FINAL LAPKAS ANAK CHF-----

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Chapter 1

INTRODUCTION

1.1. Background

Congestive heart failure (CHF) refers to a clinical state of systemic and pulmonary

congestion resulting from inability of the heart to pump as much blood as required for the

adequate metabolism of the body. The clinical picture of CHF results from a combination of

relatively low output and compensatory responses to increase it. Excellent reviews on CHF

in infants and children are available.1 The diagnosis of CHF in older children is often straight

forward, but it may be difficult at times, to diagnose CHF or to distinguish it from pulmonary

disease or sepsis in the neonate. Feeding difficulties and excessive sweating are the usual

presenting features. Tachycardia >150/min is common, and heart rates >180/min are

abnormal even in the setting of respiratory distress and suggests CHF. Thus, the grading of

the severity of CHF in infants should include an accurate description of these historical and

clinical variables.2

In developing countries structural heart defects are the most common cause of heart

failure in infants and children. The incidence of congenital heart disease in children is

approximately 8 per 1000 live births, or 0.8%. 3 About one third to one half of these defects

are severe enough to produce symptoms that prompt treatment, catheterization or surgery, or

to cause death in the first year of life. Only about one half of these severe defects results in

CHF; interventions are performed in there maining defects because of cyanosis or potential or

actual circulatory collapse related to closure of the ductus arteriosus. Thus the yearly

incidence of heart failure from structural defects is about 0.1% to 0.2% of live births. The

defects most likely to cause heart failure include left-to-right shunt lesions (eg, ventricular

septal defect, common atrioventricular canal defect, patent ductus arteriosus,

aorticopulmonary window, truncus arteriosus), left heart obstructive lesions (eg, critical

aortic stenosis, severe aortic coarctation, congenital mitral stenosis), and congenital

atrioventricular or semilunar valve regurgitation.4

A ventricular septal defect (VSD) is a hole or a defect in the septum that divides the 2

lower chambers of the heart, resulting in communication between the ventricular cavities. A

VSD may occur as a primary anomaly, with or without additional major associated cardiac


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defects. A VSD occurs in approximately 2-6 of every 1000 live births and accounts for more

than 20% of all congenital heart diseases. The symptoms and physical findings associated

with ventricular septal defects (VSD) depend on the size of the defect and the magnitude of

the left-to-right shunt. Chest radiography, magnetic resonance imaging (MRI), andelectrocardiography (ECG) may all provide useful information in the workup of a VSD.

Although cardiac catheterization was a standard part of the evaluation in the past, detailed

echocardiography is now preferred in most institutions.5

Children with small VSD are asymptomatic and have an excellent long-term prognosis.

Neither medical therapy nor surgical therapy is indicated. In children with moderate or large

VSD, a trial of medical therapy is indicated to manage symptomatic congestive heart failure

(CHF) because many VSD may become smaller with time. Uncontrolled CHF with growth

failure and recurrent respiratory infection is an indication for surgical repair. Neither the age

nor the size of the patient is prohibitive in considering surgery.5

1.2. Objective

The aim of writing this paper is in order to complete the assignment in following the doctors

professional education program in the department of pediatrics. In addition, providing

knowledge to the author and readers about congestive heart failure ec ventricular septal

defect.


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Chapter 2

LITERATURE REVIEW

2.1. Congestive Heart Failure

2.1.1. Defenition


congestion resulting from inability of the heart to pump as much blood as required for the

adequate metabolism of the body. The clinical picture of CHF results from a combination of

relatively low output and compensatory responses to increase it.1

Over time, decreased cardiac output leads to a cascade of compensatory responses

that are aimed directly or indirectly at restoring normal perfusion to the body's organs and

tissues. For most adults, heart failure (HF) results from diminished myocardial contractility

caused by ischemic heart disease. In contrast, decreased contractile states account for a

smaller percentage of causes of pediatric HF. Instead, the various triggers of HF in children

can be categorized broadly as syndromes of excessive preload, excessive afterload, abnormal

rhythm, or decreased contractility, which all can lead to a final common HF pathway. 1,2

2.1.2. Prevalence

The over all incidence and prevalence of pediatric HF is unknown, largely because

there is no accepted universal classification applied to its many forms. The largest HF burden

comes from children born with congenital malformations. It has been estimated that 15% to

25% of children who have structural heart disease develop HF. 1 Although cardiomyopathy is

relatively rare, approximately 40% of patients who experience cardiomyopathy develop heart

failure of such severity that it leads to transplantation or death.6

2.1.3. Etiology


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Heart failure can result from cardiac and noncardiac causes. Cardiac causes include

those associated with congenital structural malformations (Table 2.1.) and those involving no

structural anomalies (Table 2.2.).

