Case Report

Atrial Septal Defect and Ventricular Septal Defect

Supervisor
dr. Muhammad Ali, Sp.A(K)
Presented by :
Qarina Hasyala Putri
080100367
Dian Primadia Putri
100100013

Departement of Pediatrics
Haji Adam Malik General Hospital
Faculty of Medicine Sumatera Utara University
Medan 2014
Case Report

ATRIAL SEPTAL DEFECT AND VENTRICULAR SEPTAL DEFECT

Presenter

: Qarina Hasyala Putri (080100367)

Dian Primadia Putri (100100013)

Day/Date

: Tuesday/ April22nd2014

Supervisor

: dr. Muhammad Ali,Sp.A(K)

Introduction
Cardiac defects are the most common type of birth defect. An infant with a cardiac
defect is more likely to die from this type defect than other types of defects (Forrester 2002).
An atrial septal defect (ASD) is a defect or hole in the septum between the two atria of the
heart. ASDs are typically divided into several categories depending on the part of the atrial
septum where the defect occurs secundum ASD (also called ASD 2 or ASD II), defect in the
midroportion of the atrial septum concerning the fossa ovails. Primum ASD (also called ASD
1 or ASD I) defect is in the endocardial cushion section of the atrial septum and is typically
grouped with other endocardial cushion defects such as endocardial cushion ventricular septal
defect and atrioventricular canal. Sinus venous ASD defect is in the upper portion of the atrial
septum near the entry of the superior vena cava.1
Ventricular septal defect is one of the commonest congenital malformations of the
heart.2 Ventricular septal defect (VSD) is a condition whereby there is a hole between the two
pumping chambers of the heart. The defect can be small or large. The VSD may be termed
muscular, perimembranous, inlet, outlet, apical or doubly committed depending on its
position and the surrounding substance of the heart. Where the VSD is small, there is no
elevation of the low pressures found in the right ventricle (pumping chamber to the lungs)
and therefore the lungs are also low pressure (as they should be). Where the hole is large, the
pressure in this right ventricle can be elevated; sometimes equal to that of the high pressure
left ventricle (pumping chamber to the body).3
Epidemiology
Research indicates that congenital heart disease is diagnosed in 0.8% of children in
the first year of life. Atrial septal defect is the second most common congenital heart defect in
children and adults and occurs in anywhere from 0.67-2.1 per 1000 live births. Secundum
atrial septal defects comprise just over 90% of all atrial septal defects, whereas sinus venosus
and primum atrial septal defects comprise between 3-4% each. About 15-30% of healthy
adults have an unfused foramen ovale in which the valve functions normally but has failed to
fuse. In these individuals, a cardiac catheter passed into the right atrium can pass into the left
atrium through the foramen ovale (ie, probe-patent foramen ovale). In developed countries,
mortality rate of atrial septal defect is low (< 1%). Morbidity secondary to atrial septal defect

is unusual and typically limited to 3 groups of patients.Approximately 1% of infants with

moderate or large (ie, nonrestrictive) atrial septal defects, but no other left to right shunting
lesion (eg, patent ductus arteriosus, ventricular septal defect), have tachypnea and failure to
thrive. In these individuals, the pulmonary artery pressure, when measured during
catheterization or Doppler echocardiography, is at or near systemic level. In most instances,
this is a flow-related phenomena (high flow/low resistance), but in infants predisposed to
abnormal pulmonary vasculature, there may be a combination of both elevated flow and
resistance. Attempts to exclude mitral or left ventricular diastolic abnormalities as a cause of
these hemodynamics must be undertaken, as well as a thorough assessment of pulmonary
anatomy and mechanics, as both left-sided cardiac disease and primary pulmonary disease
can mimic symptoms of pretricuspid shunting. The female-to-male ratio is approximately 2:1.
Atrial septal defect , a congenital abnormality, is present at birth. However, in most cases, a
murmur is not audible until the child is a few months old. Symptoms usually do not occur in
individuals with atrial septal defect until late childhood, adolescence, or adulthood.Secundum
type (ie, ostium secundum), sinus venosus, and unroofed coronary sinus defects are
sometimes not diagnosed until the third decade of life.Ostium primum atrial septal defects are
usually diagnosed in the first few years of life because of mitral regurgitation murmur or an
abnormal ECG.A common atrium (ie, a combination of sinus venosus, ostium secundum, and
ostium primum defects) is usually diagnosed in the first few years of life because systemic
venous blood and pulmonary venous blood often partially mix before entering each ventricle;
this condition manifests as cyanosis. In addition, a common atrium may be associated with
complex CHD, and patients may present relatively early because of other intracardiac
abnormalities.4
VSDs affect 2-7% of live births. The patients area of residence may influence the
prevalence of known VSDs. For example, small muscular VSDs are most likely to be
identified in urban locations, possibly because of ready access to sophisticated healthcare in
these locations.An echocardiographic study revealed a high incidence of 5-50 VSDs per 1000
newborns. The defects in this study were small restrictive muscular VSDs, which typically
spontaneously close in the first year of life.VSDs are the most common lesion in many
chromosomal syndromes, including trisomy 13, trisomy 18, trisomy 21, and relatively rare
syndromes. However, in more than 95% of patients with VSDs, the defects are not associated
with a chromosomal abnormality.VSDs are slightly more common in female patients than in
male patients (56% vs 44%).4

