CASE REPORT

LANDAU KLEFFNER SYNDROME

PRESENTED BY
KOKILAVNI CHANDRAN

110100383

SUPERVISED BY:
dr. Johannes Harlan Saing, M. Ked (Ped), Sp.A(K)

PEDIATRIC HEALTH DEPARTMENT

HAJI ADAM MALIK GENERAL HOSPITAL
UNIVERSITY OF NORTH SUMATERA
2015

CHAPTER 1

BACKGROUND
1.1 Background
Congestive Heart Failure (CHF) in infant and children is an emergency
that is very often encountered by health worker. Complaints and symptoms are
often variable cash and so it is often difficult to distinguish from other disease
outside the heart. Cause, clinical symptoms, determinants and treatment of
CHF in infant and children are different from adults, although the mechanism
is basically the same for all ages. Infant and children is not a miniature adults
size, there are differences in the structure, function, biochemical, and
pharmacological aspects of the heart.
Systematic study of heart failure in children began in earnest in the mid20th century. The most cause of pediatric heart failure remained rheumatic
fever but in the 1950s the novel concept that congenital heart disease
disproportionately caused heart failure in infancy began to gain currency
The cause of heart failure in children depending age so must pay attention
to what the main cause of heart failure is so that we can treat patients according
to the main causes and heart failure is a important complication heart disease
should be aware of all heart defects so that it will never occur and can be
prevented.
1.2 Objective
This paper is one of the requirements to fullfil in the senior clinical
assistance programs in Pediatric Department of Haji Adam Malik General
Hospital, University of Sumatera Utara. In addition, this paper can be used as
reference to know and understanding a little about congestive heart failure and
rheumatic heart disease.

CHAPTER 2
LITERATURE REVIEW

2.1. Rheumatic Heart Disease

2.1.1 Definition
RHD is a chronic heart condition caused by rheumatic fever that can be
prevented and controlled. Rheumatic fever is caused by a preceding group A
streptococcal (strep) infection. Treating strep throat with antibiotics can prevent
rheumatic fever. Moreover, regular antibiotics (usually monthly injections) can
prevent patients with rheumatic fever from contracting further strep infections and
causing progression of valve damage.(1)
2.1.2 Epidemiology
The incidence of rheumatic fever and rheumatic heart disease has not
decreased in developing countries. Retrospective studies reveal developing
countries to have the highest figures for cardiac involvement and recurrence rates
of rheumatic fever. Worldwide, there are over 15 million cases of RHD, with
282,000 new cases and 233,000 deaths from this disease each year (6). At least
15.6 million people are estimated to be currently affected by RHD with a
significant number of them requiring repeated hospitalization and often
unaffordable heart surgery in the next five to 20 years. The worst affected areas
are sub-Saharan Africa, south-central Asia, the Pacific and indigenous populations
of Australia and New Zealand. Up to 1 per cent of all school children in Africa,
Asia, the Eastern Mediterranean region, and Latin America show signs of the
disease.(7)
2.1.3 Etiology and Pathophysiology
Rheumatic fever develops in some children and adolescents following
pharyngitis with group A beta-hemolytic Streptococcus (ie, Streptococcus
pyogenes). The organisms attach to the epithelial cells of the upper respiratory
tract and produce a battery of enzymes allowing them to damage and invade
human tissues. After an incubation period of 2-4 days, the invading organisms

