PENYAKIT JANTUNG

BAWAAN
PADA DEWASA
Abdullah Afif Siregar
Departemen Kardiologi dan Kedokteran
Vaskuler
Fakultas Kedokteran USU
Medan

Standard Kompetensi Dokter

(Konsil Kedokteran Indonesia)
Level of expected ability :
Level 1 = mengetahui dan menjelaskan
Level 2 = pernah melihat atau pernah
didemonstrasikan
Level 3 = pernah melakukan atau pernah
menerapkan dibawah
supervisi
Level 4 = mampu melakukan secara
mandiri

Congenital Heart Disease :

I.
Acyanotic :
1. Atrial Septal Defect (ASD)
2. Ventricle Septal Defect (VSD)
3. Patent Ductus Arteriosus (PDA)
4. Aortic Stenosis
5. Pulmonary Stenosis
6. Aortic Coarctation
II.
Cyanotic :
1. Tetralogy of Fallot (TOF)
2. Ebsteins Anomalies
3. Transposition of the great arteries (TGA)
4. Eisenmenger Syndrome

Atrial Septal Defect

ASD accounts for 1/3 of CHD cases in adult

Women two to three times than men.
Anatomically, it may take the form of

Additional cardiac abnormalities may occur with each

type of defect; these include

Ostium secundum, in the region of the fossa ovalis (75%)

Ostium primum, in the lower part of the atrial septum (15%) ;
and
Sinus venosus, in the upper atrial septum (10%)

mitral-valve prolapse (with ostium secundum defects),

mitral regurgitation (due to a cleft in the anterior mitral-valve
leaflet, which occurs with ostium primum defects),
partial anomalous drainage of the pulmonary veins into the
right atrium or venae cavae (with sinus venosus defects).

Most of atrial septal defects

Result from spontaneous genetic mutations, some are

inherited.

The physiologic consequences of ASD are the result of

the shunting of blood from one atrium to the other
The direction and magnitude of the shunt are
determined by

the size of the defect and

the relative compliance of the ventricles.

Small defect (< 0.5 cm in diameter) = a small shunt and

no hemodynamic sequelae.
Large defect (> 2 cm in diameter) = a large shunt, with
substantial hemodynamic consequences.
In most adults with atrial septal defects,
The RV is more compliant than the LV ; as a result :

left atrial blood is shunted to the right atrium , causing

increased pulmonary blood flow and dilatation of RA, RV,
and MPA

Pulmonary artery
Atrial septaldefect
Pulmonaryveins
Left"
atrium

Right"
atrium

Left"
ventricle
Right"
ventricle

PHYSICAL EXAMINATION

In a patient with a large atrial septal

defect, a right ventricular or pulmonary
arterial impulse may be palpable.
The first heart sound is normal, and there
is wide and fixed splitting of the second
heart sound.

A systolic ejection murmur, audible in the

second left intercostal space, peaks in
mid-systole, ends before the second
heart sound, and is usually so soft

Electrocardiography
A patient with atrial septal defect often has right-axis deviation
and incomplete right bundle-branch block, right ventricle
hypertrophy and right atrial enlargement.
A patient with ostium primum defects has left-axis deviation
A patient with sinus venosus defects has a junctional or low
atrial rhythm (inverted P waves in the inferior leads) occurs.
A patient with an atrial septal defect usually has normal sinus
rhythm for the first three decades of life, after which atrial
arrhythmias, including atrial fibrillation and supraventricular
tachycardia, may appear.

Chest X ray of VSD with various

pattern of the heart and lung
vascularity

http://myweb.lsbu.ac.uk/dirt/museum/simon/56-141-gsa30.jpg

Echocardiography :

Sub xiphoid view

A. Sinus venosus defect
B. ASD secundum
C. ASD primum

Kateterisasi ASD

Natural history :

A small defect with minimal left-to-right shunting

(characterized by Qp / Qs <1.5) usually causes no
symptoms or hemodynamic abnormalities and therefore
does not require closure.
A moderate or large ASD often have no symptoms until
the third or fourth decades of life despite substantial
left-to-right shunting (characterized by Qp / Qs 1.5 or
more).
Over the years, the increased volume of blood flowing
through the chambers of the right side of the heart
usually causes RV dilatation and failure.
Obstructive pulmonary vascular disease (Eisenmengers
syndrome) occurs rarely in adults with atrial septal
defect.

Natural history :

A symptomatic patient with an ASD typically

reports fatigue or dyspnea on exertion.
Anti heart failure agent (such as diuretics, digoxin)
could be given if heart failured developed.
Alternatively, the development of such sequelae
as supraventricular arrhythmias, right heart
failure, paradoxical embolism, or recurrent
pulmonary infections may prompt the patient to
seek medical attention.
Although a few patients with an unrepaired atrial
septal defect have survived into the eighth or
ninth decade of life, those with sizable shunts
often die of right ventricular failure or arrhythmia
in their 30s or 40s.

