1

Case Report :
CONGESTIVE HEART FAILURE
Presenter
Supervisor

: Thirukumaran Thiagarajan

(100100274)

: Kanagavalli Vijayakumar

(100100403)

: dr. Lily Irsa, Sp. A (K)

INTRODUCTION
Congenital heart disease occurs in 0.50.8% of live births. The incidence is
higher in stillborns (34%), abortuses (1025%), and premature infants (about 2%
excluding patent ductus arteriosus [PDA]). Congenital cardiac defects have a wide
spectrum of severity in infants: about 23 in 1,000 newborn infants will be
symptomatic with heart disease in the 1st year of life. The diagnosis is established by
1 week of age in 4050% of patients with congenital heart disease and by 1 month of
age in 5060% of patients.1
The initial evaluation for suspected congenital heart disease involves a
systematic approach with three major components. First, congenital cardiac defects
can be divided into two major groups based on the presence or absence of cyanosis,
which can be determined by physical examination aided by pulse oximetry. Second,
these two groups can be further subdivided according to whether the chest radiograph
shows evidence of increased, normal, or decreased pulmonary vascular markings.
Finally, the electrocardiogram can be used to determine whether right, left, or
biventricular hypertrophy exists. The character of the heart sounds and the presence
and character of any murmurs further narrow the differential diagnosis. The final
diagnosis is then confirmed by echocardiography or cardiac catheterization, or by
both.2

Congestive heart failure is the clinical condition in which the heart fails to meet
the circulatory and metabolic needs of the body. Almost all infants who will develop
congestive heart failure from congenital heart disease do so by age 6 months. 3 The
clinical syndrome of congestive heart failure as seen in infants and children
represents the inability of the heart and circulation to meet the metabolic demands of
the

body

despite

various

compensatory

hemodynamic

and

neurohumoral

mechanisms. This clinical picture may emerge when the myocardium is subjected to
excessive loading conditions (volume and/or pressure), primary alterations in
contractile

function,

marked

changes

in

chronotropic

state

(tachy-

or

bradydysrhythmias), or various combinations of these factors. As a result, signs

develop of pulmonary and systemic venous congestion, impaired systemic perfusion,
and findings that indicate such adaptive mechanisms as changes in heart rate,
vasoconstrictor tone, renal function, and ventricular hypertrophy. Over time, these
changes may become maladaptive.4

DEFINITION
Congestive heart failure (CHF) is a clinical syndrome in which the heart is
unable to pump enough blood to the body to meet its needs, to dispose of venous
return adequately, or a combination of the two. 5
ETIOLOGY5
Common causes of Congestive heart failure (CHF) are volume or pressure
overload, or both, caused by congenital heart disease, acquired heart disease and
myocardial diseases. Tachyarrhythmias and heart block can also cause heart failure at
any age. By far the most common causes of CHF in infancy are congenital heart
diseases (CHDs). Beyond infancy, myocardial dysfunctions of various etiologies are
important causes of CHF. Among the rare causes of CHF are metabolic and endocrine

disorders, anemia, pulmonary diseases, collagen vascular diseases, systemic or

pulmonary hypertension, neuromuscular disorders, and drugs such as anthracyclines.
a) Congenital Heart Disease (CHD)
Volume overload lesions such as ventricular septal defect (VSD), patent ductus
arteriosus (PDA), and endocardial cushion defect (ECD) are the most common causes
of CHF in the first 6 months of life. In infancy, the time of the onset of CHF varies
predictably with the type of defect. Table 1 list commons defects according to the age
at which CHF develops. When looking at the table, the following should also be
noted.
1. Children with tetralogy of Fallot (TOF) do not develop CHF unless they
have received a large aortatopulmonary artery (PA) shunt procedure, for
example, too large a Gore-Tex interposition shunt (modified BlalockTaussig shunt).
2. Atrial septal defect (ASD) rarely causes CHF in the pediatric age group,
although it causes CHF in adulthood.
3. Large left-to-right shunt lesions, such as VSD and PDA, do not cause CHF
before 6 to 8 weeks of age because the pulmonary vascular resistance does
not fall low enough to cause a large left-to-right shunt until this age. The
onset of CHF resulting from these left-to-right shunt lesions may be earlier
in premature infants (within the first month) because of an earlier fall in the
pulmonary vascular resistance in these infants.
Table 1: Causes of Congestive Heart Failure Resulting from Congenital Heart
Disease
Age of Onset

Cause

At birth

HLHS
Volume overload lesions:
Severe tricuspid or pulmonary insufficiency

Age of Onset

Cause
Large systemic arteriovenous fistula

First week

TGA
PDA in small premature infants
HLHS (with more favorable anatomy)
TAPVR, particularly those with pulmonary venous obstruction
Others:
Systemic arteriovenous fistula
Critical AS or PS

1st 4th week

COA with associated anomalies

Critical AS
Large left-to-right shunt lesions (VSD, PDA) in premature
infants
All other lesions previously listed

th

th

4 6 week
th

th

6 week4 month

Some left-to-right shunt lesions such as ECD

Large VSD
Large PDA
Others such as anomalous left coronary artery from the PA

AS, aortic stenosis; COA, coarctation of the aorta; ECD, endocardial cushion defect; HLHS,
hypoplastic left heart syndrome; PA, pulmonary artery; PDA, patent ductus arteriosus; PS, pulmonary
stenosis; TAPVR, total anomalous pulmonary venous return; TGA, transposition of the great arteries;
VSD, ventricular septal defect.

b) Acquired Heart Disease

Acquired heart disease of various causes can lead to CHF. With acquired heart
disease, the age at onset of CHF is not as predictable as with CHD, but the following
generalities apply:
1. Endocardial fibroelastosis, a rare primary myocardial disease, causes CHF in
infancy; 90% of cases occur in the first 8 months of life.