elicit an acute inflammatory response with 3-5 days of sore throat, fever, malaise,
headache, and an elevated leukocyte count.
In 0.3-3% of cases, infection leads to rheumatic fever several weeks after
the sore throat has resolved. Only infections of the pharynx have been shown to
initiate or reactivate rheumatic fever. However, epidemiological associations in
certain populations have led to speculation that group A Streptococcus impetigo
could predispose to or cause rheumatic fever as well (2). The organism spreads by
direct contact with oral or respiratory secretions, and spread is enhanced by
crowded living conditions. Patients remain infected for weeks after symptomatic
resolution of pharyngitis and may serve as a reservoir for infecting others.
Penicillin treatment shortens the clinical course of streptococcal pharyngitis and,
more importantly, is effective in decreasing the incidence of major sequelae.(3)
Group A Streptococcus is a gram-positive coccus that frequently colonizes
the skin and oropharynx. This organism may cause suppurative disease, such as
pharyngitis, impetigo, cellulitis, myositis, pneumonia, and puerperal sepsis. It also
may be associated with nonsuppurative disease, such as rheumatic fever and acute
poststreptococcal glomerulonephritis. Group A streptococci elaborate the cytolytic
toxins streptolysins S and O. Of these, streptolysin O induces persistently high
antibody titers that provide a useful marker of group A streptococcal infection and
its nonsuppurative complications.(3)
Group A Streptococcus, as identified using the Lancefield classification,
has a group A carbohydrate antigen in the cell wall that is composed of a branched
polymer of L- rhamnose and N- acetyl-D-glucosamine in a 2:1 ratio. Group A
streptococci may be subserotyped by surface proteins on the cell wall of the
organism. The presence of the M protein is the most important virulence factor for
group A streptococcal infection in humans. More than 120 M protein serotypes or
M protein genotypes have been identified (3), some of which have a long terminal
antigenic domain (ie, epitopes) similar to antigens in various components of the
human heart.(3)

Rheumatogenic strains are often encapsulated mucoid strains, rich in M

proteins, and resistant to phagocytosis. These strains are strongly immunogenic,
and anti-M antibodies against the streptococcal infection may cross-react with
components of heart tissue (ie, sarcolemmal membranes, valve glycoproteins).(3)
Acute rheumatic heart disease often produces a pancarditis characterized
by endocarditis, myocarditis, and pericarditis. Endocarditis is manifested as valve
insufficiency. The mitral valve is most commonly and severely affected (65-70%
of patients), and the aortic valve is second in frequency (25%). The tricuspid valve
is deformed in only 10% of patients and is almost always associated with mitral
and aortic lesions. The pulmonary valve is rarely affected. Severe valve
insufficiency during the acute phase may result in congestive heart failure and
even death (1% of patients). Whether myocardial dysfunction during acute
rheumatic fever is primarily related to myocarditis or is secondary to congestive
heart failure from severe valve insufficiency is not known. Pericarditis, when
present, rarely affects cardiac function or results in constrictive pericarditis.
Chronic manifestations due to residual and progressive valve deformity
occur in 9-39% of adults with previous rheumatic heart disease. Fusion of the
valve apparatus resulting in stenosis or a combination of stenosis and
insufficiency develops 2-10 years after an episode of acute rheumatic fever, and
recurrent episodes may cause progressive damage to the valves. Fusion occurs at
the level of the valve commissures, cusps, chordal attachments, or any
combination of these. Rheumatic heart disease is responsible for 99% of mitral
valve stenosis in adults in the United States. Associated atrial fibrillation or left
atrial thrombus formation from chronic mitral valve involvement and atrial
enlargement may be observed.
Molecular/genetic factors
Familial studies of rheumatic heart disease suggest a vulnerable population
with increased risk. Relationships between the development of rheumatic fever
and human leukocyte antigen (HLA)-DR subtypes have been found. (3)

2.1.4 Clinical Manifestation

The following are the most common symptoms for rheumatic fever;
however, each individual may experience symptoms differently. Symptoms, which
vary greatly, typically begin one to six weeks after a bout of strep throat, although,
in some cases, the infection may have been too mild to have been recognized. A
diagnosis of rheumatic heart disease is made after confirming antecedent
rheumatic fever. The modified Jones Criteria revised in 1992, provide guidelines
for the diagnosis of rheumatic fever (8). Symptoms may include:
-Fever
-Swollen, tender, red and extremely painful joints--particularly the knees, ankles,
elbows, or wrists
-Nodules over swollen joints
-Red, raised, lattice-like rash, usually on the chest, back, and abdomen
-Shortness of breath, chest discomfort
-Uncontrolled movements of arms, legs, or facial muscles
-Weakness and shortness of breath
2.1.5 Physical Examination
Physical findings in a patient with rheumatic heart disease include cardiac
and noncardiac manifestations of acute rheumatic fever. Some patients develop
cardiac manifestations of chronic rheumatic heart disease. Pancarditis is the most
serious and second most common complication of rheumatic fever (50%). In
advanced cases, patients may complain of dyspnea, mild-to-moderate chest
discomfort, pleuritic chest pain, edema, cough, or orthopnea. Upon physical
examination, carditis is most commonly detected by a new murmur and
tachycardia out of proportion to fever. New or changing murmurs are considered
necessary for a diagnosis of rheumatic valvulitis (9).