Pathophysiology
1. Atrial Septal Defect
Atrial Septal Defect (ASD) is a congenital heart disease that allows blood to flow
from the left atrium into the right atrium, and occasionally from the right atrium to the left
atrium. This defect creates what is called shunting, or mixing of the oxygenated blood, from
the left side of the heart with the deoxygenated blood of the right side. Such defects in the
atrial septum account for 12% of all congenital heart diseases and are more common in
women. Aside from incomplete closure of the foramen ovale, septal defect occurs during the
development of the fetal heart in the first two weeks after conception. Although there are
some suggestions that some forms may be genetic, the cause of most ASDs is unknown.6

Main Types of the Defect

The classifications of ASD refer to the different locations at which the defect in the
septum occurs, they include: Patent foramen ovale (Ostium Secundum), Ostium primum and
Sinus Venosus. The diagram below shows the location of such defects.

The most common ASD (70-90%) is Ostitium Secundum, also known as a

patent foramen ovale. It is important to note that incomplete closure of the foramen ovale,
which allows blood to bypass pulmonary circulation during fetal circulation, occurs in
approximately 35% of adults. However, this is not considered a true atrial septal defect until
it reaches a clinically appreciable size or promotes significant shunting. This defect occurs in
the center of the septum and is usually asymptomatic. Although in some cases, if left
uncorrected, ostitium secundum ASD may result in pulmonary hypertension, right-side heart
failure, or stroke.6
Ostium Primum is the second most common ASD and is associated with a mitral
valve defect known as a mitral valve cleft. This defect is most common among patients with
Downs syndrome. Unlike the other types of ASD, Ostium Primum patients present with
signs and symptoms of congenital heart failure at a young age. Ostium Primum is the most
difficult ASD to fix and requires the closure of the defect prior to valve reconstruction;
however, if done before the age of one, the surgery yields excellent results.6
The rarest form of ASD is Sinus Venosus. This defect is in the upper portion of the
atrial septum and often results in an abnormal connection between the pulmonary veins and
the heart. There are four pulmonary veins that bring oxygenated blood from the lungs back to
the heart. In Sinus Venosus ASD patients, however, one of these veins is connected to the
right atria instead of the left, dumping oxygenated blood into the right atria where it mixes
with deoxygenated blood that has just been returned from the body.6
In all forms of ASD, the magnitude of blood flowing through the defect depends on
the size of the defect and compliance of the ventricles. In general, the right ventricle is more
compliant than the left, and therefore blood flows from left to right through the defect. This
extra blood in the right ventricle causes the right ventricle to hypertrophy. This problem is
exacerbated by the development of pulmonary hypertension and incomplete ventricular
emptying, which often occurs later in adult life. As the resistance in the right ventricle
increases the left to right shunt declines and eventually the right ventricle becomes less
compliant than the left creating a shunt from right to left. This right to left shunt can
quickly become a serious problem as deoxygenated blood is mixed with blood about to be
pumped into systemic circulation, causing systemic ischemia.6
2. Ventricular Septal Defect
A defect in the ventricular septum that allows shunting of blood between the
ventricles. The direction of the shunt depends on the relative pressure between the two system
(pulmonic and systemic.7

Left-to-right shunt
A left-to-right shunt at the ventricular level has 3 hemodynamic consequences:

Increased LV volume load

Excessive pulmonary blood flow

Reduced systemic cardiac output

Blood flow through the defect from the LV to the RV results in oxygenated blood
entering the pulmonary artery (PA). The addition of this extra blood to the normal pulmonary
flow from the vena cava increases blood flow to the lungs and subsequently increases
pulmonary venous return into the left atrium (LA) and ultimately into the LV. This increased
LV volume results in LV dilatation and then hypertrophy. It increases end-diastolic pressure
and consequently LA pressure, then raises pulmonary venous pressure.
The increased pulmonary blood flow raises pulmonary capillary pressure, which can
increase pulmonary interstitial fluid. When this condition is severe, patients can present with
pulmonary edema. Therefore, both PA pressure and pulmonary venous pressure are elevated
in a VSD. The increase in pulmonary venous pressure is not seen with an atrial septal defect:
LA pressures are low because as blood can readily exit from this chamber through the atrial
communication.
Finally, as blood is shunted through the VSD away from the aorta, cardiac output
decreases, and compensatory mechanisms are stimulated to maintain adequate organ
perfusion. These mechanisms include increased catecholamine secretion and salt and water
retention by means of the renin-angiotensin system.
The degree of the left-to-right shunt determines the magnitude of the changes described
above. The left-to-right shunt depends on 2 factors, of which one is anatomic and the other
physiologic.
The anatomic factor is the size of the VSD. (The location of the VSD is irrelevant in terms of
the degree of the shunt.) In a normal heart, RV pressure is about 25-30% that of the LV. In a
large VSD, this pressure difference is no longer maintained, because a large hole offers no
resistance to blood flow. Consequently, these defects are called nonrestrictive VSDs.
On the other hand, in a small VSD, the normal pressure difference between the
ventricles is maintained. These defects are called restrictive VSDs because blood flow across
the defects is somewhat restricted, so that the normal pressure difference is maintained. The
physiologic factor is the resistance of the pulmonary vascular bed.
Changes in pulmonary vasculature