2. Viral myocarditis tends to be more common in small children older than 1

year. It occurs occasionally in the newborn period, with a fulminating clinical
course with poor prognosis.
3. Myocarditis associated with Kawasaki disease is seen in children 1 to 4 years
of age.
4. Acute rheumatic carditis is an occasional cause of CHF that occurs primarily
in school-age children.
5. Rheumatic valvular heart diseases, usually volume overload lesions such as
mitral regurgitation (MR) or aortic regurgitation (AR), cause CHF in older
children and adults. These diseases are uncommon in industrialized countries.
6. Dilated cardiomyopathy may cause CHF at any age during childhood and
adolescence. The cause of the majority of dilated cardiomyopathy is
idiopathic, but it may be caused by infectious, endocrine, or metabolic
disorders or autoimmune diseases or may follow antineoplastic treatment
(e.g., anthracycline).
7.Doxorubicin cardiomyopathy may manifest months to years after the
completion of chemotherapy for malignancies in children.
8.Cardiomyopathies associated with muscular dystrophy and Friedreich's ataxia
may cause CHF in older children and adolescents.
9.Patients who received surgery for some types of CHD (such as a Fontan
operation, surgery for TOF, transposition of the great arteries, and other
cyanotic defects) may remain in or develop CHF.
c) Miscellaneous Causes Miscellaneous causes of CHF include the following:
1.Metabolic abnormalities (severe hypoxia and acidosis, as well as
hypoglycemia and hypocalcemia) can cause CHF in newborns.
2. Endocrinopathy such as hyperthyroidism can cause CHF.
3. Supraventricular tachycardia (SVT) causes CHF in early infancy.

4. Complete heart block associated with structural heart defects causes CHF in
the newborn period or early infancy.
5. Severe anemia may be a cause of CHF at any age. Hydrops fetalis may be a
cause of CHF in the newborn period and severe sicklemia at a later age.
6.Bronchopulmonary

dysplasia

seen

in

premature

infants

causes

predominantly right-sided heart failure in the first few months of life.

7. Primary carnitine deficiency (plasma membrane carnitine transport defect)
causes progressive cardiomyopathy with or without skeletal muscle
weakness that begins at 2 to 4 years of age.
8. Acute cor pulmonale caused by acute airway obstruction (such as seen with
large tonsils) can cause CHF at any age but most commonly during early
childhood.
9.

Acute

systemic

hypertension,

as

seen

in

acute

postinfectious

glomerulonephritis, causes CHF in school-age children. Fluid retention with

poor renal function is important as the cause of hypertension in this
condition.
PATHOPHYSIOLOGY4
According to the Frank-Starling law, as the ventricular end-diastolic volume (or
preload) increases, the healthy heart increases cardiac output until a maximum is
reached and cardiac output can no longer be augmented (see Figure. 1 ). When the
left ventricular (LV) end-diastolic pressure reaches a certain point, pulmonary
congestion develops with pulmonary congestive symptoms (tachypnea and dyspnea).
Congestive symptoms occur even with normally functioning myocardium if the enddiastolic pressure is greatly increased, such as with infusion of a large amount of fluid
or blood. An increase in the stroke volume is also achieved in the failing heart when
the preload is increased, but the failing heart does not achieve the same level of
maximal cardiac output as the normal heart, and congestive symptoms (dyspnea and
hepatomegaly results).

Figure 1. Effects of anticongestive medications on the Frank-Starling relationship for ventricular

function. In persons with a normal heart, cardiac output increases as a function of ventricular filling
pressure (upper curve). In patients with heart failure, the normal relationship between cardiac output
(or stroke volume) and filling pressure (preload) is shifted lower and to the right such that a low-output
state and congestive symptoms may coincide. Congestive symptoms (dyspnea, tachypnea) may appear
even in a normal heart if the filling pressure reaches a certain point. At one extreme, the addition of a
pure inotropic agent, such as digoxin, primarily increases the stroke volume with minimal impact on
filling pressure (so that the patient may still have congestive symptoms). Conversely, the addition of a
diuretic primarily decreases the filling pressure (with improved congestive symptoms) but without
improving cardiac output. Clinically, it is common to use multiple classes of agents (usually a
combination of inotropic agents, diuretics, and vasodilators) to produce both increased cardiac output
and decreased filling pressure.

The increased stroke volume results in increased wall tension, which in turn
increases oxygen consumption. Increase in the wall tension is also seen in dilated
ventricular cavity, according to the Laplace law.
Wall stress = pressure ; radius/2 wall thickness
The Laplace law, although an oversimplification, emphasizes two points:
1
.

The bigger the left ventricle and the greater the radius, the greater the

wall stress.
2

At any given radius (LV size), the greater the pressure developed in the

LV, the greater the wall stress.

In heart failure, cardiac hypertrophy (with increased wall thickness) develops to

balance the increased pressure and keep the wall stress unchanged, and reduction of
heart size decreases wall stress and improves cardiac output or LV function.
Among many compensatory responses to the failing heart is the activation of
two important neurohormonal mechanisms: the sympathetic nervous system and the
renin-angiotensin-aldosterone system. Although these responses are an attempt to
preserve cardiovascular homeostasis and thus are beneficial initially, chronic
stimulation of these systems may be deleterious in the natural history of myocardial
dysfunction.
1. One major compensatory mechanism for increasing cardiac output is an
increase in sympathetic tone, secondary to increased adrenal secretion of
circulating epinephrine and increased neural release of norepinephrine. The
initial beneficial effects of adrenergic stimulation include increased heart
rate and myocardial contractility with resulting increase in cardiac output.
However, chronic adrenergic stimulation eventually leads to adverse