Some cardiologists have proposed that echo-Doppler evidence of mitral

insufficiency, particularly in association with aortic insufficiency, may be
sufficient for a diagnosis of carditis (even in the absence of accompanying
auscultatory findings). However, given the sensitivity of modern Doppler devices,
this remains controversial (9).
Other cardiac manifestations include congestive heart failure and
pericarditis. The murmurs of acute rheumatic fever are typically due to valve
insufficiency. The following murmurs are most commonly observed during acute
rheumatic fever (9):
a. Apical pansystolic murmur is a high-pitched, blowing-quality murmur of
mitral regurgitation that radiates to the left axilla. The murmur is
unaffected by respiration or position. Intensity varies but is grade 2/6 or
greater. The mitral insufficiency is related to dysfunction of the valve,
chordae, and papillary muscles.Apical diastolic murmur (also known as a
Carey-Coombs murmur) is heard with active carditis and accompanies
severe mitral insufficiency. The mechanism for this murmur is relative
mitral stenosis, as the large volume of regurgitant flow traverses the mitral
valve during ventricular filling. It is heard best with the bell of the
stethoscope, while the patient is in the left lateral position and the breath
held in expiration.
b. Basal diastolic murmur is an early diastolic murmur of aortic regurgitation
and is high-pitched, blowing, decrescendo, and heard best along the right
upper and mid-left sternal border after deep expiration while the patient is
leaning forward.

Heart failure may develop secondary to severe valve insufficiency or

myocarditis.The physical findings associated with heart failure include tachypnea,
orthopnea, jugular venous distention, rales, hepatomegaly, a gallop rhythm,
edema, and swelling of the peripheral extremities. Whereas, a pericardial friction
rub indicates that pericarditis is present. Increased cardiac dullness to percussion

and muffled heart sounds are consistent with pericardial effusion. A paradoxical
pulse (and accentuated fall in systolic blood pressure with inspiration) with
decreased systemic pressure and perfusion and evidence of diastolic indentation of
the right ventricle on echocardiogram reflect impending pericardial tamponade. In
this clinical emergency, the pericardial effusion should be evacuated by
pericardiocentesis (9).
Common noncardiac manifestations of acute rheumatic fever include
polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. Other
clinical, noncardiac manifestations include abdominal pain, arthralgias, epistaxis,
fever, and rheumatic pneumonia (9).
Polyarthritis is the most common symptom and is frequently the earliest
manifestation of acute rheumatic fever (70-75%) (7).Characteristically, the
arthritis begins in the large joints of the lower extremities (knees and ankles) and
migrates to other large joints in the lower or upper extremities (elbows and
wrists). Affected joints are painful, swollen, warm, erythematous, and limited in
their range of motion. The pain is out of proportion to clinical findings.(9)
The arthritis reaches maximum severity in 12-24 hours, persists for 2-6
days (rarely more than 3 wk) at each site, and rapidly responds to aspirin. Aspirin
improves symptoms in affected joints and prevents further migration of the
arthritis.Polyarthritis is more common and more severe in teenagers and young
adults than in younger children (8).
Sydenham chorea occurs in 10-30% of patients with rheumatic fever.
Patients present with difficulty writing, involuntary grimacing, purposeless
(choreiform) movements of the arms and legs, speech impairment, generalized
weakness, and emotional lability. Physical findings include hyperextended joints,
hypotonia, diminished deep tendon reflexes, tongue fasciculations ("bag of
worms"), and a "milk sign" or relapsing grip demonstrated by alternate increases
and decreases in tension when the patient grips the examiner's hand (8).