The terms pulmonary hypertension, high pulmonary resistance, and pulmonary

vascular disease are often confused. Pulmonary hypertension merely indicates a high blood
pressure in the pulmonary circuit; depending on the duration, it may be reversible. Pulmonary
resistance is a function of numerous factors, including age, altitude, hematocrit, and diameter
of the pulmonary arterioles.
A neonate has increased resistance secondary to the increase in the media of the
pulmonary arterioles; this decreases the effective diameter of the vessels. In addition,
neonates have a relative polycythemia. The elevated pulmonary resistance usually declines to
adult levels by 6-8 weeks.
Pulmonary vascular disease is ultimately an irreversible condition and may occur over
time in individuals with a large left-to-right shunt. It may also occur in the absence of a shunt;
this condition is called primary pulmonary hypertension. A characteristic series of histologic
changes ranging from grade I to grade VI has been described. The ultimate consequences of
pulmonary vascular obstructive disease are irreversible vascular changes and pulmonary
resistance equal to or exceeding systemic resistance.
Natural history
The natural history of VSD has a wide spectrum, ranging from spontaneous closure to
congestive heart failure (CHF) to death in early infancy.
Spontaneous closure frequently occurs in children, usually occurs by age 2 years.
Closure is uncommon after age 4 years. Closure is most frequently observed in muscular
defects (80%), followed by perimembranous defects (35-40%). Outlet VSDs have a low
incidence of spontaneous closure, and inlet VSDs do not close.
Closure may occur by means of hypertrophy of the septum, formation of fibrous
tissue, subaortic tags, apposition of the septal leaflet of the tricuspid valve, or (in rare cases)
prolapse of a leaflet of the aortic valve. When perimembranous VSDs close because of
development of fibrous tissue or the apposition of the tricuspid valve, an aneurysm of the
interventricular septum may appear.

A small VSD that does not spontaneously close is generally associated with a good
prognosis. Patients are at risk for infective endocarditis, but small muscular VSDs pose no
other adverse possibilities.
Small perimembranous VSDs, however, are associated with an increased risk of
prolapse of the aortic cusp over time. In addition, a small but definite risk of malignant
ventricular arrhythmia was reported in the Second Natural History Study. This study group
consisted of about 1000 patients (about 76% of the original cohort). The original cohort was
the First Natural History Study, which included 1280 patients (mostly children) with VSDs
admitted after cardiac catheterization between 1958 and 1969.
Wu et al reported a 45% incidence of LV-to-RA shunts and a 6% incidence of
subaortic ridges during a 20-year follow-up of about 900 patients with perimembranous
VSDs. This group later reported an increased incidence of infective endocarditis in patients
who had LV-to-RA shunts.8

Clinical Presentation of Atrial Septal Defect

1. Symptoms
Isolated ASD patients are usually asymptomatic and are most often detected at the
time of preschool physical examination. Sometimes these defects are detected when
echocardiographic studies are performed for some unrelated reason. A few patients do present
with symptoms of heart failure in infancy, although this is uncommon.
2. Physical examination.9
The right ventricular and right ventricular outflow tract impulses are increased and
hyperdynamic. No thrills are usually felt. The second heart sound is widely split and fixed
(splitting does not vary with respiration) and is the most characteristic sign of ASD. Ejection
systolic clicks are rare with ASDs. The ejection systolic murmur of ASD is soft and is of
grade I-II/VI intensity and rarely, if ever, louder. The murmur is secondary to increased blood
flow across the pulmonary valve and is heard best at the left upper sternal border. A grade III/VI mid-diastolic flow rumble is heard (with the bell of the stethoscope) best at the left
lower sternal border. This is due to large volume flow across the tricuspid valve. There is no
audible murmur because of flow across the ASD.9
3. Noninvasive evaluation

3.1. Chest x-ray

Chest film usually reveals mild to moderate cardiomegaly, prominent main pulmonary
artery segment and increased pulmonary vascular markings.9

3.2. Electrocardiogram
This ECG also shows right atrial overload as evidenced by P wave amplitude of 0.3
mV in lead II. Incomplete RBBB pattern is seen as slurred S waves in lead I and rSrS pattern
in V1..9

3.3. Echocardiogram
Echocardiographic studies reveal enlarged right ventricle with paradoxical septal
motion, particularly well-demonstrable on M-mode echocardiograms in patients with
moderate to large ASDs. Dilatation of the right ventricle may not be present in small defects.9

Clinical Presentation of Ventricular Septal Defect

Patients with ventricular septal defects may not have symptoms. However, if the hole
islarge, the baby often has symptoms related to heart failure.
The most common symptoms include:

Shortness of breath

Fast breathing

Hard breathing

Paleness

Failure to gain weight

Fast heart rate

Sweating while feeding

Frequent respiratory infections.10

Exams and Tests

Listening with a stethoscope usually reveals a heart pansistolic murmur (the sound of
the blood crossing the hole). The loudness of the murmur is related to the size of the defect
and amount of blood crossing the defect.
Tests may include:

Cardiac catheterization (rarely needed, unless there are concerns of high blood
pressure in the lungs)

Chest x-ray -- looks to see if there is a large heart with fluid in the lungs

ECG -- shows signs of an enlarged left ventricle

Echocardiogram -- used to make a definite diagnosis

MRI of the heart -- used to find out how much blood is getting to the lungs.10

Treatment
1. Treatment of Atrial Septal Defect
Treatment of an ASD depends on the type and size of the defect, its effect on the
heart, and the presence of any other related conditions, such as pulmonary hypertension,
valve disease or coronary artery disease. In general, when a patient has a large ASD that
causes significant shunting (flow of blood through the defect) and right-sided heart

enlargement, Cleveland Clinic specialists recommend correcting the defect. The size of the
defect correlates with the degree of shuntingthe more shunting, the greater the risk of longterm complications such as atrial fibrillation and pulmonary hypertension. The degree of
shunting is determined by echocardiography, MRI or oxygen saturations measured during
catheterization. The degree of right-heart enlargement, as measured by echocardiography or
MRI, usually correlates with the degree of shunting.11
ASD Repair
Nonsurgical Treatment
Nonsurgical, percutaneous (through the skin) repair is the preferred treatment for most
secundum ASDs, but surgery may be needed to repair other types of ASDs (see Surgerical
Repair section below for more information). Your doctor will determine what type of repair
procedure is best for you.Two different brands of closure devices are approved by the U.S.
Food and Drug Administration for percutaneous ASD closureAmplatzer Septal Occluder
and the GORE HELEX Septal Occluder. The closure devices differ in design, but the
placement method and their function are similar.The device is attached to a catheter, which is
inserted into a vein in the groin and advanced to the heart and through the defect, guided by
X-ray and intracardiac echo. As the device slowly is pushed out of the catheter, it opens up to
cover each edge of the defect, sealing it closed. Over time, tissue grows over the implant and
it becomes part of the heart.
Before a percutaneous closure device procedure, the patient will have a cardiac
catheterization to determine the size and location of the defect. Pressures inside the heart
chambers also will be measured. For at least the first six months after the repair, the patient
will need to take an anticoagulant such as aspirin, clopidogrel or warfarin (Coumadin) to
prevent clots from forming on the device.11
Percutaneous Closure Devices for ASD Repair

AMPLATZER Septal Occluder

The AMPLATZER Septal Occluder is a transcatheter closure device used to treat
ASDs. It consists of two Nitinol wire mesh discs filled with polyester fabric. It is folded into
a special delivery catheter, similar to the catheter used to cross the heart defect during
catheterization.
The catheter is inserted into a vein in the leg, advanced into the atrial septum and
through the defect. When the catheter is in the proper position, the device slowly is pushed
out of the catheter until the discs of the device sit on each side of the defect, like a sandwich.
The two discs are linked together by a short connecting waist that matches the size of the
defect. The discs and the waist are filed with polyester fabric to increase the devices closing
ability. Over time, heart tissue grows over the implant, and it becomes part of the heart,
permanently correcting the defect.
GORE HELEX Septal Occluder

The GORE HELEX Septal Occluder is a transcatheter closure device used to treat
ASDs. It is a disc-like device that consists of ePTFE patch material supported by a single
Nitinol wire frame. The device is folded into a special catheter and inserted into a vein in the
leg. Using a guide wire, the device is advanced through the atrial septum. When the catheter
is in the correct position, the device slowly is pushed out of the catheter until it covers the
defect. The device bridges the septal defect. Over time, heart tissue grows over the implant,
and it becomes part of the heart, permanently correcting the defect.11

Surgical Repair
Prior to the introduction of percutaneous techniques, surgical closure was the only
treatment option for an ASD, regardless of the type of defect. Surgical repair may be needed
for large secundum ASDs and other types of ASDs.
Surgical repair usually is performed using a tissue patch, preferably from the patients
own pericardium (the membrane around the heart). Some secundum ASDs can be surgically
closed with sutures alone.11
Follow-Up Care
The patient usually returns to the cardiologist 3, 6 and 12 months after a procedure for
a follow-up physical exam and echocardiogram, and once a year thereafter. After a secundum
ASD is repaired, most people can return to their regular activities without any activity
restrictions (other than those associated with all heart catheterizations). Patients usually take a
blood thinner for six months to a year after the repair to prevent blood clots and help the
healing process. Patients who have had a stroke may need to take blood thinners indefinitely,
and those with other heart problems, such as coronary artery disease or pulmonary
hypertension, may need to take additional medication.
Patients who have heart surgery to repair a defect or receive a transcatheter closure
device will need to take preventive antibiotics for at least six months after the repair
procedure to reduce the risk of infective endocarditis. The doctor will provide specific
guidelines about when to take antibiotics. According to the American Heart Association, there
is not enough evidence to recommend taking preventive antibiotics for longer than six
months.11