Table 2.1. Cardiac Malformations Leading to Heart Failure1,5

Shunt Lesions

Ventricular septal defect

Patent ductus arteriosus

Aortopulmonary window

Atrioventricular septal defect

Single ventricle without pulmonary stenosis

Atrial septal defect (rare)Total/Partial Anomalous Pulmonary Venous Connection

Valvular Regurgitation

Mitral regurgitation

Aortic regurgitation

Inflow Obstruction

Cor triatriatum

Pulmonary vein stenosis

Mitral stenosis

Outflow Obstruction

Aortic valve stenosis/subaortic stenosis/supravalvular aortic stenosis

Aortic coarctation

Table 2.2. Sources of Heart Failure With a Structurally Normal Heart1,5

Primary Cardiac

Cardiomyopathy

Myocarditis

Myocardial infarction

Acquired valve disorders

Hypertension Kawasaki syndrome

Arrhythmia (bradycardia or tachycardia)

Noncardiac

Anemia

Sepsis

Hypoglycemia

Diabetic ketoacidosis

Hypothyroidism

Other endocrinopathies

Arteriovenous fistula

Renal failure Muscular dystrophies


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2.1.4. Pathophysiology

Unmet tissue demands for cardiac output result in activation of the renin-aldosteroneangiotensin system, the sympathetic nervous system, cytokine-induced

inflammation, and recently appreciated signaling cascades that trigger cachexia.7 A vicious

cycle begins when decreased cardiac output leads to increased metabolite production in

downstream organ systems. These metabolites, in turn, stimulate local vasodilation and

decreased blood pressure. Falling blood pressure stimulates angiotensin and

mineralocorticoid release further, inducing fluid-retaining mechanisms in the kidney and

stimulating increases in systemic vascular resistance. Stimulation of the sympathetic nervous

system and increased release of catecholamines cause tachycardia, enhanced myocardial

contractility, and maladaptive forms of cardiac hypertrophy.

Initially, these effects help to improve cardiac output and maintain blood pressure. The

Pediatric Advanced Life Support course offered by the American Heart Association terms

this stage of HF compensated shock. However, HF occurs in diseases that are not readily

reversible. In these situations, long standing increases in myocardial work and myocardial

oxygen consumption (MVO2) ultimately worsen HF symptoms and lead to a chronic phasethat involves cardiac remodeling (Fig. 1).8

Cardiac remodeling is a structural transformation in which the normally elliptical heart

increases in mass and becomes more spherical. This increase in cardiac mass (maladaptive

cardiac hypertrophy) involves an expansion of the myofibrillar components of individual

myocytes (new cells rarely form), an increase in the myocyte/ capillary ratio, and activation

and proliferation of abundant nonmyocyte cardiac cells, some of which produce cardiac

scarring. Taken together, these processes produce a poorly contractile and less compliant

heart, resulting in increased filling pressures, pulmonary or systemic edema, hypoxia,

redistribution of blood flow away from skeletal muscle and the splanchnic circulation, tissue

lactic acidosis, and loss of lean body mass (cachexia). Cachexia is a state of

catabolic/anabolic imbalance leading to weight loss and disordered homeostasis and involves

inflammatory cytokines such as tumor necrosis factor-alpha (TNF-alpha) and interleukins, as

well as neurohormonal activation. Recent

work suggests That activation of cachexia

pathways may drive worsening HF.8


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Figure 2.1. Heart failure results from an interaction of beneficial and deleterious pathways

that ultimately modulate cardiac output and remodeling.8

Although decreased cardiac output stimulates deleterious neuroendocrine

mechanisms, endogenous mechanisms defend the heart from progressive HF. Thesemechanisms include stimulation of insulin-like growth factor and growth hormone and

secretion of atrial and brain natriuretic peptides (ANP and BNP). For example, growth

hormone deficiency and low insulin-like growth factor concentrations have been associated

with poor HF outcomes in adults, and increased concentrations appear to be protective. 9

2.1.5. Clinical Manifestation and Diagnosis

In infants, the symptoms of heart failure most commonly exhibited include tachypnea,

tachycardia, poor feeding, and failure to thrive. Other signs of heart failure in this group of

patients include hepatomegaly,diastolic gallop on physical examination, and cardiac

enlargement with or without pulmonary edema on chest radiograph. Toddlers and older

children may also exhibit tachycardia and tachypnea but typically manifest symptoms of

fatigue and exercise intolerance; poor appetite and growth failure are typical of this age

groupas well. In older children, venous distension and peripheral edema may be apparent as

well.

Until 1987, the only system available for grading HF in children was the New York

Heart Association (NYHA) classification. However, this system was based on limitations to

physical activity for adults, which did not translate well for use with children, particularly

infants. Therefore, we developed a symptom-based classification using more age-appropriate

variables (Table 3) and demonstrated that plasma norepinephrine correlated in a stepwise

fashion with this new Ross HF classification from grades I to IV. 10

Tabel 2.3 Original Ross classification10

I: No limitations or symptoms

II: Mild tachypnea or diaphoresis with feedings in infants, dyspnea at exertion in older

children; no growth failure

III: Marked tachypnea or diaphoresis with feedings or exertion and prolonged feeding

times with growth failure from CHF

IV: Symptomatic at rest with tachypnea, retractions, grunting, or diaphoresis


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2.1.6. Laboratory Studies

Because knowledge of the specific underlying disease is critical in the understanding ofthe pathophysiology, management, and response to therapy for HF, certain laboratory tests

are essential. Pulse oximetry is helpful in identifying cyanosis in infants who have HF caused

by increased pulmonary blood flow (left-to-right shunts) because recognizing cyanosis in an

infant is nearly impossible by physical examination alone. Decreased percutaneous oxygen

saturation never is associated with acyanotic heart disease unless poor tissue perfusion or

intrapulmonary right-to-left shunting occurs. The 12- lead electrocardiogram is essential to

assess arrhythmia induced HF. The chest radiograph may demonstrate cardiac enlargement,

increased pulmonary blood flow, venous congestion, or pulmonary edema. However, chest

radiographs generally have a high specificity but low sensitivity for detecting cardiac

enlargement. Although not useful for the evaluation of HF, which is a clinical diagnosis,

echocardiography is essential for identifying causes of HF such as structural heart disease,

ventricular dysfunction (both systolic and diastolic), chamber dimensions, and effusions (both

pericardial and pleural).11

2.1.7. Management

The first goal of HF care is to treat the specific cause. Prompt treatment of noncardiac

causes of HF such as anemia or endocrinopathies as well as timely referral for surgical

corrections of structural cardiac anomalies can prevent or ameliorate HF. Examples closing a

ventricular septal defect or patent ductus arteriosus, repairing a coarctation, or relieving a

valve obstruction.12

HF-causing cardiac malformations usually can be approached surgically. Accordingly,

medical management of HF serves as a temporizing measure if surgery must be delayed.