In the absence of a family history of Huntington chorea, the diagnosis of

acute rheumatic fever is almost certain. A long latency period (1-6 months)
between streptococcal pharyngitis and the onset of chorea is observed; a history of
an antecedent sore throat is frequently not obtained. Patients with chorea often do
not demonstrate other Jones criteria. Chorea is slightly more common in females
than males. It is also known as rheumatic chorea, Sydenham chorea, chorea minor,
and St Vitus dance. Daily handwriting samples can be used as an indicator of
progression or resolution of disease. Complete resolution of the symptoms
typically occurs with improvement in 1-2 weeks and full recovery in 2-3 months.
However, cases have been reported in which symptoms wax and wane for several
years (9).
Pediatric

autoimmune

neuropsychiatric

disorders

associated

with

streptococcal infections (PANDAS) may be associated with chorea. Children have

been identified in whom group A streptococcal infection appears to have triggered
a relapsing-remitting symptom complex characterized by obsessive-compulsive
disorder (somatic obsessions and checking, cleaning, and repeating compulsions),
and neurologic abnormalities, such as cognitive defects and motoric hyperactivity.
The symptoms are prepubertal in onset and may include emotional lability,
separation anxiety, and oppositional behaviors (1).
Streptococcal infection has been proposed to trigger the formation of
antibodies that cross-react with the basal ganglia of genetically susceptible hosts
in a manner similar to the proposed mechanism for Sydenham chorea, thus
causing the symptom complex (1).
Erythema marginatum, also known as erythema annulare, is a
characteristic rash that occurs in 5-13% of patients with acute rheumatic fever. It
begins as 1-3 cm in diameter, pink-to-red nonpruritic macules or papules located
on the trunk and proximal limbs but never on the face. The lesions spread outward
to form a serpiginous ring with erythematous raised margins and central clearing.
The rash may fade and reappear within hours and is exacerbated by heat. Thus, if

the lesions are not well visualized, they can be accentuated by the application of
warm towels, a hot bath, or the use of tangential lighting. The rash occurs early in
the course of the disease and remains long past the resolution of other symptoms
(4).
Subcutaneous nodules are currently an infrequent manifestation of
rheumatic fever. The frequency has declined over the past several years to 0-8%
of patients with rheumatic fever. When present, the nodules appear over the
extensor surfaces of the elbows, knees, ankles, knuckles, and on the scalp and
spinous processes of the lumbar and thoracic vertebrae where they are attached to
the tendon sheath. They are firm, nontender, and free from attachments to the
overlying skin and range in size from a few mm to 1-2 cm. They vary in number
from one to dozens (mean 3-4). Histologically, they contain areas resembling the
Aschoff bodies seen in the heart (3).
Subcutaneous nodules generally occur several weeks into the disease and
resolve within a month. These nodules are strongly associated with severe
rheumatic carditis, and, in the absence of carditis, the diagnosis of subcutaneous
nodules should be questioned (3).
Valve deformities, thromboembolism, cardiac hemolytic anemia, and atrial
arrhythmias are the most common cardiac manifestations of chronic rheumatic
heart disease. Mitral stenosis occurs in 25% of patients with chronic rheumatic
heart disease and in association with mitral insufficiency in another 40%.
Progressive fibrosis (ie, thickening and calcification of the valve) takes place over
time, resulting in enlargement of the left atrium and formation of mural thrombi in
that chamber (4). The stenotic valve is funnel-shaped, with a "fish mouth"
resemblance. Upon auscultation, S1 is initially accentuated but becomes reduced
as the leaflets thicken. P2 becomes accentuated, and the splitting of S2 decreases
as pulmonary hypertension develops. An opening snap of the mitral valve often is
heard at the apex, where a diastolic filling murmur also is heard (3).