Natural
history :

An Atrial Septal Defect with a ratio of pulmonary to

systemic flow of 1.5 or more should be closed
surgically to prevent right ventricular dysfunction.
Surgical closure is not recommended for patients with
irreversible pulmonary vascular disease and
pulmonary hypertension.
Devices for percutaneous atrial septal closure are
under investigation, their safety and efficacy are
known.
Prophylaxis against infective endocarditis is not
recommended
Patients with atrial septal defects (repaired or
unrepaired) unless a concomitant valvular abnormality
(e.g., mitral-valve cleft or prolapse) is present.

Surgical
closure
ASD procedure

http://www.hsforum.com/stories/storyReader$4137

Devices for percutaneous atrial septal closure

procedure (Amplatzs Septal Occluder)

Devices for percutaneous atrial septal closure procedure

Devices for percutaneous atrial

septal closure procedure

Ventricular Septal Defect

Ventricular Septal Defect is the most common congenital

cardiac abnormality in infants and children.
It occurs with similar frequency in boys and girls.
25 to 40 percent of such defects close spontaneously by
the time the child is 2 years old; 90 percent of those that
eventually close do so by the time the child is 10.
Anatomically, located are

70 percent in the membranous portion of the

interventricular septum,
20 percent in the muscular portion of the septum,
5 percent just below the aortic valve (thereby undermining
the valve annulus and causing regurgitation), and
5 percent near the junction of the mitral and tricuspid
valves (so-called atrioventricular canal defects).

Pulmonary artery

Pulmonaryveins
Left"
atrium

Right"
atrium
Left"
ventricle

Right"
ventricle

Ventricle
Septal
Defect

A view of the ventricular septal defect (VSD)

from the left side. The VSD lies immediately
beneath the aortic valve.

Hemodynamic VSD
The

physiologic consequences of a ventricular septal

defect are determined by the size of the defect and the
relative resistance in the systemic and pulmonary
vascular beds.
If the defect is small, there is little or no functional
disturbance, since pulmonary blood flow is increased only
minimally.
In contrast, if the defect is large, the ventricular systolic
pressures are equal and the magnitude of flow to the
pulmonary and systemic circulations is determined by the
resistances in the two beds. Initially, systemic vascular
resistance exceeds pulmonary vascular resistance, so
that left-to-right shunting predominates

Hemodynamic VSD

Over time, the pulmonary vascular resistance

usually increases, and the magnitude of left-toright shunting declines.
Eventually, the pulmonary vascular resistance
equals or exceeds the systemic resistance; the
shunting of blood from left to right then ceases,
and right-to left shunting begins.
With substantial left-to-right shunting and little or
no pulmonary hypertension, the left ventricular
impulse is dynamic and laterally displaced, and
the right ventricular impulse is weak.
If pulmonary hypertension develops, a right
ventricular heave and a pulsation over the
pulmonary trunk may be palpated.

holosystolic murmur

short mid-diastolic apical rumble

systolic ejection murmurs

Auscultation
:

The murmur of a moderate or large defect is

holosystolic, loudest at the lower left sternal border,
and usually accompanied by a palpable thrill.

A short mid-diastolic apical rumble (caused by

increased flow through the mitral valve) may be
heard.

Small, muscular ventricular septal defects may

produce high frequency systolic ejection murmurs
that terminate before the end of systole (when the
defect is occluded by contracting heart muscle).
The holosystolic murmur and thrill diminish and
eventually disappear as flow through the defect
decreases, and a murmur of pulmonary
regurgitation (Graham Steells murmur) may
appear. Finally, cyanosis and clubbing are present.

A. PA chest radiograph demonstrates cardiomegaly,

B. the pulmonary outflow tract is convex and
C. the pulmonary arterial markings are increased

Electrocardiography (ECG) and chest

radiography (CXR)

Electrocardiography (ECG) and chest radiography (CXR)

provide insight into the magnitude of the hemodynamic
impairment.
Small VSD, the ECG and CXR are normal.
With a large defect, there is electrocardiographic
evidence of left atrial and ventricular enlargement ,
The left ventricular enlargement and shunt
vascularityare evident on the radiograph.
If pulmonary hypertension occurs, the QRS axis
shifts to the right,and right atrial and ventricular
enlargement are noted on the electrocardiogram.
The chest film of a patient with pulmonary
hypertension shows marked enlargement of the
proximal pulmonary arteries, rapid tapering of the
peripheral pulmonary arteries, and oligemic lung
fields.

VSD + Eisenmenger

VSD +
Eisenmenger

Echocardiography :

Two-dimensional
echocardiographywith Doppler flow
can confirm the presence and
location of the ventricular septal
defect,
Color-flow mapping provides
information about the magnitude and
direction of shunting.

Echocardiography examination

Kateterisasi VSD

With catheterization and angiography, one can

confirm the presence and location of the
defect, as well as determine the magnitude of
shunting and the pulmonary vascular
resistance.