Because there is no cure for primary childhood cardiomyopathies, the goal of medical

management is to delay or eliminate the need for cardiac transplantation. 13

Medical management (Table 2.4) aims to maximizecardiac output and tissue perfusion

while minimizing stresses that increase MVO2. These goals are accomplished by reducing

the amount of force the heart needs to generate to eject blood (reducing afterload stress) and

by reducing overfilling of the heart (preload). Thus, treatments that rest the heart, such as

vasodilators, are preferred to inotropic agents that increase MVO2.14


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Afterload reduction is accomplished by using drugs that decrease the systemic vascular

resistance. These agents include angiotensin-converting enzyme inhibitors and type 4

phosphodiesterase inhibitors (milrinone) or systemic nitrates (nitroprusside).12,13 Another

approach to resting the failing heart is through inhibition of the sympathetic nervous system.Beta-blocker therapy is a cornerstone of the medical management of HF in adults.

Diuretics reduce preload, theory improving Frank- Starling relationships in the heart.

Decreased preload helps to prevent pulmonary edema-producing high cardiac filling

pressures. Besides loop diuretics such as furosemide, other classes of diuretics are used,

including thiazides and mineralocorticoid inhibitors (spironolactone). Recent data suggest

that aldosterone inhibition also helps to

prevent maladaptive cardiac remodeling and interstitial fibrosis.14

Digoxin is the only commonly used oral inotropic agent. However, itsmost important

mechanism of action may be its ability to blunt the sympathetic nervous system, slow the

heart rate, and increase cardiac filling time.

Table 2.4. Principles of Managing Heart Failure13

Recognition and Treatment of Underlying Systemic Disease

Timely Surgical Repair of Structural Anomalies

Afterload Reduction

Angiotensin-converting enzyme inhibitors

Angiotensin receptor blockers

Milrinone

Nitrates

Brain natriuretic peptide (BNP)

Preload Reduction

Diuretics

BNP

Sympathetic Inhibition

Beta blockers BNP

Digoxin

Cardiac Remodeling Prevention

Mineralocorticoid inhibitors Inotropy

Digoxin


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2.1.8. Prognosis

The outcome for patients experiencing HF depends largely on its cause. Whennoncardiac disorders are responsible, the improvement in HF is related to successful

treatment of the systemic disease. For many cardiac malformations (preloading and

afterloading conditions), surgical correction can be curative. Unfortunately, surgery for many

congenital heart lesions only palliates the underlying disease.13

In general, too little is known about HF risk assessment in children to permit confident

statements about prognosis and response to treatment. The New York Heart Association

Classifications, routinely used in adult studies, fail in pediatric applications because of the

unique presentations of HF in children. Alternatives, such as the Ross classification or the

New York University Pediatric HF Index also have not gained wide acceptance.12,14

2.2. Ventricular Septal Defect

2.2.1. Definition and Classification

A ventricular septal defect (VSD) is a hole or a defect in the septum that divides the 2

lower chambers of the heart, resulting in communication between the ventricular cavities.

The defect can be small or large . A VSD may occur as a primary anomaly, with or without

additional major associated cardiac defects.6

Many classifications of VSD have been proposed:6

a. Perimembranous (infracristal, conoventricular) VSDs lie in the LV outflow tract just

below the aortic valve. Because they occur in the membranous septum with defects in the

adjacent muscular portion of the septum, they are subclassified as perimembranous inlet,

perimembranous outlet, or perimembranous muscular. These are the most common types

of VSD and account for 80% of such defects.

b. Supracristal (conal septal, infundibular, subpulmonic, subarterial, subarterial doubly

committed, outlet) VSD account for 5-8% of isolated VSDs in the United States but 30%

of such defects in Japan. These defects lie beneath the pulmonic valve and communicate

with the RV outflow tract above the supraventricular crest and are associated with aortic

regurgitation secondary to the prolapse of the right aortic cusp.

c. Muscular VSDs (trabecular) are entirely bounded by the muscular septum and are often

multiple. The term Swiss-cheese septum has been used to describe multiple muscular

VSD. Other subclassifications depend on the location and include central muscular or
http://emedicine.medscape.com/article/899873-overviewhttp://emedicine.medscape.com/article/899873-overview


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midmuscular, apical, and marginal (when the defect is along the RV-septal junction).

These VSDs account for 5-20% of all defects. Any single defect observed from the LV

aspect may have several openings on the RV aspect.

d. Posterior (canal-type, endocardial cushiontype, AV septumtype, inlet, juxtatricuspid)VSDs lie posterior to the septal leaflet of the tricuspid valve. Although the locations of

posterior VSDs are similar to those of VSDs observed with AV septal defects, they are

not associated with defects of the AV valves. About 8-10% of VSDs are of this type.