2. Treatment of Ventricular Septal Defect

Medical Management
The management in the infant and child depends on symptoms. A small defect does
not require medical management or likely require any intervention. The medium and larger

defects require various degrees of medical management and eventual surgical closure.
Congestive heart failure in the infant is treated with diuretics, digoxin, and afterload
reduction at times.
The adult with an unrepaired VSD in the current era likely has a small defect without
evidence of left ventricular volume overload or alterations in the adjacent structures. Those
with evidence of left ventricular volume overload or progressive aortic valve disease in most
institutions are referred for closure.
The adult who has had VSD repair needs surveillance for aortic valve dysfunction.
Those adults with residual defects need continued monitoring and consideration for
reoperation if there is left ventricular volume overload or progressive aortic valve
dysfunction.
The patient with Eisenmenger syndrome needs very specialized care at centers, with
trained personnel capable of managing myriad medical problems. Arrhythmias, endocarditis,
gallstones, gouty arthritis, hemoptysis, pulmonary artery thrombosis, and symptomatic
hypertrophic osteoarthropathy are frequently seen. Pregnancy is poorly tolerated and many
believe contraindicated in this disorder. Echocardiography and magnetic resonance imaging
are used to evaluate right ventricular function. Cardiac catheterization is reserved for cases in
which surgical or device closure is a question. Vasodilator therapy is an important adjunct to
management and can provide functional improvement. Changes in VVO2with exercise or
Qp:Qs from magnetic resonance imagingderived cardiac output can be determined but are
not generally used to guide therapy.
Endocarditis is a lifelong risk in unoperated patients (18.7 per 10 000 patient-years)
and those with residual defects. Proper prophylaxis and periodic follow-up are indicated.12
Surgical Closure
Location has been used as an indication for surgical closure regardless of the need for
medical management in the case of infundibular defects. Chamber enlargement is another
measure of the degree of shunting and may indicate the need for closure. Catheterization can
be used in some individuals to determine Qp:Qs and pulmonary artery pressure and resistance
to help guide clinicians. Generally, a Qp:Qs of 1.5:1 to 2:1 or evidence of increased

pulmonary arteriolar resistance is an indication for closure. Multiple Swiss cheese defects
refractory to medical management may require a palliative pulmonary artery band procedure.
Advances in surgical and bypass techniques and timing of surgical repair have
decreased the morbidity associated with surgical closure. The early era of repair showed an
80% closure rate in catheterized patients at long-term follow-up. In that study, 9 of 258
patients had complete heart block, 37 had transient heart block, and 168 had right bundlebranch block. Endocarditis occurred in 9 patients (11.4 of 10 000 patient-years).
More recent studies have shown residual defects in 31% of patients and an incidence
of complete heart block of 3.1%. Another natural history study showed occurrence rates for
pacemaker placement of 9.8 per 10 000 patient-years and occurrence rates for endocarditis of
16.3 per 10 000 patient-years in operated patients.12
Catheter Closure
Advancements in catheter techniques and devices are leading us into the era of
percutaneous closure of VSDs. The benefits of avoiding bypass are intuitive, and the relative
ease of placement makes this procedure ultimately attractive. Currently, these devices are in
the investigational stage. In 1987, Lock and colleagues used the Rashkind double-umbrella
device to close VSDs. The defects closed in that study included congenital, postoperative
congenital, and postmyocardial infarction VSDs. The Amplatzer VSD occluder, of which
there are the muscular and perimembranous types (AGA Medical Corp, Golden Valley,
Minn), is another investigational devices. A phase 1 clinical trial for the Amplatzer
membranous device showed a 96% complete closure rate at 6 months with a serious adverse
event rate of 8.6%. Likewise, there was 100% occlusion of single defects at 3 to 96 months of
follow-up with the Amplatzer muscular VSD occluder. Using the device for iatrogenic defects
after aortic valve replacement has also been successful.
Imaging during deployment traditionally has been transesophageal echocardiography.
Intracardiac echocardiography can now be used with accurate measurements and safety
similar to that with transesophageal echocardiography.
Device placement is not without its own risks and potential long-term complications.
Complete heart block has been observed as a temporary complication in 1.07% to 1.9% of

patients. There was also transient bundle-branch block in 2.8%. There was no late
development of complete heart block. Tricuspid stenosis was seen in 1 patient requiring
ballooning of the valve as a result of hemodynamic instability, after which the stenosis was
reduced and remained stable. Tricuspid regurgitation developed in 1 patient (0.7%).
Placement failure was experienced by 5.1% of patients as a result of proximity to the aortic
valve and acute insufficiency, chordae of the tricuspid valve, and inability to pass the delivery
sheath.
Studies using an open chest animal model and perventricular technique for device
deployment have been successful for perimembranous defects. A similar technique has been
used for muscular or multiple muscular defects. This provides a further reduction in the
invasiveness of closure and could allow therapy for those with contraindications to bypass in
the future.12

Oblique short-axis view shows Amplatzer device (arrow) placed to close a

midmuscular VSD. LV indicates left ventricle.