myocardial effects, including increased afterload, hypermetabolism,

arrhythmogenesis, and direct myocardial toxicity.
a) Catecholamines are toxic to cardiac muscle, perhaps by producing
calcium overload or by inhibiting the synthesis of contractile proteins.
b) High catecholamine levels decrease the density of -adrenergic
receptors on the surface of the myocardial cell, which may be the
major cause of functional loss of the catecholamine-mediated positive
inotropic response.
In clinical settings, the reduction of adrenergic stimulation by the use
of -adrenergic blockers has resulted in clinical improvement in
patients with dilated cardiomyopathy, in whom increased levels of
catecholamines have been shown to be present.
2. The reduced blood flow to the kidneys in patients with CHF causes a marked
increase in renin output, and this in turn causes the formation of angiotensin
II. Angiotensin II leads to further increase in reabsorption of both water and
salt from the renal tubules. Angiotensin II may cause a trophic response in
vascular

smooth

muscle

(with

vasoconstriction)

and

myocardial

hypertrophy. Angiotensin II also promotes myocardial fibrosis. Thus,

although the hypertrophic response is adaptive by attempting to restore wall
stress to normal, angiotensin II plays a maladaptive role in CHF by initiating
fibrosis and altering ventricular compliance.
Thus, reasons for using -adrenergic blockers and angiotensin-converting enzyme
(ACE) inhibitors in the treatment of CHF are to block the maladaptive role of the
adrenergic and renin-angiotensin-aldosterone systems.
DIAGNOSIS
History
1. Poor feeding of recent onset, tachypnea that worsens during feeding, poor
weight gain, and cold sweat on the forehead suggest CHF in infants.

10

2. Older children may complain of shortness of breath, especially with

activities, early fatigability, puffy eyelids, or swollen feet.
Physical Examination
Physical findings of CHF may be classified as follows, depending on their
pathophysiologic mechanisms. The more common findings are in italics.
1 The following are found as compensatory responses to impaired cardiac
function:
a. Tachycardia, gallop rhythm, and weak and thready pulses are common.
b.Cardiomegaly is almost always present. Chest x-ray films are more reliable
than physical examination in demonstrating cardiomegaly.
c.There are signs of increased sympathetic discharges (e.g., growth failure,
perspiration, cold and wet skin).
2. Pulmonary venous congestion (from left-sided failure) results in the
following manifestations:
a.

Tachypnea is common and is an early manifestation of CHF in infants.

b. Dyspnea on exertion (equivalent to poor feeding in small infants) is

common in children.
c.

Orthopnea may be seen in older children.

d.

Wheezing and pulmonary crackles are occasionally audible.

3. Systemic venous congestion (related to right-sided failure) results in the

following:
a.

Hepatomegaly is common but it is not always indicative of CHF. A large

liver may be palpable in conditions that cause hyperinflated lungs (asthma,
bronchiolitis, during hypoxic spells) and in infiltrative liver disease.

11

Conversely, the absence of hepatomegaly does not rule out CHF;

hepatomegaly may be absent in (early) left-sided failure.
b.

Puffy eyelids are common in infants.

c. Distended neck veins and ankle edema, which are common in adults, are
not seen in infants.
d.

Splenomegaly is not indicative of CHF; it usually indicates infection.

X-Ray Studies
The presence of cardiomegaly should be demonstrated by chest x-ray films. The
absence of cardiomegaly almost rules out the diagnosis of CHF. The only exception
to this rule arises when the pulmonary venous return is obstructed; in such cases, the
lung parenchyma shows pulmonary edema or venous congestions.
Electrocardiography
ECGs help determine the type of heart defect causing heart failure but are not
helpful in deciding whether CHF is present.
Echocardiography
Echo studies may confirm enlargement of ventricular chambers and impaired
LV systolic function (decreased fractional shortening or ejection fraction) as well as
impaired diastolic function by the use of Doppler techniques. A more important role
of echo may be due to its ability to determine the cause of CHF. Echo is also helpful
in serial evaluation of the efficacy of therapy.
Cardiac Catheterization
Endomyocardial biopsy obtained during cardiac catheterization offers a new
approach to specific diagnosis of the cause of CHF, such as inflammatory disease,
infectious process, or metabolic disorder. When viral myocarditis is suspected, the

12

polymerase chain reaction provides a means of isolating the offending viral agent
from biopsy specimens. In a patient with dilated cardiomyopathy, evaluation of
biopsy specimens, including genetic analysis, may provide data permitting the
diagnosis of specific metabolic causes, such as carnitine deficiency.
TREATMENT4
The treatment of CHF consists of (1) elimination of the underlying causes, (2)
treatment of the precipitating or contributing causes (e.g., infection, anemia,
arrhythmias, fever), and (3) control of the heart failure state. Eliminating the
underlying causes is the most desirable approach whenever possible. Surgical
correction of congenital heart defects is such an approach. Every patient with CHF
should receive maximal medical treatment, but continuing with long-term
anticongestive measures is unwise when the heart defect can be safely repaired
through surgery. The heart failure state is controlled by the use of multiple drugs,
including inotropic agents, diuretics, and afterload-reducing agents, along with
general supportive measures.
TREATMENT OF UNDERLYING CAUSES OR CONTRIBUTING FACTORS
1. When surgically feasible, treatment of underlying congenital heart defects
and valvular heart disease is the best approach for complete cure.
2. If hypertension is the underlying cause of CHF, antihypertensive treatment
should be given.
3. If arrhythmias or advanced heart block is the cause of or a factor
contributing to heart failure, antiarrhythmic agents or cardiac pacemaker
therapy is indicated.
4. If hyperthyroidism is the cause of heart failure, this condition should be
treated.
5. Fever should be controlled with antipyretics.
6. When there is a concomitant infection, it should be treated with appropriate
antibiotics.