2.2.2. Epidemiology

The most frequent types of congenital malformations affect the heart. It is estimated that

approximately eight in 1,000 newborns have CHD. A VSD is the most frequent of the various

types of CHD (25%-30% of all CHD). Approximately one infant in 500 will be born with a

VSD. The most common form of congenital heart disease in childhood is the VSD, occurring

in 50% of all children with congenital heart disease and in 20% as an isolated lesion. 15

2.2.3. Etiology

The definitve cause of any individual congenital heart defect is rarely determined.

Congenital heart defects are believed to be multifactorial with both environmental and

genetic components. Genetic risk factors include the presence of certain chromosomal

syndromes including: trisomies 13, 18, 21 or a family history of cardiac defects.

Environmental factors include: maternal diabetes or phenylketonuria, exposure to disease or

teratogens within in the first 8 weeks of gestation. It should be mentioned that a recent study

in Northern Ireland found that the risk of VSD was not increased by maternal smoking,

alcohol consumption or maternal age. Parent/family education should include the complexity

of cardiac development and medical sciences's limited inability to ascertain causality.16,17

2.2.4. Pathophysiology

The size of the VSD, the pressure in the right and left ventricular chambers, and

pulmonary resistance are factors that influence the hemodynamic significance of VSDs. A

VSD may not be apparent at birth because of the nearly equal pressures in the right and left

ventricles and a lack of shunting. With increasing shunt corresponding to the increasing

pressure difference between the ventricles, these defects become clinically apparent.

Exceptions to this rule are patients with Down syndrome who may not undergo the natural


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drop in pulmonary resistance and do not manifest signs of a VSD. Routine screening of all

Down syndrome patients is the standard of care.18

The shunt volume in a VSD is determined largely by the size of the defect and the

pulmonary vascular resistance. Without pulmonary hypertension or obstruction to the rightventricle, the direction of shunt is left to right, with corresponding pulmonary artery, left

atrial, and left ventricular volume overload. In the setting of elevated pulmonary vascular

resistance, right ventricular obstruction resulting from muscle bundles, or pulmonary

stenosis, the shunt volume is limited and may be right to left, depending on the difference in

pressure. Eisenmenger syndrome results from long-term left-to-right shunt, usually at higher

shunt volumes. The elevated pulmonary artery pressure is irreversible and leads to a reversal

in the ventricular level shunt, desaturation, cyanosis, and secondary erythrocytosis.

Muscular VSDs can undergo spontaneous closure as a result of muscular occlusion.

Perimembranous defects can close by tricuspid valve aneurysm formation. Infundibular

defects can close by prolapse of the right aortic cusp. A reduction in the size of the defect by

any of these mechanisms results in changes in the hemodynamic significance of the defect.

The integrity of structures immediately adjacent to ventricular defects is a concern. For

example, the development of aortic insufficiency in the case of infundibular defects is a result

of the deficiency of the support apparatus of the aortic valve and results in damage to the

aortic valve leaflets.19

2.2.5. Clinical Feature

VSDs can be detected by auscultation. The murmurs are typically described as

holosystolic or pansystolic. The grade of murmur depends on the velocity of flow; the

location of murmur is dependent on the location of the defect. Smaller defects are loudest and

may have a thrill. Muscular defects can be heard along the lower left sternal border and may

vary in intensity as the defect size changes with muscular contraction throughout systole.

Infundibular defects shunt close to the pulmonary valve and can be heard best at the left

upper sternal border. Perimembranous defects may have an associated systolic click of a

tricuspid valve aneurysm.20

In the setting of low pulmonary vascular resistance, larger defects have murmurs of

constant quality that vary little throughout the cardiac cycle and less commonly have an

associated thrill. These defects will have a corresponding increase in mitral flow, resulting in

a diastolic rumble at the apex. There may be evidence of left ventricular volume overload on


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palpation of the precordium with a laterally displaced impulse. Elevated pulmonary pressure

causes an increase in the pulmonary component of the second heart sound. Large defects with

no shunt and defects with Eisenmenger physiology and right-to-left shunt often do not have a

VSD murmur.

18,19

2.2.6. Diagnostic Evaluation

Electrocardiography

The ECG is most likely normal in patients with small VSDs. With increasing

shunt, there may be evidence of left ventricular volume load and hypertrophy. Left atrial

enlargement may be present. In cases of elevated pulmonary artery pressure, right axis

deviation, right ventricular hypertrophy, and right atrial enlargement may be evident on

ECG.

Chest Radiography

Small defects have no apparent radiographic abnormality. With larger defects,

chamber enlargement is present to various degrees, depending on the volume of the shunt.

Increased pulmonary vascularity is present. As patients develop Eisenmenger syndrome

or increasing pulmonary resistance, there is loss of pulmonary vascularity and pruning of

the vasculature. In these patients, there is evidence of right heart enlargement and a

dilated main pulmonary artery.

Echocardiography

Echocardiographic evaluation of VSDs is a noninvasive tool that accurately

delineates the morphology and associated defects. Hemodynamic evaluation of the defect,

the presence of elevated pulmonary artery pressure, obstruction of the right ventricular

outflow tract (double chamber physiology), insufficiency of the aortic valve, and

distortion of the valve apparatus are all evaluated by echocardiography. When limitations

in image quality of transthoracic echocardiography prevent evaluation of these aspects of

the cardiovascular physiology, transesophageal imaging can be performed. Three-

dimensional echocardiography has proved accurate for quantifying shunt and can provide

accurate visualization of defects that otherwise are difficult to evaluate by 2-dimensional

imaging alone.