The aim of this paper is to report a case of atrial septal defect and ventricular septal defect
in a 2 months old boy.
Case
Name

: GPPS

Age

: 2 months

Sex

: Male

Date of Admission

: March, 25th 2014

Main Complaint: diarrhea

History: diarrhea presents since last night before came to Adam Malik. Frequency 10 times.
Water > dregs. Slime (-), blood (-).
Vomiting presents since 3 days ago. Frequency 5 times every time water and milk are
given. Vomit is yellowish and white yellowish slime was found.
Fever (+). Fever is not too high. Decrease with antipyretic.
Spastic (-).
Decreasing weight (+). Presents since 29 days ago when was hospitalized in RSUP Lubuk
Pakam.
Cyanotic (-). History of cyanotic (-). Shortness of breath (-).
History of previous illness : the patient was referred from RSU Medistra Lubuk Pakam by
a general practitioner. Hospitalized for 1 night in RSU Medistra with diagnosis GE mildmoderate dehydration. Was hospitalized in RSU Lubuk Pakam for 29 days. according to
patients parents, the patient has a heart disorder without knowing the name of the disorder.
History of previous medications: D10% 10 gtt/i micro
History of labor

: sectio caesaria , no cyanosis

History of feeding

: lactogen (formula milk)

History of immunization

: (-)

Presens status
Sensorium

: Compos Mentis

Anemis

: (-)

Body temperature

: 37,9oC

Icteric

: (-)

Respiratory Rate

: 32 x/minute

Cyanosis

: (-)

Pulse

: 170 bpm

Dyspnea

: (-)

Physical Examination
weight : 2435 gr

BW/Age: z-score < -3

length : 47 cm

BH/Age: -2 < z-score < 0

BW/BL: -3 < z-score < -2

1. Head: fonticulus anterior flatly opened. Eye: light reflexes(+/+), isocor pupil, pale
conjunctiva palpebra inferior (-/-), icteric (-/-), Ear : normal, Nose: normal, Mouth:
normal
2. Neck: Lymph node enlargement (-)
3. Thorax

: Symmetrical fusiformis, retraction (-)

HR: 170 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 32 x/minute, regular, crackles (-/-), wheezing (-)
4. Abdomen: soepel, peristaltic (+) normal. Liver/Spleen/Renal: not palpable. Anogenital :
male, Anus (-)
5. Extremities: Pulse 170 bpm, regular, adequate vascular pressure and
volume, warm acral, CRT< 3
Laboratory Findings on March 25th 2014
Carbohydrate metabolism : Random blood glucose 116 mg/dL
Electrolyte : Na 132 mEq, K 4.7 mEq, Cl 101 mEq
Complete Blood Count : hemoglobin 7.5 g%, erythrocyte

2.56x106/mm3, leucocyte

23.79x103/mm3, haematocryte 22.7 %,Trombocyte 489x103/mm3, MCV 88.70 fL, MCH 29.30
pg, MCHC 33 g%, RDW 15.50 %, MPV 9.20 fL, PCT 0.45 %, PDW 9.8 fL, Neutrophil
77.90 %, lymphocyte 9.70 %, monocyte 11.70 %, eosinophil 0.30 %, basophil 0.400 %,
Absolute neutrophil 18.52x103/L, Absolute lymphocyte 2.3x103/L, Absolute monocyte
2.79x103/L, Absolut eosinophil 0.08x103/L, Absolute basophil 0.10x103/L.
Renal : urea 50.30, creatine 0.27
Working Diagnosis
GE mild-moderate dehydration +
ASD/VSD/PDA

Treatment

moderate malnutrition + acyanotic CHD ec

NGT
IVFD RL 75cc/kgBW/4hr 45cc/hr IVFD D5% NaCl 0,225% 10gtt/i
Vitamin A 100000 IU (once)
Multivitamin without Fe 3xCthI
Advices : consult to nutrition and cardiology division
Follow up on March 26th - 28th 2014
S : diarrhea (-), fever (-), vomit (-)
O: Sensorium : Compos Mentis, Body temperature: 36.9oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(-)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 124 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 26 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : soepel, peristaltic (+) normal,Liver/Spleen/Renal: not palpable

Extremities : Pulse 124 bpm, regular, adequate vascular pressure and volume, Warm acral,
CRT< 3
A : GE without dehydration + moderate malnutrition + acyanotic CHD ec dd/ ASD/VSD/PDA
P :

IVFD D5% NaCl 0.225% 10 gtt/i

Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg
Diet Neosure 30cc/2hr/NGT + 0,5cc mineral mix

Laboratory findings on March 26th 2014

ALP 95 U/L , SGOT 53 U/L , SGPT 43 U/L
Ureum 30.80 mg/dL , Creatine 0.26 mg/dL , Uric Acid 3.6 mg/dL
CRP negative
Procalcitonin 1.30 mg/dL
Echocardiograph (March 27th 2014) : Small VSD PM + Small ASD + Left Sided
Enlargement
Advices : Furosemid 2x3 mg