13

7. For anemia, packed cell transfusion is given to raise the hematocrit to 35%
or higher.
GENERAL MEASURES
1. A cardiac chair or infant seat is used to keep infants in a semiupright
position to relieve respiratory distress.
2. Oxygen (40% to 50%) with humidity is administered to infants with
respiratory distress if pulse oximetry indicates compromise of blood
oxygenation.
3. Adequate calories and fluid should be provided to permit appropriate
weight gain. Infants in CHF need significantly higher caloric intakes than
recommended for average children. The required caloric intake may be as
high as 150 to 160 kcal/kg/day for infants in CHF. Compounding this problem
is that these infants typically cannot take in needed calories even for normal
growth owing to tachypnea, increased work of breathing, diminished strength
of sucking, and difficulty with coordination of sucking and swallowing.
a. Increasing caloric density of feeding may be required, and it may be
accomplished with fortification of feeding.
b. Frequent small feedings are better tolerated than large feedings in
infants.
c. If oral feedings are not well tolerated, intermittent or continuous
nasogastric (NG) feeding is indicated. To promote normal development of
oral-motor function, infants may be allowed to take calorie-dense oral
feeds throughout the day and then be given continuous NG feeds
overnight.
d. Salt restriction in the form of a low-salt formula and severe fluid
restriction are not indicated in infants. Use of diuretics has replaced these
measures.
e. Parents should be taught proper feeding techniques.
4. In older children, salt restriction (<0.5 g/day) and avoidance of salty snacks
(chips, pretzels) and table salt are recommended. Bed rest remains an

14

important component of management. The availability of a television screen

and computer games for entertainment ensures bed rest in older children.
5. If respiratory failure accompanies cardiac failure, intubation and positivepressure ventilation are occasionally required. Respiratory failure usually
signifies that surgical intervention will be needed for CHDs when the patient
is stabilized.
6. Daily weight measurement is essential in hospitalized patients.
General support to improve congestive symptoms and nutritional support are
important.
DRUG THERAPY
Three major classes of drugs are commonly used in the treatment of CHF in
children: diuretics, inotropic agents, and afterload-reducing agents. Diuretics are
usually used with inotropic agents. Rapid-acting inotropic agents (dopamine,
dobutamine) are used in critically or acutely ill infants and children. Afterloadreducing agents, such as ACE inhibitors, have gained popularity because they can
increase cardiac output without increasing myocardial oxygen consumption.
Recently, low-dose -adrenergic blockade has been added to the treatment of dilated
cardiomyopathy with encouraging results.

Diuretics.
Diuretics remain the principal therapeutic agent to control pulmonary and

systemic venous congestion. Diuretics reduce preload and improve congestive

symptoms but do not improve cardiac output or myocardial contractility ( Fig. 1).
Patients with mild CHF may improve rapidly after a dose of fast-acting diuretics,
such as ethacrynic acid or furosemide, even before digitalization. Table 3 shows

15

dosages of commonly available diuretic preparations. There are three main classes of
diuretics that are commercially available.
1.

Thiazide diuretics (e.g., chlorothiazide, hydrochlorothiazide), which act at

the proximal and distal tubules, are no longer popular.

2.

Rapid-acting diuretics, such as furosemide and ethacrynic acid, are the drugs
of choice. They act primarily at the loop of Henle (loop diuretics).

3.

Aldosterone antagonists (such as spironolactone) act on the distal tubule to

inhibit sodium-potassium exchange. The serum aldosterone level is
significantly increased in patients with persistent CHF, contributing to fluid
and salt retention. Patients with an increased level of circulating aldosterone
have a diminished response to diuretic agents because aldosterone increases
tubular reabsorption of sodium and water at a site distal to the sites of action
of other diuretic agents (thiazides or furosemide). These drugs have value in
preventing hypokalemia produced by other diuretics and thus are used in
conjunction with a loop diuretic. However, when ACE inhibitors are used,
spironolactone should be discontinued to avoid hyperkalemia.
Table 3 -- Diuretic Agents and Dosages

Preparation

Route Dosage

Thiazide Diuretics
Chlorothiazide (Diuril)

Oral

Hydrochlorothiazide (HydroDIURIL) Oral

2040 mg/kg/day in 23 divided doses

24 mg/kg/day in 23 divided doses

Loop Diuretics
Furosemide (Lasix)
Ethacrynic acid (Edecrin)

IV

1 mg/kg/dose

Oral

23 mg/kg/day in 23 divided doses

IV

1 mg/kg/dose

Oral

23 mg/kg/day in 23 divided doses

Oral

13 mg/kg/day in 23 divided doses

Aldosterone Antagonist
Spironolactone (Aldactone)

16

Rapidly Acting Inotropic Agents.

In critically ill infants with CHF, in those with renal dysfunction (such as infants with
coarctation of the aorta), or in postoperative cardiac patients with heart failure,
rapidly acting catecholamines with a short duration of action are preferable to
digoxin. This class of agents includes dopamine, dobutamine, isoproterenol, and
epinephrine. These agents possess inotropic and vasodilator actions and thus are
useful in acute situations. Inotropic agents increase the contractile property of the
myocardium toward the normal curve (see Fig. 1 ). There is an added beneficial effect
when an inotropic agent has vasodilating action as in dopamine. Dobutamine has less
chronotropic effects than dopamine. Dopamine in high doses causes -receptor
stimulation with vasoconstriction and reduction of renal blood flow. Dosages for
intravenous drip of these catecholamines are suggested in Table 4 .

