acyanotic

Ria Nova
Structures of the heart
Normal Heart
 Atrial Septal defect (ASD )

• Insidence : + 10 %
  :  ratio = 1,5 to 2 : 1
• Anatomy :
 Defect on foramen ovale : Secundum ASD (B)
 Defect at SVC (superior vena cava) and RA
junction: sinus venosus ASD (A)
 Defect at ostium primum : primum ASD (C) (the
most common)
 Atrial Septal Defect

Diagram of ASD
 Shunt physiology
• Shunting through an ASD is determined by the
relative compliances of the two ventricles and not
by 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 in
pressure 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 than

the left, resulting in less resistence to filling in the
right atrium
 Shunt physiology
• Most shunting is left to right, because left atrial
pressure exceeds right atrial pressure through most
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
 Atrial septal Defect

RA LA

RA LA

RV LV
RV LV
 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
 Atrial Septal Defect

Auscultation :1st HS N or loud

widely split and fixed 2nd HS
Ejection Sistolic Murmur
 Electrocardiography

• Right axis deviation of + 90 to + 180

degrees and mild right ventricular
hypertrophy (RVH) or right bundle
branch block (RBBB) with an rsR’
pattern in V1 are typical findings
 X-ray studies

• Cardiomegaly with enlargement of

the right atrium and right ventricle
with a cardiothoracic ratio greater
than 50%
• A prominent pulmonary artery
segment and increased pulmonary
vascular markings
 Natural History
• Spontaneous closure of the secundum defect
occurred in about 40% of pt in the first 4 years of
life
• The defects may be decrease in size in some pt
(patient)
• Defek tetap seperti itu, tidak membesar, tidak
mengecil
• 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
 Natural History
• Most children with an ASD remain active and
asymptomatic. Rarely, congestive heart failure
(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
 Atrial Septal Defect

Diagnosis Differential

 Primary Atrial Septal Defect

ECG : LAD
 Partial Anomalous Pulmonary Vein
Drainage (vena pulmonalis tidak
semuanya masuk ke atrium kiri)
 Pulmonary Stenosis
 Innocent Murmur (ada murmur walaupun
tidak ada kelainan jantung)
 Management

• Nonsurgical closure/ catheter

closure
ASO: atrial septal occluder
melalui arteri femoralis

• Surgical closure : preschool age

 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 pars
membranous septum
 Muscular defect
 Physiologic effects

• The primary anatomic variable that

determines the physiologic state of the
patient is defect size

• In small or medium sized VSD, the size of the

defect limits the left to right shunt

• In large VSD, approximately the size of the

aortic orifice, the relative resistances
(mempengaruhi) of the systemic and
pulmonary circulations regulate flow across
the defect
 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
 Clinical manifestations
• Infants with small VSD: well development and
acyanotic

• 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 may
be holosystolic or early systolic. An apical
diastolic rumble is present with a moderate to
large shunt
 Ventricular Septal Defect

Murmur: pansystolic
grade 3/6 or higher at
LSB 3 Small VSD

Large VSD
 Ventricular septal defect

RA
LA
RA LA

RV LV RV LV
 Ventricular septal Defect

Diagnosis Differential

 PDA with PH
 Tetralogy Fallot non cyanotic
 Inoscent murmur
 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 some
infants with large defect
• Infective endocarditis rarely occurs
 Ventricular septal defect

Management:
Medical : treatment of CHF
Definitive : VSD closure
 Surgery

 Transcatheter closure
 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
 Shunt Physiology
• With PDA, as with all left to right shunts, the
major interrelated factors that control the
magnitude 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
 Patent Ductus Arteriosus

RA LA
RA LA

RV LV
RV LV
 Clinical manifestations

• History

– Patients are usually asymptomatic when the

ductus is small

– A large shunt PDA may cause a lower respiratory

tract infection, atelectasis, and CHF
 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
 Clinical manifestations
• The P2 is usually normal, but its intensity
may be accentuated if pulmonary
hypertension 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
 Patent Ductus Arteriosus

Auscultation : continuosus murmur

at upper LSB 2
 Natural history
• Unlike PDA in premature infants,
spontaneous closure of a PDA does not
usually 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 decreased
responsiveness of the premature ductus to
oxygen

• CHF and/or recurrent pneumonia develop if

the shunt is large
 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
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
 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

 Pulmonary Stenosis
• Incidence : 8-10%

• Anatomy:
Pulmonary stenosis valvular :
 Bicuspid pulmonary valve
 Valve leaflet thickening and adhession
Pulmonary stenosis infundibular :
 Hyperthropy infundibulum
 Pulmonary Stenosis

• Clinical findings
Valvular stenosis
Mild : Ejection systolic
Wide 2nd HS
ejectiin click
Moderate: ejection systolic, early systolic click
Severe : ejecstion systolic, ejection click (-)
Stenosis infundibular
Ejection click ( - )
1st HS normal, 2nd HS weak, ejection systolic
Pulmonary stenosis periphery
1st & 2nd HS normal, ejection systolic
 Pulmonary Stenosis

Mild : ejection systolic

2nd HS wide split
ejection click
Moderate: ejecsi systolic , early ejection click
Severe : ejection systolic, click ejection (-)
 Poulmonary Stenosis

• Diagnosis !!!
Asymptomatic patient:
click systolic (stenosis valvular)
systolic murmur
wide split 2nd HS vary with respiration
 Pulmonary Stenosis

ECG : RAD
Echocardiograhhy : confirmation diagnosis
Catheterization: increased RV pressure
without increased oxygen saturation
 Pulmonary Stenosis

• Management

Medicamentosa : useless
Mild stenosis: intervention (-)
Moderate stenosis: observation
Severe stenosis: balloon valvuloplasty
 Coarctation Aorta
Incidence
• 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 and
pulmonary artery junction
 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
 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.
 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 ribs
may be seen in older children but rarely in
children younger than 5 years of age

• Symptomatic
– Marked cardiomegaly and pulmonary edema or
pulmonary venous congestion are usually present
 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
 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
 Coarctation Aorta

• Management
Symptomatic
Neonates :
PGE1 to maintain PDA
Diuretic
Correction acid-base imbalance
Prepared to undergo surgery

Big children:
Surgery should be done as soon as diagnosis
made
Balloon angioplasty