Magnetic Resonance Imaging

Magnetic resonance imaging can be used to delineate VSDs in patients with

complex associated lesions.


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Cardiac Catheterization

Catheterization can give accurate measurements of pulmonary vascular resistance,

pulmonary reactivity, and volume of shunting. Response to pulmonary vasodilators can

be determined and can guide therapy. Angiography can provide information on thelocation of a defect, number of defects, and the degree of aortic insufficiency. The aortic

valve can be inspected for integrity.

2.2.7. Management

Medical Management of of Symptomatic CHF:5

Therapies used to manage symptomatic CHF in children with moderate or large VSDs may

include the following:

Increased caloric density of feedings to ensure adequate weight gain - Occasionally,

oral feeds must be supplemented with tube feeds because a baby in CHF may be

unable to consume adequate calories for appropriate weight gain

Diuretics (eg, furosemide) to relieve pulmonary congestion - Furosemide is usually

given in a dosage of 1-3 mg/kg/d divided in 2 or 3 doses; long-term furosemide

treatment results in hypercalcemia and renal damage and electrolyte disturbances

Angiotensin-converting enzyme (ACE) inhibitors (eg, captopril and enalapril) - Thesemedications reduce both the systemic and pulmonary pressures (the latter to a greater

degree), thereby reducing the left-to-right shunt

Digoxin (5-10 g/kg/d) - This may be indicated if diuresis and afterload reduction do

not relieve adequately symptoms.

Surgical Closure5

At present, direct surgical repair using cardiopulmonary bypass is the preferred

surgical therapy in most centers. Most perimembranous and inlet VSDs are repaired via a

transatrial surgical approach. Defects in the outlet septum are approached through the

pulmonary valve. Multiple muscular defects, especially near the apex, pose a difficult

problem. Initial pulmonary banding or left ventricular (LV) approach through an apical left

ventriculotomy and closing the defect with a single patch are the standard techniques.

Short-term results of video-assisted cardioscopy for intraventricular repair of VSD have led

to its wide adoption as a means of reducing surgical trauma. Short-term results are

excellent.


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Transcatheter therapy (see below) remains an experimental approach. A hybrid operation is a

joint procedure involving the interventional cardiologist and the cardiac surgeon, who

concomitantly optimize surgical management of complex congenital heart disease. This

approach may be used for multiple VSDs where the perimembranous VSD is repairedsurgically and the muscular VSDs are closed with a transcatheter device.

Transcatheter closure

Muscular VSDs have been closed with transcatheter devices for the past 15 years.

Perimembranous VSDs, though relatively common, can be difficult to close percutaneously.

With previous devices (eg, Rashkind or button devices), attempts to close the VSDs have

been unsuccessful, because of the proximity of the defects to the aortic valve and potential

aortic valve damage.

Most procedures are performed with the patient under general anesthesia and with

echocardiographic guidance. Reported complications have included aortic and tricuspid

regurgitation, device embolization, complete heart block, transient left bundle-branch block

(LBBB), hemolysis, small residual shunts, and perforation.

In a phase I study, Fu et al reported 3 adverse events of complete heart block, perihepatic

bleeding, and rupture of tricuspid valve chordae tendineae. In a previous article, they reported

2 cases of transient heart block that responded to high-dose steroids. Subsequent studies

found that the Amplatzer membranous VSD occluder resulted in excellent closure rates but

had an unacceptably high rate of complete heart block.


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Chapter 3

CASE REPORT

IDENTITY

Name : FR

Age : 4 months

Sex : Female

RM : 54.14.51

Address : Jl. Brigjen Katamso Gg. Pemuda No. 35 Medan

Date of Admission : December, 17th 2012

Major Complaint : Shortness of breath

History :

FR, 4- month- year old girl, weight 4200 gram, height 60 cm, was admitted to Pediatric

Department at Haji Adam Malik General Hospital on December17th 2012 with the main

complaint of shortness of breath.

It has already been experienced since the birth of patient. This shortness of breath is not

related to the weather. The complaint is not accompanied with a wheezing soundor bluish

around the mouth or on the fingertips of hands and feet. According to her mother, she has

drinking problems since birth, sweating a lot during feeding and also crying. Coughing is

found for 2 weeks by now, with white phlegm. Fever has been found for these two weeks,

fever is up and down, fever is down when she is given a febrifuge.

History of Previous Illness : FR previously went to MitraSejati Hospital with the same

complain and was diagnosed with Acyanotic Congenital Heart Disease + pneumoniae by

pediatrician.

History of Previous Medication: Asering, Inj. Ceftriaxone, duminsupp

History of Pregnancy: The age of mother was 27 years. History of fever and sore throat was

found. She took aspirin and herbal when the first month of pregnancy. There is no history of

diabetes mellitus, hypertension, smoking, and alcohol consumption. History of stillbirth was

not found.


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History of birth: Patient is the fourth child. Patient was born spontaneously by the midwife

and not immediately crying, and motion was positive. Birth weight was 3900 grams, birth

length was 50 cm, and history of cyanosis was not found.

Feeding History

From birth to 2 months : Breast milk only

From 2 months to 3 months 3 weeks : Breast milk and rice porridge

From 3 months 3 weeks to now : Breast milk and SGM 1 milk

Physical Examination

Generalized status

Body weight : 4200 g

Body length : 60 cm

Body weight in 50th percentile according to age : 6200 g

Body length in 50th percentile according to age : 62 cm

Body weight in 50th percentile according to body length : 5800 g

BW/age :6,2

4,2x 100% = 67,74 %

BL/age : 62

60

x 100% = 96,7 %

BW/BL :5,8

4,2x 100% = 72,41 %

Presence Status

Sensorium: alert, BP= 100/50 mmHg, HR= 140 bpm, RR= 60 x/min, temperature: 36.5oC.