Table 4 -- Suggested Starting Dosages of Catecholamines

Dosage and
Drug

Route

Side Effects

Epinephrine

0.11

Hypertension, arrhythmias

(Adrenalin)

g/kg/min IV

Isoproterenol

0.10.5

(Isuprel)

g/kg/min IV

Dobutamine

28 g/kg/min

Less tachycardia than with dopamine,

(Dobutrex)

IV

vasodilatation, arrhythmias

Dopamine

510 g/kg/min

Tachycardia, arrhythmias, hypertension

(Intropin)

IV

or hypotension

Peripheral and pulmonary vasodilatation

Dose-related cardiovascular effects

17

Dosage and
Drug

Route

Side Effects
(g/kg/min):
Renal vasodilatation: 25
Inotropic: 58
Tachycardia: >8
Mild vasoconstriction: >10
Vasoconstriction: 1520

Digitalis Glycosides
Digoxin is the most commonly used digitalis preparation in pediatric patients.
Inotropic agents increase the cardiac output (or contractile state of the myocardium),
resulting in an upward and leftward shift of the ventricular function curve relating
cardiac output to filling volume of pressure (see Fig. 1 ). When inotropic agents are
used with a vasodilator or a diuretic, a much greater improvement is seen both in the
contractile state and in congestive symptoms than when a single class of agent is used

Table 5 -- Oral Digoxin Dosage for Congestive Heart Failure

Age

Total Digitalizing Dose (g/kg)

Maintenance Dose[*] (g/kg/day)

Prematures 20

Newborns

30

<2 yr

4050

1012

>2 yr

3040

810

The maintenance dose is 25% of the total digitalizing dose in two divided doses. The IV dose is
75% of the oral dose.

18

The pediatric dosage of digoxin is much larger than the adult dosage on the
basis of body weight. Pharmacokinetic studies indicate that infants and children
require a larger dose of digoxin than adults to attain comparable serum levels,
primarily because of a larger volume of distribution and, less important, more rapid
renal clearance, including tubular secretion. The volume of distribution of digoxin is
7.5 L/kg in neonates, 16 L/kg in infants and children, and 4 L/kg in adults.

Afterload-Reducing Agents
Vasoconstriction that occurs as a compensatory response to reduced cardiac

output seen in CHF may be deleterious to the failing ventricle. Vasoconstriction is

produced by a rise in sympathetic tone and circulating catecholamines and an
increase in the activity of the renin-angiotensin system. Reducing afterload tends to
augment the stroke volume without a great change in the contractile state of the heart
and therefore without increasing myocardial oxygen consumption (see Fig. 1). When
a vasodilator is used with an inotropic agent, the degree of improvement in the
inotropic state as well as in congestive symptoms is much greater than when a
vasodilator alone is used. Combined use of an inotropic agent, a vasodilator, and a
diuretic produces most improvement in both inotropic state and congestive symptoms
(see Fig. 1).
Afterload-reducing agents now occupy a prominent role in the treatment of
infants with CHF secondary to large left-to-right shunt lesions (e.g., VSD, AV canal,
PDA). Infants with large left-to-right shunts have been shown to benefit from
captopril and hydralazine. Beneficial effects of afterload-reducing agents are also
seen in dilated cardio-myopathy, doxorubicin(Adriamycin)-induced cardiomyopathy,
myocardial ischemia, postoperative cardiac status, severe MR or AR, and systemic

19

hypertension. These agents are usually used in conjunction with digitalis glycosides
and diuretics for a maximal benefit.
Afterload-reducing agents may be divided into three groups based on the site of
action: arteriolar vasodilators, venodilators, and mixed vasodilators. Dosages of these
agents are presented in Table 6 .
1.

Arteriolar vasodilators (hydralazine) augment cardiac output by acting

primarily on the arteriolar bed, with resulting reduction of the afterload.
Hydralazine is often administered with propranolol because it activates
the baroreceptor reflex, with resulting tachycardia.

2.

Venodilators (nitroglycerin, isosorbide dinitrate) act primarily by dilating

systemic veins and redistributing blood from the pulmonary to the
systemic circuit (with a resulting decrease in pulmonary symptoms).
Venodilators are most beneficial in patients with pulmonary congestion
but may have adverse effects when preload has been restored to normal
by diuretics or sodium restriction.

3.

Mixed vasodilators include ACE inhibitors (captopril, enalapril),

nitroprusside, and prazosin. These agents act on both arteriolar and
venous beds. ACE inhibitors are popular in children with chronic severe
CHF, whereas sodium nitroprusside is used primarily in acute situations
such as following cardiac surgery under cardiopulmonary bypass,
especially in patients who had pulmonary hypertension and those with
postoperative rises in PA pressure. When nitroprusside is used, blood
pressure must be monitored continuously. ACE inhibitors reduce
systemic vascular resistance by inhibiting angiotensin II generation and
augmenting production of bradykinin.
Table 6 -- Dosages of Vasodilators

20

Drug

Route and Dosage

Comments

Hydralazine

IV: 0.10.2 mg/kg/dose,

May cause tachycardia; may be used

(Apresoline)

every 46 hr (maximum 2 with propranolol

mg/kg every 6 hr)
Oral: 0.753 mg/kg/day,

May cause gastrointestinal symptoms,

in 24 doses (maximum

neutropenia, and lupus-like syndrome

200 mg/day)
Nitroglycerin

IV: 0.52 g/kg/min

Start with small dose and titrate based

(maximum 6 g/kg/min)

on effects

Captopril

Oral: Newborn: 0.10.4

May cause hypotension, dizziness,

(Capoten)

mg/kg/dose, 14 times a

neutropenia, and proteinuria

day
Infant: 0.56 mg/kg/day, Dose should be reduced in patients with
14 times a day

impaired renal function

Child: 12.5 mg/dose, 12

times a day
Enalapril

Oral: 0.1 mg/kg, once or

Patient may develop hypotension,

(Vasotec)

twice daily

dizziness, or syncope

Nitroprusside

IV: 0.58 g/kg/min

May cause thiocyanate or cyanide

(Nipride)

toxicity (e.g., fatigue, nausea,

disorientation), hepatic dysfunction, or
light sensitivity

Prazosin

Oral: first dose, 5 g/kg;

Has fewer side effects than hydralazine;

(Minipress)

increase to 25150

orthostatic hypotension or

g/kg/day in 4 doses

tachyphylaxis may develop

OTHER DRUGS
-Adrenergic Blockers.

