrill may be present at lower left
sternal border. Precordial bulge and
hyperactivity are present with a large shunt
VSD
A grade 2-5/6 regurgitant systolic murmur is
audible at the lower left sternal border. It maybe holosystolic or early systolic. An apical
diastolic rumble is present with a moderate to
large shunt
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Small VSD
Large VSD
VentricularSeptal Defect
Murmur: pansystolic
grade 3/6 or higher atLSB 3
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RA
RV
RA LALA
RV LVLV
Ventricularseptal defect
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VentricularSeptal Defect
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VentricularSeptal Defect
CardiomegalyApex down ward
Prominence pulmonaryartery segmentIncreased pulmonary vascularmarking
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Ventricularseptal Defect
Diagnosis Differential
PDA with PH
Tetralogy Fallot non cyanotic
Inoscent murmur
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Natural History
Spontaneous closure occurs in 30% to 4o%
of patients with membranous VSDs and
muscular VSDs during the first 6 months of
life.
CHF develops in infants with large VSDs
Pulmonary vascular obstructive disease may
begin to develop as early as 6 to 12 months
of age in pt with large VSDs
Infundibular stenosis may develop in someinfants with large defect
Infective endocarditis rarely occurs
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Ventricularseptal defect
Management:
Medical : treatment of CHF
Definitive : VSD closure
Surgery
Transcatheter closure
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Patent Ductus Arteriosus
Insidence
+ 10%
Female : Male = 1.2 to 1.5 : 1
Premature and LBW higher
Anatomy
Fetus: ductus arteriosus connects PA and aorta.
If ductus does not closs Patent Ductus arteriosus
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PDA
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Shunt Physiology
With PDA, as with all left to right shunts, the
major interrelated factors that control themagnitude of shunting are as follows:
Diameter and length of the ductus arteriosus
Pressure difference between the aorta and the
pulmonary artery
Systemic and pulmonary vascular resistances
Because systemic vascular resistance does not
change sifnificantly after birth, changes in
pulmonary vascular resistance are the major
determinant in regulating left to right shunting
through a PDA This relationship is particularly important in the
first 2 months after birth when pulmonary vascular
resistance normally is decreasing
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RA
RV
LA
LV
RA LA
RV LV
PatentDuctusArteriosus
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Clinical manifestations
History
Patients are usually asymptomatic when theductus is small
A large shunt PDA may cause a lower respiratory
tract infection, atelectasis, and CHF
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Clinical manifestations
Physical examination
Tachycardia and exertional dyspnea may be
present in children with a large shunt PDA
With pulmonary vascular obstructive disease, a
right to left ductal shunt results in cyanosis only
in the lower half of the body (i,e,.differential
cyanosis)
The precordium is hyperactive. A systolic thrill
may be present at the upper left sternal border.
Bounding peripheral pulses (pulse celler)with
wide pressure (elevated systolic pressure and
lower diastolic pressure) are characteristic
findings
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Clinical manifestations
The P2 is usually normal, but its intensity
may be accentuated if pulmonaryhypertension is present. A grade 1-4/6
continuous (machinery) murmur is best
audible at the left infraclavicular area or
upper left sternal border
The heart murmur may be crescendo
systolic at the upper left sternal border in
small infants or infants with pulmonary
hypertension.