Spironolacton 2x6.25 mg
Follow up on March 29th 30th 2014
S : diarrhea (-), fever (-), vomit (-)
O: Sensorium : Compos Mentis, Body temperature: 36.4oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(-)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 140 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 28 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : soepel, peristaltic (+) normal,Liver/Spleen/Renal: not palpable

Extremities : Pulse 140 bpm, regular, adequate vascular pressure and volume, Warm acral,
CRT< 3
A : small ASD + small VSD + moderate malnutrition
P:
IVFD D5% NaCl 0.9% 10 gtt/i
Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg
Diet Neosure 30cc/2hr/NGT + 0,5cc mineral mix
Follow up on March 31st 2014
S: fever (-), diarrhea (+), vomit (+)
O: Sensorium : Compos Mentis, Body temperature: 36,7oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(-)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 130 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 30 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : distention (+), peristaltic (+) , Liver/Spleen/Renal: not palpable

Extremities : Pulse 130 bpm, regular, adequate vascular pressure and volume, Warm acral,

CRT< 3
A : small ASD + small VSD + moderate malnutrition
P:
IVFD D5% NaCl 0.9% 10 gtt/i
Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg
Diet Neosure 30cc/2hr/NGT + 0,5cc mineral mix
Follow up on April 1st 2014
S: diarrhea (+), abdomen circumference
O: Sensorium : Compos Mentis, Body temperature: 36,7oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(-)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 100 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 25 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : soepel, peristaltic (+) N , Liver/Spleen/Renal: not palpable

Extremities : Pulse 100 bpm, regular, adequate vascular pressure and volume, Warm acral,
CRT< 3
A : small ASD + small VSD + moderate malnutrition
P:

IVFD D5% NaCl 0.9% 10 gtt/i

Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg
Folat Acid 1x1 mg
Diet Neosure 30cc/2hr/NGT + 0,5cc mineral mix

Follow up on April 2nd 2014

S:diarrhea (+), abdomen circumference
O: Sensorium : Compos Mentis, Body temperature: 36,6oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(-)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 120 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 25 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : soepel, peristaltic (+) N , Liver/Spleen/Renal: not palpable

Extremities : Pulse 115 bpm, regular, adequate vascular pressure and volume, Warm acral,
CRT< 3, pitting edema (+)
A : small ASD + small VSD + moderate malnutrition
P:

IVFD D5% NaCl 0.9% 10 gtt/i

Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg
Folat Acid 1x1 mg
Diet F100 45cc/3hr + 0.9cc mineral mix

Follow up on April 3rd 2014

S:diarrhea (+), dyspneu (+)
O: Sensorium : Compos Mentis, Body temperature: 37oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(+)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: normal, Mouth: normal

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 108 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 26 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : soepel, peristaltic (+) N , Liver/Spleen/Renal: not palpable

Anogenital : rectal tube (+)
Extremities : Pulse 108 bpm, regular, adequate vascular pressure and volume, Warm acral,
CRT< 3
A : small ASD + small VSD + moderate malnutrition
P:

IVFD D5% NaCl 0.9% 10 gtt/i

Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Zinc 1x10mg

Folic Acid 1x1 mg

Diet F100 45cc/3hr + 0.9cc mineral mix
PRC transfusion 8cc

Laboratory findings on April 3rd 2014

Hemoglobin 2.90 g% , Haematocryte 9% , Leucocytes 10.22x103/mm3 , Platelets 49x103/mm3
Ferum 14 mg/dL , TIBC 54 g/dL
Albumin 1.4 g/dL
Random blood glucose 107.2 mg/dL
Calcium 7.7 mg/dL, Natrium 129 mEq/dL , Kalium 5.2 mEq/dL , Chloride 96 mEq/dL ,
Magnesium 2.04 mEq/dL
Follow up April 4th 2014
S: fever (-), vomit (+), diarrhea (-), pale (+), dyspneu (+), cyanosis (-)
O: Sensorium : Compos Mentis, Body temperature: 36.9oC
Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(+)
Head

: Eye: light reflexes(+/+), isocor pupil, pale conjunctiva palpebra inferior (-/-),
icteric (-/-), Ear : normal, Nose: NGT (+), Mouth: pale mucosa on lips (+)

Neck

: Lymph node enlargement (-)

Thorax

: Symmetrical fusiformis, retraction (-)

HR: 142 bpm, regular, pansystolic murmur (+) grade III/6, LMCS III-IV
RR: 38 x/minute, regular, crackles (-/-), wheezing (-)

Abdomen : distention (+), peristaltic (+) , Liver/Spleen/Renal: not palpable

Anogenital : male
Extremities : Pitting edema (+). Pulse 142 bpm, regular, adequate vascular pressure and
volume, Warm acral, CRT< 2
A : small ASD + small VSD + moderate malnutrition
P:

IVFD D5% NaCl 0.225% 4cc/hr

Ceftazidime injection 70mg/8hours/IV skin test
Gentamicine injection 22mg/24hours/IV 17mg/24hours/IV (if UOP is enough)
Furosemid 2x3 mg
Spironolacton 2x6,25 mg
Multivitamin Syrup without Fe 1xcthI
Folic Acid 1x1 mg
Diet F75 30cc/2hr/NGT + 0.6cc mineral mix
Resomal 20cc/diarrhea