21

As with their beneficial effects reported in adult patients with dilated

cardiomyopathy, -adrenergic blockers have been shown to be beneficial in some
pediatric patients with chronic CHF who were symptomatic despite being treated with
standard anticongestive drugs (digoxin, diuretics, ACE inhibitors). Recent evidence
suggests that the adrenergic overstimulation often seen in patients with chronic CHF
may have detrimental effects on the hemodynamics of heart failure by inducing
myocyte injury and necrosis rather than being a compensatory mechanism, as
traditionally thought. -Adrenergic blockers should not be given to those with
decompensated heart failure. They should be deferred until reestablishment of good
fluid balance and stable blood pressure and should be started with a small dose and
gradually increased.

Carvedilol, a nonselective -adrenergic blocker with additional 1antagonist activities, when added to standard medical therapy for CHF, has
been shown to be beneficial in children with dilated cardiomyopathy ( Bruns
et al, 2001 ). The patients included in the study were those with idiopathic
dilated

cardiomyopathy,

chemotherapy-induced

cardiomyopathy,

postmyocarditis myopathy, and muscular dystrophy and those who had

chronic heart failure following surgeries for congenital heart defects (such
as a Fontan or Senning operation). The initial dose was 0.09 mg/kg twice
daily and the dose was increased gradually to 0.36 and 0.75 mg/kg as
tolerated, up to the maximum adult dose of 50 mg/day. Side effects of the
drug include dizziness, hypotension, and headache (also see Dilated
Cardiomyopathy).

Metoprolol was added to standard anticongestive medicines in patients with

chronic CHF from dilated cardiomyopathy. Metoprolol increased LV
fractional shortening and ejection fraction and improved symptoms. The
starting dose was 0.1 to 0.2 mg/kg per dose twice a day and was slowly

22

increased over a period of weeks to 1.1 mg/kg/day (range 0.5 to 2.3

mg/kg/day) ( Shaddy et al, 1999 ). The improvement in the LV fractional
shortening appears slightly better with carvedilol than with metoprolol.
SURGICAL MANAGEMENT
If medical treatment with the previously mentioned regimens does not improve
CHF caused by congenital heart defects within a few weeks to months, one should
consider either palliative or corrective cardiac surgery for the underlying cardiac
defect when technically feasible. Cardiac transplantation is an option for a patient
with progressively deteriorating cardiomyopathy despite maximal medical treatment.

23

CASE REPORT
Name

: Jihan Talita Ulfa Siregar

Age

: 11 months 16 days

Sex

: Female

Date of Admission

: December, 21th 2014

Chief Complaint

: Shortness of Breath

History: These was realized by the Os parents one month ago. Restlessness and
shortness of breath was seen during activities such as when Os is crying and
consuming milk. These complain was encountered by os since birth but it got worst in
this few months. In the past two months, Os has a history of interrupted consumption
of milk and heavy sweating during consumption of milk. Fever (-), diarrhea (-),
cough (+) flam (-), vomiting (-) and sweating (+) in the last one month. Os have
defecation problem (constipation) in the last two weeks. Os has no urination problem.
Pregnant History
Patient was conceived at second pregnancy at the age 28, first child (2 yrs old):
normal delivery and healthy. There was no history of fever, hypertension, diabetic
mellitus, and consumption of drugs and herbal medicine as well jaundice during
pregnancy period.
Birth History
Spontaneous; attended by midwives; BW 3800 gram; BL 50 cm, cyanotic (-)

24

Immunization History
BCG I (no scar), DPT II, Polio III, Measles I, Hepatitis III
Feeding History
From birth to 4 months

: Breast milk only

From 4 months up to date

: Breast milk, biscuit porridge (SUN) and

Formulated Milk
History of Growth and Development
Sitting

- months

Crawling

- months

Standing

- months

Walking

- months

History of previous illness

The patient has been suffering from growth stunt where the growth doesnt match the
age and was experiencing shortness of breath when crying and ingesting milk for
almost more than a month. Patient was then brought by the parents to Tapsel District
General Hospital and was diagnosed as noncynotic CHF. Later the patient was then
referred to Dr. Pringadi District General Hospital where was diagnosed as CHF ec
acynotic CHD. Finally the patient was referred to Haji Adam Malik General Hospital
on the 21th of Dec 2014.
History of previous medications

: none

History of Family Disease

: unclear

Pysical Examination
Generalized status
Body weight: 5kg, Body length: 62 cm

25

Body weight in 50th percentile according to age: 8.5 kg

Body length in 50th percentile according to age: 73 cm
Body weight in 50th percentile according to body length: 6.5 kg
BW/BL: 5/6.5 x 100% = 77 % (Moderate Malnutrition)
BW/age: 5/8.5 x 100% = 59 % (Severe Malnutition)
BL/age : 62/73 x 100% = 85% (Mild malnutrition)
Weight for lenght: -3 < Z scores < -1 (underweight)
Presens status
Consciousness: Compos mentis , Body temperature: 36,7 oC. HR 140x/i, RR 50x/i,
BP 90/70 mmHg,
Anemic (-); Icteric (-); Cyanosis (-); Edema (-). Dyspnea (+).
Localized status
Head :
Large crown closed. Black hair, normal.
Right Eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale (-). Icteric sclera
(-). Light reflex (+).
Left eye: Pupil diameter 3 mm. Inferior palpebra conjunctiva pale (-). Icteric sclera
(-). Light reflex (+).
Ear, nose and mouth were within normal limit.
Neck :
Lymph node enlargement (-). TVJ R+2 cmH20
Thorax:
Symmetrical fusiformis. Chest retraction (+) epigastrial, intercostals, suprasternal.
HR: 140 bpm, regular, murmur (+) pansistolik grade III/6 LMCS ICR III-IV.

