Lapkas Anak - Qarina Dian Edited

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 patient’s 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

Down’s 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)

http://emedicine.medscape.com/article/957343-overview

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 I-

II/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 shunting—the more shunting, the greater the risk of long-

term 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 closure—Amplatzer 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 device’s 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 patient’s

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 VM O2with exercise or

Qp:Qs from magnetic resonance imaging–derived 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 bundle-

branch 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 post–myocardial 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 mild-

moderate 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/µL, Absolute lymphocyte 2.3x103/µL, Absolute monocyte

2.79x103/µL, Absolut eosinophil 0.08x103/µL, Absolute basophil 0.10x103/µL.

Renal : urea 50.30, creatine 0.27

Working Diagnosis

GE mild-moderate dehydration + moderate malnutrition + acyanotic CHD ec

ASD/VSD/PDA

Treatment

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 (-)



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 (-)









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


CRT< 3”

A : small ASD + small VSD + moderate malnutrition

P :


Furosemid 2x3 mg

Spironolacton 2x6,25 mg


Zinc 1x10mg


Follow up on March 31st 2014

S: fever (-), diarrhea (+), vomit (+)

O: Sensorium : Compos Mentis, Body temperature: 36,7oC








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


CRT< 3”


P :


Furosemid 2x3 mg



Zinc 1x10mg


Follow up on April 1st 2014

S: diarrhea (+), abdomen circumference ↑









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


CRT< 3”


P:


Furosemid 2x3 mg



Zinc 1x10mg

Folat Acid 1x1 mg


Follow up on April 2nd 2014

S:diarrhea (+), abdomen circumference ↓











CRT< 3”, pitting edema (+)


P:


Furosemid 2x3 mg



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(+)








Anogenital : rectal tube (+)


CRT< 3”


P:


Furosemid 2x3 mg



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 µg/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 (-)


Anemis (-),Icteric (-),Cyanosis(-),Dyspnea(+)


icteric (-/-), Ear : normal, Nose: NGT (+), Mouth: pale mucosa on lips (+)





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”


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



Folic Acid 1x1 mg

Diet F75 30cc/2hr/NGT + 0.6cc mineral mix

Resomal 20cc/diarrhea

PRC transfusion 8cc

Dipstick result:

Leu Nit Uro Pro pH Blo SG Ket Bil Glu

- - 0.2(3.5

)

- 5 + 1.015 - - -

April 5th 2014 → Follow up before exitus

Time Sense HR (bpm) RR(x/minute) Temp.(oC ) Notes

12.00 GCS 12 154 48 36,5 Dyspnea

12.15 GCS 12 148 32 36,4

12.30 GCS 9 118 36 36,2

13.00 GCS 9 110 28 36

13.30 GCS 9 86 22 36 VTP

40-60X/minute

14.00 GCS 5 54 14 36 VTP+RJP, pupil

dilatation

4mm,inj.Epineprine

1:10.000 0,3cc/IV

14.30 GCS 3 - - 36 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 ASD13.

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 after-

load 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 broad-

spectrum gram-negative activity, including Pseudomonas; has lower efficacy against gram-

positive 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.

References

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http://circ.ahajournals.org/content/114/20/2190.full

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