acyanotic CHD 2008

8/8/2019 acyanotic CHD 2008

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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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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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CardiomegalyApex down ward

Prominence pulmonaryartery segmentIncreased pulmonary vascularmarking


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Ventricularseptal Defect


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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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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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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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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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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- 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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PDA before occludedusingADO


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PDA after occludedusingADO


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PDA before occludedusingcoil


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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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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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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

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acyanotic CHD 2008