26

RR: 50x/i, ireguler, rales -/Abdomen:

Soepel, Normoperistaltic. Liver, spleen and renal unpalpable..
Extremities:
Pulse 136x/i, regular, adequate p/v, cold acral , CRT < 3.
Urogenital:
Female; within normal limit.
Laboratory Findings (December, 21st 2014):
Parameters
Complete Blood Count
Hemoglobin
Hematocrite
Erithrocyte
Leucocyte
Platelet
MCV
MCH
MCHC
RDW
MPV
PCT
PDW
Hitung Jenis
Neutrofil

Value

Normal Value

10.30 gr%
31.60 %
4.06 x 106 /mm3
12.06 x 103 /mm3
385.000 /mm3
77.80 fl
25.40 pg
32.60 gr%
18.80 %
7.80 fl
0.30%
7.2%

12,0 14,4 gr%

37 41%
4,40 4,48 x 106 /mm3
4,5 13.5x 103 /mm3
150.000 450.000 /mm3
81 95 fl
25 29pg
29 31 gr%
11.6 14.8 %
7,0 10,2 fl

24,30

37-80

Limfosit

70,00

20-40

Monosit

5,20

2-8

Eosinofil

0,20

1-6

Basofil

0,300

0-1

Neutrofil absolute

2.92

1,9-5,4

27

Limfosit absolute

8,44

3,7-10,7

Monosit absolute

0.63

0,3-0,8

Eosinofil absolute

0.03

0,2-0,5

Basofil absolute

0,04

0-0,1

Laboratory Findings (December, 21st 2014):

Parameters
Carbohydrate Metabolism
Blood Glucose ad random
Blood Gas Analysis
pH
pCO2
pO2
Bicarbonate (HCO3)
Total CO2
Base Excessive (BE)
O2 Saturation
Cardiac Marker
Troponin T

Radiologic Imaging

Value

Normal Value

91,00 mg/dL

< 200

7,437
21,7 mmHg
137,7 mmHg
14,3 mmol/L
15,0 mmol/L
-8.6 mmol/L
99.1%

7,35 7,45
38 42
85 100
22 26
19 25
(-2) (+2)
95 - 100

Negative (g/L)

0 0,1

28

Chest x-ray interpretation:

KV weak. Less Inspiration, no trachea deviation found(middle trachea), both
costophrenicus angel were sharp, smooth diaphragm, not seen infiltrates in both lung
fields, Egg shaped heart was found. Cardio thoracic Ratio is more than 50 %, apex
embedded (apex upwards), bones and soft tissues in good condition, waist of heart
not prominent.

29

Result : CTR of 65% Cardiomegaly, Heart characteristics: egg on site

Differential Diagnosis:

VSD
ASD
PDA

Working Diagnosis:
CHF d/t acynotic CHD
Management:
-

Bed rest
O2 Nasal kanul L/i
Furosemide 2x5mg
Spironolactone 2x6,25mg
Sildenafil 3x1.5mg
IV Dobutamin 5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in 1cc perhour
Diet F75 125cc/2jam/ggt

Diagnostic Planning:
-

Consul cardiologist

Urinalysis
Echocardiography

FOLLOW UP
December , 21st 2014
S Dyspnoe +
O Sens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-). Edema (-).
Cyanosis (-) Dyspnoe (+)

30

Body weight: 5 kg, Body length: 62 cm

Head

Right Eye: Pupil diameter 3 mm. Inferior palpebra

conjunctiva pale (-). Icteric sclera (-). Light reflex (+).
Left eye: Pupil diameter 3 mm. Inferior palpebra

Neck
Thorax

conjunctiva pale (-). Icteric sclera (-). Light reflex (+)..

Lymph node enlargement (-)
Simetris fusiformis. Retraction (+) epigastrial; intercostals,
suprasternal. HR: 150 bpm, reguler; murmur (+) pansistolik
grade III/6 LMCS III-IV.

Abdomen
Extremitie

A
P

RR: 50 x/i, regular, rales (-/-)

Soepel. normoperistaltic. Liver, spleen and renal unpalpable.
Pulse 140 x/i, iregular, adequate p/v, cold, CRT < 3.

s
Genital
Female; within normal limit.
CHF ec acynotic CHD d/t dd/ VSD
ASD
PDA
Management:
-

Bed rest
O2 Nasal kanul L/i
Furosemide 2x5mg
Spironolactone 2x6,25mg
Sildenafil 3x1.5mg
IV Dobutamin 5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in
1cc perhour
Diet F75 125cc/2jam/ggt

Diagnostic Planning:
-

Echocardiography
Urinalisis
Consul cardiologist

December , 22nd 2014

S Dyspnoe +
O Sens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-). Edema (-).
Cyanosis (-) Dyspnoe (+)

31

Body weight: 5 kg, Body length: 62 cm

Head

Right Eye: Pupil diameter 3 mm. Inferior palpebra

conjunctiva pale (-). Icteric sclera (-). Light reflex (+).
Left eye: Pupil diameter 3 mm. Inferior palpebra

Neck
Thorax

conjunctiva pale (-). Icteric sclera (-). Light reflex (+)..

Lymph node enlargement (-)
Simetris fusiformis. Retraction (+) epigastrial; intercostals,
suprasternal. HR: 150 bpm, reguler; murmur (+) pansistolik
grade III/6 LMCS III-IV.