Anemic (-), dyspnea (+), cyanotic (-), edema (-), icteric (-). Body weight (BW): 4200 g. Body

length (BL): 60 cm. CDC: BW/Age = 67,74 %, BL/Age = 96,7 %, BW/BL = 72,41 %

Localized Status

Head : Eye: light reflex (+/+), isochoric pupil, 3 mm, pale inferiorcon-

junctivapalpebra (-/-) Old man face (+),

Nose: Nostril breathing (+), NGT (+). Mouth: within normal limit. Ear :

within normal limit.

Neck : Lymph node enlargement (-), TVJ R-2 cm H2O


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Thorax : Symmetric fusiform, retraction (+) suprasternal, intercostals, epigastrial,

bulging (+), HR: 140 bpm, regular, pansystolic murmur(+), RR: 60 x/minute,

regular, ronchi (+/+), wheezing (+/+)

Abdomen : Soepel (+), peristaltic (+), liver and spleen not palpable.Extremities : Pulse = 140 bpm, regular, adequate pressure/volume, warm axilla, capillary

refill time(CRT) < 3, clubbing finger (-).

Genitalia : Female, within normal limit.

Chest X-Ray AP 17 December 2012:


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Laboratory Result : December 17, 2012

HEMATOLOGY

Test Result Unit ReferralHb 9,0 gr% 10,7 17,1

WBC 7,6 x 103/mm 6,0 17,5

RBC 3,4 x 106/mm 3,75 4.95

Hematocrite 26,5 % 38 52

PLT 373 x 10/mm 217 497

MCV 77,9 fL 93 115

MCH 26,5 Pg 29 35

MCHC 34 g% 28 34

RDW 15,2 % 14.9 18.7Neutrophil 51,3 % 37 48

Lymphocyte 34,3 % 20 40

Monocyte 14,2 % 2 8

Eosinophil 0,10 % 1 6

Basophil 0,100 % 0 1

CLINICAL CHEMISTRY

Test Result Unit Referral

Analysis of the gas blood

pH 7,460 7,35 7,45

pCO2 46,3 mmHg 38 42

pO2 179,9 mmHg 85 100

HCO3 32,1 mmol/L 22 26

Total CO2 33,6 mmol/L 19 25

BE 7,2 mmol/L (-2) (+2)

Saturation O2 99,3 % 95 100

Metabolism of CarbohidrateBlood glucose 70 mg/dl


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Differential Diagnosis:

1. CHF ec. Acyanotic CHD ec. VSD + Bronkhopneumonia + Marasmus type malnutrion

2. CHF ec. Acyanotic CHD ec. ASD + Bronkhopneumonia + Marasmus type malnutrion

3. CHF ec.Acyanotic CHD ec. PDA + Bronkhopneumonia + Marasmus type malnutrion

Working Diagnosis: CHF ec. Acyanotic CHD ec. VSD + Bronkhopneumonia + Marasmus

type malnutrion

Management:

- O2 1 2 L/i

- IFVD D5% NaCl 0,225% 5 gtt/i micro

- Inj. Ceftriaxone 100 mg/12 h/IV (skin test)

- Inj. Gentamicine 20 mg/24 h/IV (skin test)

- Furosemide 2 x 4 mg

- Spironolactone 2 x 6,25 mg

- NebuleNaCl 0,9% 2,5 cc/8 h

- Diet of Breast milk/supplementary of breast milk 60 cc/3h from NGT

- Chest Fisioteraphy

- Fluid balance/ 6 h

Diagnostic Planning:

Echocardiografi

Picture of FR:


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FOLLOW UP

December 18, 2012

S Shortness of breath (+)


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O

Sensorium : Compos mentis Temp : 37.3oC

BW : 4200 g BL : 60 cm

Head : Eye: light reflex (+/+), isochoric pupil, 3 mm, pale inferior

conjunctiva palpebra (-/-) Old man face (+), Ear and mouth : within

normal limit, Nose: Nostrils breathing (+), NGT (+).

Neck : lymph node enlargement (-)

Thorax : symmetric fusiform, retraction (+) suprasternal,intercostals,

epigastrial, bulging (+), HR: 144 bpm, regular,

pansystolicmurmur(+), RR: 60 bpm, regular, ronchi (+/+),

wheezing (+/+)

Abdomen : Soepel, Peristaltic (+) N, Liver/Spleen : not palpable

Extremity : Pulse : 144 bpm, regular, p/v adequate, acral warm, CRT < 3

BP : 100/60 mmHg

ACHF ec. Acyanotic CHD ec 1. VSD 2. ASD 3. PDA + Bronkhopneumonia +

Marasmus type malnutrion

P

- O2 1 2 L/I


- Inj. Ceftriaxone 100 mg/12 h/IV (skin test)aff.

- Inj. Gentamicine 20 mg/24 h/IV



- Nebule NaCl 0,9% 2,5 cc/8 h

- Diet of Breast Milk/supplementary of breast milk 60 cc/3h from NGT



Planning : Echocardiografi

Balance 06.00

December 19, 2012


Input : 80cc Output : 100 cc Balance : -20cc


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O


BW : 4200 g BL : 60 cm

Head : Eye: light reflex (+/+), isochoric pupil, 3 mm, pale inferior

conjunctiva palpebra (-/-) Old man face (+), Ear and mouth : within

normal limit, Nose: Nostril breathing (+), NGT (+).