Angiography VSD

Aortic valve

e
erv
Int
tum
sep
icle
ntr

Natural history and

treatment

The natural history of ventricular septal defect depends

on the size of the defect and the pulmonary vascular
resistance. Adults with small defects and normal
pulmonary arterial pressure are generally asymptomatic,
and pulmonary vascular disease is unlikely to develop.
Persistent defects, however, may predispose patients to
endocarditis, arrhythmias, heart failure, aortic
regurgitation, and pulmonary hypertension.
Therefore, periodic clinical and laboratory evaluations
with electrocardiography, chest radiography, and
echocardiography are recommended, depending on the
presence of these complications or associated lesions.

Natural history and

treatment :

Congestive heart failure due to chronic volume

overload of the ventricles occurs in patients with
isolated medium or large VSDs, is rarely seen in adults
because most patients present and undergo repair
before adulthood.
If congestive heart failure appeared, diuretics, digoxin
and inotropic agent could be given
Surgical closure of the defect is recommended.
VSD is uncommon in women of reproductive age.
Patients who have small defects with a shunt ratio less
than 2.0, normal pulmonary pressure, and preserved
functional aerobic capacity can undergo pregnancy
with little or no risk.
However, pregnancy is contraindicated in patients with
the Eisenmenger complex because of the significant
risk for maternal death, up to 50%

Surgical
closure
VSD procedure

Natural history and

treatment :

Clamshell-type catheter occlusion devices

are being tested as a means of closing apical
muscular VSDs. Successful transcatheter
device closure of trabecular (muscular) and
perimembranous VSDs has been reported

Aortic valve

le
tric
en
erv
Int

Aortic valve

sep
t um

Patent Ductus Arteriosus

The ductus arteriosus connects the descending aorta

(just distal to the left subclavian artery) to the left
pulmonary artery.
In the fetus, it permits pulmonary arterial blood to
bypass the unexpanded lungs and enter the
descending aorta for oxygenation in the placenta.
It normally closes soon after birth, but in some infants
it does not close spontaneously. PDA accounts for
about 10 percent of cases of congenital heart disease.
Its incidence is higher than average in pregnancies
complicated by persistent perinatal hypoxemia or
maternal rubella infection and among infants born at
high altitude or prematurely.

Causes
The cause of patent ductus arteriosus
(PDA) is not known.
Genetics may play a role. A defect in one or
more genes could prevent the ductus
arteriosus from closing normally after birth.
PDA is more common in:

Premature infants (babies born too early)

Infants with genetic abnormalities such as Down
syndrome
Infants whose mother had German measles
(rubella) during pregnancy

Normal Circulation in a Fetus

Physical
Examination :

A patient with PDA and a moderate or large shunt

has bounding peripheral arterial pulses , a widened
pulse pressure, and a hyperdynamic left
ventricular impulse.
The first heart sound is normal.A continuous
machinery murmur, audible in the second left
anterior intercostal space.
With a large shunt, mid-diastolic and systolic
murmurs (from increased flow through the mitral
and aortic valves, respectively) may be noted.
If pulmonary vascular obstruction and
hypertension develop,the continuous murmur
decreases in duration and intensity and eventually
disappears and a pulmonary ejection click and a
diastolic decrescendo murmur of pulmonary
regurgitation may appear.

CHEST X RAY
Small PDA, the ECG and CXR film are
normal.
Large PDA and substantial left-to right
shunting, left atrial and ventricular
hypertrophy are evident,
The chest film shows pulmonary
plethora, proximal pulmonary arterial
dilatation, and a prominent ascending
aorta. The ductus arteriosus may be
visualized as an opacity at the
confluence of the descending aorta
and the aortic knob.
If pulmonary hypertension develops,
right ventricular hypertrophy is noted.

Echocardiograph

y With
:
two-dimen

sional echocardi
ography,the
ductus arteriosus
can usu ally be
visualized
Doppler studies
demonstrate
continuous flow
in the pulmonary
trunk.

In patent ductus arteriosus,

pulmonary blood flow, LA and
LV volumes, and ascending AO
volume are increased.

AO = aorta; LA = left atrium;

LV = left ventricle; PA = pulmonary artery.

Kateterisasi PDA

TREATMENT
:

Pharmacological :

Medical management such as diuretics and fluid restriction

Adequate calories and minerals with fluid restriction
Indomethacin (0.1- 0.2 mg/kg IV) is a potent stimulator of
ductal closure. It blocks the enzyme cyclooxygenase
inhibiting prostaglandin synthesis thereby facilitating
ductal
closure (for infants)
Ibuprofen, another non-selective cyclooxygenase inhibitor,
given on the third day of postnatal life appears to be as
effective as indomethacin for PDA closure but less likely to
induce oliguria (for infants)

Non Pharmacological :

Surgical ligation
Trans-catheter closure

Surgical ligation :
The classical approach via a
left lateral sternotomy with
ligation can be performed in
an operating room or at the
bedside with low mortality.

Trans-catheter closure :
Coil
ADO = Amplatz Ductal
Occluder

Closure of a PDA by coil catheterization. (A) Injection into the aorta reveals a
large PDA at baseline. (B) Following placement of a coil the angiographic dye
no longer crosses into the pulmonary artery confirming ductal closure.
(MPA = main pulmonary artery, PDA = patent ductus arteriosus, DA = descen
ding aorta)

TERIMA KASIH