An apical diastolic rumble may be heard
when the PDA shunt is large
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PatentDuctusArteriosus
Auscultation : continuosus murmur
at upper LSB 2
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Natural history
Unlike PDA in premature infants,
spontaneous closure of a PDA does notusually occur in full term infants. This is
because the PDA in term infants results
from a structural abnormality of the ductal
smooth muscle rather than a decreasedresponsiveness of the premature ductus to
oxygen
CHF and/or recurrent pneumonia develop if
the shunt is large
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Natural history
Pulmonary vascular obstructive disease
may develop if a large PDA with pulmonary
hypertension remains untreated
SBE which may be more frequent with
small PDAs than with large ones, may
occur
Although rare, an aneurysm of PDA may
develop and possibly rupture
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Diagnosis Differential
- Coronary arteriovenous fistula- Systemic arterivenous fistula
- Pulmonary arteriovenous fistula
- Venous hum
- Collaterals in CoA and TOF
- VSD with AR
- Absence of the pulmonary valve
- Persistent truncus arteriosus
- TAPVD
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Management
Medical
Indomethacin : ineffective in term infants with
PDA and should not be used
Prophylaxis for SBE is indicated when
indications arise
Nonsurgical closure: transcatheter closure
Surgical closure: ligation of PDA
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PatentDuctusArteriosus
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PatentDuctusArteriosus
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PatentDuctusArteriosus
PDA before occludedusingADO
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PatentDuctusArteriosus
PDA after occludedusingADO
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PatentDuctusArteriosus
PDA before occludedusingcoil
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PatentDuctusArteriosus
PDA after occludedusingcoil
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P
ulmonaryS
tenosis
Incidence : 8-10%
Anatomy:Pulmonary stenosis valvular :
h Bicuspid pulmonary valve
h Valve leaflet thickening and adhession
Pulmonary stenosis infundibular :h Hyperthropy infundibulum
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PulmonaryStenosis
Clinical findingsValvular stenosis
Mild : Ejection systolic
Wide 2nd HS
ejectiin clickModerate: ejection systolic, early systolic click
Severe : ejecstion systolic, ejection click (-)
Stenosis infundibularEjection click ( - )
1st HS normal, 2nd HS weak, ejection systolic
Pulmonary stenosis periphery
1st & 2nd HS normal, ejection systolic
P l St i
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PulmonaryStenosis
Mild : ejection systolic
2nd
HS wide splitejection click
Moderate: ejecsi systolic , early ejection click
Severe : ejection systolic, click ejection (-)
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PoulmonaryStenosis
Diagnosis
Asymptomatic patient:click systolic (stenosis valvular)
systolic murmur
wide split 2nd HS vary with respiration
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PoulmonaryStenosis
Normal or mild cardiomegalyMarked pulmonary valvepost stenotic dilatationNormal pulmonaryvascularity
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ECG : RAD
Echocardiograhhy : confirmation diagnosis
Catheterization: increased RV pressurewithout increased oxygen saturation
PulmonaryStenosis
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PulmonaryStenosis
Management
Medicamentosa : uselessMild stenosis: intervention (-)
Moderate stenosis: observation
Severe stenosis: balloon valvuloplasty
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PulmonaryStenosis
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PulmonaryStenosis
Beforeballooning
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PulmonaryStenosis
During ballooning
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PulmonaryStenosis
After ballooning
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Coarctation AortaIncidence
In Western country 5 % of all CHD
In Asian Country incidence lower
underdiagnosis ?
Anatomy
Stenosis at any where in the aorta
(from aortic valve to abdominalis aorta)
More frequent at ductus arteriosus Botalli andpulmonary artery junction
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CoarctationAorta
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Clinical manifestations
History
Most children are asymptomatic
Occasionally a child complains of weekness
and/or pain in legs after exercise
Symptomatic infants: poor feeding, dyspnea, and
poor weight gain or signs of acute circulatory
shock may develop in the first 6 weeks of life
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Physical examination
Asymptomatic Pt grow and develop normally
Arterial pulse in the leg are either absent or
weak and delayed. There is hypertension in the
arm,or blood pressure readings in the arm are
higher than those in the leg A systolic thrill may be present in the
suprasternal notch. An ejection systolic murmur
grade 2-4/6 can be heard at the upper right
sternal border and mid or lower left sternal
border.
A well localized systolic murmur is also audible
in the left interscapular area in the back.
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X ray studies
Asymptomatic
The heart size may be normal or slightly
enlarged
Dilatation of the ascending aorta may be seen
Rib notching between the fourth and eight ribsmay be seen in older children but rarely in
children younger than 5 years of age
Symptomatic
Marked cardiomegaly and pulmonary edema orpulmonary venous congestion are usually
present
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Natural History
Symptomatic infants
About 20-30% of all patients with CoA develop
CHF by 3 months of age
If it is undetected or untreated, early death may
result from CHF and renal shutdown in
symptomatic with Coa
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Natural history
Asymptomatic CoA
A bicuspid aortic valve may cause stenosis
and/or regurgitation with age
SBE can occur on either the aortic valve or the
coarctation
LV failure, rupture of the aorta, intracranial
hemorrhage, hypertensive encephalopathy
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Management
Symptomatic
Neonates :
PGE1 to maintain PDA
Diuretic
Correction acid-base imbalancePrepared to undergo surgery
Big children:
Surgery should be done as soon as diagnosismade
Balloon angioplasty
CoarctationAorta
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CoarctationAorta
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CoarctationAorta
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CoarctationAorta