Thorax : symmetric fusiform, retraction (+) suprasternal, intercostals,



wheezing (+/+)



BP : 100/60 mmHg

A CHF ec. Acyanotic CHD ec 1. VSD 2. ASD 3. PDA + Bronkhopneumonia +Marasmus type malnutrion

P

- O2 1 2 L/I



- Inj. Ampicillin 100 mg/6 h/IV







Planning : Echocardiograf

Balance 06.00

December 20, 2012

Input : 450 cc Output : 192 cc Balance : 258 cc


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O


BW : 4200 g BL : 60 cm

Head : Eye reflex : +/+; pupil isochor; pale inferior conjunctiva palpebra (-/-).

Old man face (+), Ear/Nose/Mouth: within normal limit, O2 nasal kanul(+), NGT (+)


Thorax : symmetric fusiform, retraction (+) suprasternal,intercostals,



wheezing (+/+)



BP : 100/60 mmHg

ACHF ec. Acyanotic CHD ec 1. VSD 2. ASD 3. PDA + Bronkhopneumonia +

Marasmus type malnutrion

P

- O2 1 2 L/I







- Diet of Breast milk/supplementary of breast milk 60 cc/3h from NGT- Chest Fisioteraphy


Echocardiografi: Mod- Large PMO VSD 5,7 mm

Balance 06.00

December 21, 2012

S Shortness of breath (+) cough (+)

Input : 200cc Output : 142 cc Balance : 58 cc


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O

Sensorium : Compos mentis Temp : 37oC

BW : 4300 g BL : 60 cm


Old man face (+), Ear/Nose/Mouth: within normal limit, O2 nasal kanul

(+), NGT (+)Neck : lymph node enlargement (-)


epigastrial, bulging (+), HR: 142 bpm, regular, pansystolic

murmur(+), RR: 46 bpm, regular, ronchi (+/+), wheezing (+/+)



BP : 100/60 mmHg

ACHF ec Mod-Large PMO VSD + Bronkhopneumonia + Marasmus type

malnutrion

P

- O2 1 2 L/I






- NebuleNaCl 0,9% 2,5 cc + ventoline /8 h



Planning : Catheterization

Balance 06.00

December 22, 2012

S Shortness of breath (+) cough (+)

Input : 100cc Output : 522 cc Balance : -422 cc


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O

Sensorium : Compos mentis Temp : 37,3oC

BW : 4300 g BL : 60 cm


Old man face (+), Ear/Nose/Mouth: within normal limit, O2 nasal kanul




murmur(+), RR: 56 bpm, regular, ronchi (+/+), wheezing (+/+)



BP : 100/60 mmHg

ACHF ec Mod-Large PMO VSD + Bronkhopneumonia + Marasmus type

malnutrion

P

- O2 1 2 L/I






- Folat Acid 1x 5 mg

- Cotrimoxazole 2 x 120 mg

- Becefort syrup 1 cthI

- Vitamin A 1 x 50.000 IU- NebuleNaCl 0,9% 2,5 cc/8 h

- Diet F75 50 cc/ 3 h + 1,2 cc mineral mixed


- Planning : Catheterization

December 23, 2012

S Shortness of breath


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O


BW : 4300 g BL : 60 cm


Old man face (-), Ear/Nose/Mouth: within normal limit, O2 nasal kanul


Thorax : symmetric fusiform, retraction (+) epigastrial, bulging (+), HR: 128

bpm, regular, pansystolic murmur(+) grade 3/6, RR: 32bpm,

regular, ronchi (-/-), wheezing (-/-)



A CHF ec Mod-Large PMO VSD

P

- O2 1 2 L/I

- IFVD D5% NaCl 0,225% 4 gtt/i micro- Inj. Gentamicine 20 mg/24 h/IV

- Inj. Ampicillin 100 mg/6h/IV






- Diet F100 modification with LLM 104 g mixed in 500 cc water 65 cc/3

h + 1,3 cc mineral mix



December 24, 2012

S Shortness of breath


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O


BW : 4300 g BL : 60 cm

Head : Eye reflex : +/+; pupil isochor; pale inferior conjunctiva palpebra(-/-).

Old man face (-), Ear/Nose/Mouth: within normal limit, NGT (+)

Neck : lymph node enlargement (-)Thorax : symmetric fusiform, retraction (-) suprasternal, intercostals,


murmur(+) grade 3/6, RR: 40 bpm, regular, ronchi (-/-), wheezing

(-/-)




P


- Inj. Gentamicine 20 mg/24 h /IV- Inj. Ampicillin 100 mg/6 h/IV






Diet F100 modification with LLM 104 g mixed in 500 cc water 65 cc/3




December 25, 2012

S Shortness of breath (-)


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O

Sensorium : Compos mentis Temp : 37oC

BW : 4400 g BL : 60 cm


Old man face (-), Ear/Nose/Mouth: within normal limit, NGT (+)

Neck : lymph node enlargement (-)Thorax : symmetric fusiform, retraction (-) suprasternal, intercostals,


murmur(+) grade 3/6, RR: 30 bpm, regular, ronchi (-/-), wheezing

(-/-)




P


- Inj. Gentamicine 20 mg/24 h/IV- Inj. Ampicillin 100 mg/6h/IV






Diet F100 modification with LLM 104 g mixed in 500 cc water 65 cc/3




December 26, 2012

S Shortness of breath (-)