Abdomen
Extremitie

A
P

RR: 50 x/i, regular, rales (-/-)

Soepel. normoperistaltic. Liver, spleen and renal unpalpable.
Pulse 140 x/i, iregular, adequate p/v, cold, CRT < 3.

s
Genital
Female; within normal limit.
CHF ec acynotic CHD d/t dd/ VSD
ASD
PDA
Management:
-

Bed rest
O2 Nasal kanul L/i
Furosemide 2x5mg
Spironolactone 2x6,25mg
Sildenafil 3x1.5mg
IV Dobutamin 5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in
1cc perhour
Diet F75 125cc/2jam/ggt

Diagnostic Planning:
-

Echocardiography (today)

Echocardiography

32

Result: Large ASD, Small PDA, pulmonary stenosis and decreased systolic function

December , 23rd 2014

S Dyspnoe +
O Sens: Compos mentis, Temp: 36,7 oC. Anemic (-). Icteric (-). Edema (-).
Cyanosis (-) Dyspnoe (+)
Body weight: 5 kg, Body length: 62 cm
Head

Right Eye: Pupil diameter 3 mm. Inferior palpebra

conjunctiva pale (-). Icteric sclera (-). Light reflex (+).
Left eye: Pupil diameter 3 mm. Inferior palpebra

Neck
Thorax

conjunctiva pale (-). Icteric sclera (-). Light reflex (+)..

Lymph node enlargement (-)
Simetris fusiformis. Retraction (+) epigastrial; intercostals,
suprasternal. HR: 180 bpm, reguler; murmur (+) pansistolik
grade III/6 LMCS III-IV.

Abdomen

RR: 70 x/i, regular, rales (-/-)

Soepel. normoperistaltic. Liver, spleen and renal unpalpable.

33

Extremitie

A
P

Pulse 140 x/i, iregular, adequate p/v, cold, CRT < 3.

s
Genital
Female; within normal limit.
CHF ec acynotic CHD d/t ASD
PDA
PS
Management:
-

Bed rest
O2 Nasal kanul L/i
Furosemide 2x5mg
Spironolactone 2x6,25mg
Sildenafil 3x1.5mg
IV Dobutamin 5mcg/kgbw/minute (75mg in 50cc Nacl 0.9%) in
1cc perhour
- Diet F75 125cc/2jam/ggt
24th December 2014
Patient exitus after sudden drop in blood pressure and worsening dyspneo.
Resuscitation failed.

34

CASE DISCUSSION
Theory
Case
Common causes of Congestive heart Patient is 11 months old, with the the
failure (CHF) are volume or pressure diagnosis CHF due to CHD
overload, or both, caused by congenital
heart disease, acquired heart disease and
myocardial diseases. By far the most
common causes of CHF in infancy are
congenital heart diseases (CHDs).
History
1.Poor feeding of recent onset, tachypnea Shortness of Breath was realized by the
that worsens during feeding, poor weight patients

parents

one

month

ago.

gain, and cold sweat on the forehead Restlessness and shortness of breath was
suggest CHF in infants.

seen during activities such as when

2. Older children may complain of patient is crying and consuming milk.

shortness of breath, especially with These complain was encountered by
activities, early fatigability, puffy eyelids, patient since birth but it got worst these
or swollen feet.

past few months. In the past two months

35

patient also has a history of interrupted

consumption of milk and heavy sweating
during consumption of milk.
Physical Examination
Tachycardia, gallop rhythm, and weak HR: 180 bpm, reguler; murmur (+)
and thready pulses are common
Signs

of

discharges

increased
(e.g.,

pansistolik grade III/6 LMCS III-IV,

sympathetic Extremities

growth

failure,

perspiration, cold and wet skin).

Pulse 140 x/i, iregular,

cold,
In the past two months patient also has a

Tachypnea is common and is an early history of interrupted consumption of

manifestation of CHF in infants.

milk

and

heavy

sweating

during

Dyspnea on exertion (equivalent to poor consumption of milk.

feeding in small infants) is common in
children.
The presence of cardiomegaly should be CTR of 65% - Cardiomegaly, Heart
demonstrated by chest x-ray films. The characteristics: egg on site.
absence of cardiomegaly almost rules out
the diagnosis of CHF
Echocardiography
Echo studies may confirm enlargement of Results:

Large

ventricular chambers and impaired LV pulmonary

ASD,

stenosis

Small
and

systolic function (decreased fractional systolic function

shortening or ejection fraction) as well as
impaired diastolic function by the use of
Doppler techniques. A more important
role of echo may be due to its ability to
determine the cause of CHF.
Theraphy
Three

major

classes

of

drugs

are

Bed rest
O2 Nasal kanul L/i

PDA,

decreased

36

commonly used in the treatment of CHF

in children: diuretics, inotropic agents,
and afterload-reducing agents. Diuretics

Furosemide 2x5mg
Spironolactone 2x6,25mg
Sildenafil 3x1.5mg
IV Dobutamin 5mcg/kgbw/minute

(75mg in 50cc Nacl 0.9%) in

1cc perhour
Diet F75 125cc/2jam/ggt

are usually used with inotropic agents.

Rapid-acting inotropic agents (dopamine,
dobutamine) are used in critically or
acutely ill infants and children.

REFERENCE
1. Brennan P , 2007. Chapter 417 Epidemiology and Genetic Basis of
Congenital Heart Disease In: Nelson Textbook of Pediatrics,17th edition,
Elsevier Science Philadelphia, Pennsylvania.
2. Lister G, 2007. Chapter 418 Evaluation of the Infant or Child with
Congenital Heart Disease In: Nelson Textbook of Pediatrics,17th edition,
Elsevier Science Philadelphia, Pennsylvania.
3. William W.H., 2003. Current Pediatric Diagnosis & Treatment, 16th Edition
The McGraw-Hill Companies, Inc, United States of America.
4. Rudolph C.D., 2003. Rudolph's Pediatrics, Twenty-First Edition, The
McGraw-Hill Companies, Inc, United States of America.
5. Park M. K., 2008, Pediatric Cardiology for Practitioners. 5th edition, Mosby,
Inc., an affiliate of Elsevier Inc, Philadelphia, Pennsylvania.