PRC transfusion 8cc

Dipstick result:
Leu
-

Nit
-

Uro
0.2(3.5)

Pro
-

pH
5

Blo
+

SG
1.015

Ket
-

Bil
-

Glu
-

April 5th 2014 Follow up before exitus

Time
12.00
12.15
12.30
13.00
13.30
14.00

Sense
GCS 12
GCS 12
GCS 9
GCS 9
GCS 9
GCS 5

HR (bpm)
154
148
118
110
86
54

RR(x/minute)
48
32
36
28
22
14

Temp.(oC )
36,5
36,4
36,2
36
36
36

Notes
Dyspnea

VTP 40-60X/minute
VTP+RJP,
pupil
dilatation
4mm,inj.Epineprine

14.30

GCS 3

36

1:10.000 0,3cc/IV
EXITUS because of
severe infection

Discussion
Patient GPPS came to Adam malik general hospital with main complaint diarrhea.
Diarrhea happened since the night before GPPS came to Adam Malik. Water was more than
dregs. Slime was not found. Blood was not found. Vomit was found since these 3 days, more
than 5 times. Spastic was not found. Decreasing body weight was found for 29 days since
GPPS was hospitalized in RS Medistra Lubuk Pakam. GPPS was referred from RSU
Medistra Lubuk Pakam by a general practitioner. Hospitalized for 1 night in RSU Medistra
with diagnosis GE mild-moderate dehydration and was treated with liquid replacement. Was
hospitalized in RSU Lubuk Pakam for 29 days. according to patients parents, the patient has
a heart disorder without knowing the name of the disorder. On March 27th 2014, the
echocardiograph showed that GPPS had small ASD (1.1 mm) and small VSD (3.1 mm).
This patient had asymptomatic clinical manifestation of small ASD and small VSD.
This patient had moderate malnutrition which is typical clinic manifestation of ASD 13.
Pansystolic murmur was found in this patient which is a typical physical examination of
VSD.10 Pansystolic murmur in this patient caused by huge difference of right and left

ventricle. Murmur located at mid clavicula sinistra line III-IV so it classified as grade III/VI
of murmur.
The diagnosis of small SD and small VSD was confirmed by echocardiogram.
Echocardiogram is the procedure of choice to confirm the diagnosis and to characterize ASD
and VSD. In this patient small ASD (1.1 mm) and small VSD (3.1 mm) was imaged whereas
was told from reference that small ASD and VSD

is small than 5 mm. 13 M-mode

echocardiography is used to measure the cardiac chamber sizes and quantitate left ventricular
systolic function. In a patient with a small ASD and VSD, chamber sizes are usually normal,
although mild left atrial and or left ventricular enlargement may be present. On
echocardiogram examination, dilated left ventricle and left atrium is found in this patient.9
GPPS is given Furosemid and Spironolactone. These drugs reduce pre-load and afterload in aim to decreased burden of the heart. Furosemid is loop diuretics drug, mechanism of
action furosemid inhibits reabsorption of sodium and chloride ions at proximal and distal
renal tubules and loop of henle by interfering with chloride-binding cotransport system,
causes excretion increases in water, calcium, magnesium, sodium and chloride.15
Mechanism of action of spironolactone is aldosterone antagonist with diuretic and
antihypertensive effects. Competitive binding of receptors at aldosterone-dependent Na-K
exchange site in distal tubules results in increased excretion of Na+, Cl-, and H2O and
retention of K+ and H+. Aldosterone receptor antagonist medicines may be a good option for
people with heart failure who are already taking other medicines such as ACE inhibitors,
other diuretics, digoxin, and beta blocker.14
Administer antibiotics in patient during instances of high exposure to bacteremia is
needed. As recommended by the american heart association for the prevention of bacterial
endocarditis. Ceftazidime and gentamicin are antibiotics drugs which used for eliminate and
prevention bacterial endocarditis. Ceftazidime is third-generation cephalosporin with broadspectrum gram-negative activity, including Pseudomonas; has lower efficacy against grampositive organisms and higher efficacy against resistant organisms; arrests bacterial growth
by binding to 1 or more penicillin-binding proteins, thereby, in turn, inhibiting final
transpeptidation step of peptidoglycan synthesis in bacterial cell-wall synthesis and inhibiting
cell-wall biosynthesis.

Gentamycin is Aminoglycoside antibiotic for gram negatif coverage bacteria

including pseudomonas species. Synergistic with beta-lactamse against enterococci.
Interferes with bacterial protein synthesis by binding to 30s and 50s ribosomal subunits.

Conclusion
This paper reports a case of a 1 month old male diagnosed with moderate malnutrition
+ small ASD + small VSD. A comprehensive work up had been done to confirm the
diagnosis. The treatment for this patient includes Furosemide and Spironolactone for
reducing pre-load, Ceftazidime and Gentamicin for bacterial endocarditis prevention and
eradication. O2 to treat shortness of breath, and adequat diet for malnutrition.
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