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O


BW : 4200 g BL : 60 cm


Old man face (+), Ear/Nose/Mouth: within normal limit, NGT (+)

Neck : lymph node enlargement (-)Thorax : symmetric fusiform, retraction (-) suprasternal,intercostals,


pansystolicmurmur(+) grade 3/6, RR: 28 bpm, regular, ronchi

(-/-), wheezing (-/-)




P







- Diet F100 modification with LLM 104 g mixed in 500 cc water 65 cc/3 h

+ 1,3 cc mineral mix

Planning : - Catheterization (waiting for the schedule, there is no injector)

- Home medical treatment

Chapter 4

DISCUSSION AND SUMMARY

4.1. Discussion


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FR, 4 month-year old girl, weight 4200 gram, height 60 cm, was admitted to Pediatric

Department at Haji Adam Malik General Hospital on December17st 2012 with the main

complaint of shortness in breath and was diagnosed with Congstive Heart Failure. The

diagnose was established based on history taking and clinical manifestations where shortnessof breath experienced since thebirth, she has drinking problems since birth, sweating and

crying when feeding. The initial treatment given were, O2, IVFD D5% NaCl 0,225% 5 gtt/i

micro, Inj. Ceftriaxone 100 mg/12 h/IV, Inj. Gentamicine 20 mg/24 h/IV Furosemide,

Spironolactone, NebuleNaCl 0,9% 2,5 cc/8 h, Diet of ASI/PASI 60 cc/3h, Chest Fisioteraphy,

Fluid balance/ 6 h.


congestion as result of the inability of heart to pump as much blood as required for the a

dequate metabolism of the body. In developing countries, structural heart defects are the most

common cause of heart failure in infants and children. The incidence of congenital heart

disease in children is approximately 8 per 1000 live births, or 0.8%. The most common form

of congenital heart disease in childhood is the VSD, occurring in 50% of all children with

congenital heart disease and in 20% as an isolated lesion.

In infants, the symptoms of heart failure most commonly exhibited include tachypnea,

tachycardia, poorfeeding, and failure to thrive.The grades of HF in children were shown inthe Ross classification (tabel 2.3.) In this patient, tachypnea, poor feeding, failure to thrive

and retraction found are classified as grade IV in Ross Classification. If patient has VSD, it

can be detected by auscultation. The murmurs are typically described as holosystolic or

pansystolic. This patient has pansystolic murmur graded 3/6.

The chest radiograph of HF may demonstrate cardiac enlargement, increased pulmonary

blood flow, venous congestion, or pulmonary edema. However, chest radiographs generally

have a high specificity but low sensitivity for detecting cardiac enlargement. Although it is

not useful for the evaluation of HF, which is a clinical diagnosis, echocardiography is

essential for identifying causes of HF such as structural heart disease, ventricular dysfunction

(both systolic and diastolic), chamber dimensions, and effusions (both pericardial and

pleural). Chest x-ray of this patient demonstrate cardiac enlargement (CTR 54%). Result of

the echocardiography is Mod- Large PMO VSD 5,7 mm, therefore the patient is diagnosed

with CHF ec mod-large PMO VSD 5,7 mm.

The first goal of HF care is to treat the specific cause. HF-causing cardiac malformationsusually can be approached surgically. Medical management (Table 2.4.) aims to


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maximizecardiac output and tissue perfusion while minimizing stresses that increase MVO2.

These goals are accomplished by reducing the amount of force the heart needed to generate to

eject blood (reducing afterload stress) and by reducing the overfilling of heart (preload). This

patient was given Furosemide 2 x 4 mg and Spironolactone 2 x 6,25 mg as diuretic to reducethe preload. This patient was planned to get catheterization and surgery.

4.2. Summary

FR, 4-month-year old girl, weight 4200 gram, height 60 cm, was admitted to Pediatric

Department at Haji Adam Malik General Hospital on December17st 2012 with the main

complaint of shortness of breath and was diagnosed with Congstive Heart Failure ec mod-

large PMO VSD. The diagnose was established based on history taking and clinical

manifestations where shortness of breath has been experienced since thebirth, shehas drinking

problems since birth, sweating and crying when feeding. From the physical diagnostic

tachypnea, failure to thrive and retraction are found and by Ross classification, it is classified

in grade IV. From auscultation, this patient has pansystolic murmur grade 3/6. Chest x-ray of

this patient demonstrates cardiac enlargement (CTR 54%). Result of the echocardiography is

Mod- Large PMO VSD 5,7 mm. The initial treatment given were, O2 1 2 L/I, IVFD D5%

NaCl 0,225% 5 gtt/i micro, Inj. Ceftriaxone 100 mg/12 h/IV (skin test), Inj. Gentamicine 20

mg/24 h/IV (skin test), Furosemide 2 x 4 mg, Spironolactone 2 x 6,25 mg, NebuleNaCl 0,9%

2,5 cc/8 h, Diet of ASI/PASI 60 cc/3h from NGT, Chest Fisioteraphy, Fluid balance/ 6 h.

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3. Fyler DC. Trends. In: Fyler DC, editor. Nadas pediatric cardiology. Philadelphia: Hanley

& Belfus; 1992. p. 273-80.4. Ferencz C, Rubin JD, McCarter RJ, et al. Congenital heart disease: prevalence at

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17. Corone P, Doyon F, Gaudeau S, et al. Natural history of ventricular septal defect. A

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