Aortic stenosis from chronic rheumatic heart disease is typically associated

with aortic insufficiency. The valve commissures and cusps become adherent and
fused, and the valve orifice becomes small with a round or triangular shape. Upon
auscultation, S2 may be single because the aortic leaflets are immobile and do not
produce an aortic closure sound. The systolic and diastolic murmurs of aortic
valve stenosis and insufficiency are heard best at the base of the heart (6).
Thromboembolism occurs as a complication of mitral stenosis. It is more
likely to occur when the left atrium is dilated, cardiac output is decreased, and the
patient is in atrial fibrillation. The frequency of this complication has decreased
with the use of anticoagulation and the development of surgical repair for the
valve abnormality (6).
Cardiac hemolytic anemia is related to disruption of the RBCs by a
deformed valve. Increased destruction and replacement of platelets also may
occur. While atrial arrhythmias are typically related to a chronically enlarged left
atrium (from a mitral valve abnormality). Successful cardioversion of atrial
fibrillation to sinus rhythm is more likely to be successful if the left atrium is not
markedly enlarged, the mitral stenosis is mild, and the patient has been in atrial
fibrillation for less than 6 months. Patients should be anticoagulated before
cardioversion to decrease the risk of systemic embolization (6).

2.1.6 Laboratory studies

There are a few laboratory studies which can be carried out to diagnose
Rheumatic Heart Disease, such as:
a) Throat culture

Throat culture findings for group A beta hemolytic Streptococcus are

usually negative by the time symptoms of rheumatic fever or rheumatic heart
disease appear. Attempts should be made to isolate the organism before the
initiation of antibiotic therapy to help confirm a diagnosis of streptococcal
pharyngitis and to allow typing of the organism if it is isolated successfully.
b) Rapid antigen detection test
This test allows rapid detection of group A streptococcal antigen and
allows the diagnosis of streptococcal pharyngitis and the initiation of antibiotic
therapy while the patient is still in the physician's office. Because the rapid
antigen detection test has a specificity of greater than 95% but a sensitivity of only
60-90%, a throat culture should be obtained in conjunction with this test.
c) Antistreptococcal antibodies
The clinical features of rheumatic fever begin at the time antistreptococcal
antibody levels are at their peak. Thus, antistreptococcal antibody testing is useful
for confirming previous group A streptococcal infection. The elevated level of
antistreptococcal antibodies is useful, particularly in patients that present with
chorea as the only diagnostic criterion. Sensitivity for recent infections can be
improved by testing for several antibodies. Antibody titers should be checked at 2week intervals in order to detect a rising titer.
The most common extracellular antistreptococcal antibodies tested include
antistrptolysin O (ASO), antideoxyribonuclease (DNAse) B, antihyaluronidase,
antistreptokinase, antistreptococcal esterase, and anti-DNA. Antibody tests for
cellular components of group A streptococcal antigens include antistreptococcal
polysaccharide, antiteichoic acid antibody, and antiM protein antibody.
In general, the ratio of antibodies to extracellular streptococcal antigens
rises during the first month after infection and then plateaus for 3-6 months before
returning to normal levels after 6-12 months. When the ASO titer peaks (2-3 wk
after the onset of rheumatic fever), the sensitivity of this test is 80-85%. The anti-

DNAse B has a slightly higher sensitivity (90%) for detecting rheumatic fever or
acute glomerulonephritis. Antihyaluronidase results are frequently abnormal in
rheumatic fever patients with a normal level of ASO titer and may rise earlier and
persist longer than elevated ASO titers during rheumatic fever.
d) Acute phase reactants
The C-reactive protein and erythrocyte sedimentation rate are elevated in
rheumatic fever due to the inflammatory nature of the disease. Both tests have a
high sensitivity but low specificity for rheumatic fever. They may be used to
monitor the resolution of inflammation, detect relapse when weaning aspirin, or
identify the recurrence of disease.
e) Heart reactive antibodies
Tropomyosin is elevated in acute rheumatic fever.
f) Rapid detection test for D8/17
This immunofluorescence technique for identifying the B cell marker
D8/17 is positive in 90% of patients with rheumatic fever. It may be useful for
identifying patients who are at risk for developing rheumatic fever.

2.1.7 Electrocardiography
On ECG, sinus tachycardia most frequently accompanies acute rheumatic
heart disease. Alternatively, some children develop sinus bradycardia from
increased vagal tone. No correlation between bradycardia and the severity of the
carditis is noted.

First-degree atrioventricular (AV) block (prolongation of the PR interval)

is observed in some patients with rheumatic heart disease. This abnormality may
be related to localized myocardial inflammation involving the AV node or to
vasculitis involving the AV nodal artery. First-degree AV block is a nonspecific
finding and should not be used as a criterion for the diagnosis of rheumatic heart
disease. Its presence does not correlate with the development of chronic rheumatic
heart disease.Second-degree (intermittent) and third-degree (complete) AV block
with progression to ventricular standstill have been described. Heart block in the
setting of rheumatic fever, however, typically resolves with the rest of the disease
process.When acute rheumatic fever is associated with pericarditis, ST segment
elevation may be present and is marked most in lead II, III, aVF, and V4
-V6.Patients with rheumatic heart disease also may develop atrial flutter,
multifocal atrial tachycardia, or atrial fibrillation from chronic mitral valve disease
and atrial dilation.
2.1.8 Imaging Studies
In acute rheumatic heart disease, Doppler-echocardiography identifies and
quantitates valve insufficiency and ventricular dysfunction. Studies in Cambodia
and Mozambique demonstrated a 10-fold increase in the prevalence of rheumatic
heart disease when echocardiography is used for clinical screening compared with
strictly clinical findings (7).
With mild carditis, Doppler evidence of mitral regurgitation may be
present during the acute phase of disease but resolves in weeks to months. In
contrast, patients with moderate-to-severe carditis have persistent mitral and/or
aortic regurgitation.
The most important echocardiographic features of mitral regurgitation
from acute rheumatic valvulitis are annular dilatation, elongation of the chordae to
the anterior leaflet, and a posterolaterally directed mitral regurgitation jet.During
acute rheumatic fever, the left ventricle is frequently dilated in association with a
normal or increased fractional shortening. Thus, some cardiologists believe that

valve insufficiency (from endocarditis), rather than myocardial dysfunction (from

myocarditis), is the dominant cause of heart failure in acute rheumatic fever.
In chronic rheumatic heart disease, echocardiography may be used to track
the progression of valve stenosis and may help determine the time for surgical
intervention. The leaflets of affected valves become diffusely thickened, with
fusion of the commissures and chordae tendineae. Increased echodensity of the
mitral valve may signify calcification.The image below depicts the typical systolic
mitral insufficiency jet observed with rheumatic heart disease.

Parasternal long-axis view demonstrating the typical systolic mitral insufficiency

jet observed with rheumatic heart disease (blue jet extending from the left
ventricle into the left atrium). The jet is typically directed to the lateral and
posterior wall. (LV=left ventricle; LA=left atrium; Ao=aorta; RV=right ventricle).

The image below depicts the typical diastolic aortic insufficiency jet observed
with rheumatic heart disease.

Parasternal long-axis view demonstrating the typical diastolic aortic insufficiency

jet observed with rheumatic heart disease (red jet extending from the aorta into the
left ventricle). (LV=left ventricle; LA=left atrium; Ao=aorta; RV=right ventricle).

The World Heart Federation has published guidelines for identifying

individuals with rheumatic heard disease without a clear history of acute
rheumatic fever. Based on 2-dimensional (2D) imaging and pulsed and color
Doppler interrogation, patients are divided into 3 categories: definite rheumatic
heart disease, borderline rheumatic heart disease, and normal. For pediatric
patients (defined as age < 20 y), definite echo features include pathologic mitral
regurgitation (MR) and at least 2 morphological features of rheumatic heart
disease of the mitral valve, mitral stenosis mean gradient of more than 4 mm Hg,
pathological aortic regurgitation and at least 2 morphological features of
rheumatic heart disease of the aortic valve, or borderline disease of both the aortic
valve and mitral valve (14).

2.1.9 Differential Diagnosis

- Congenital Mitral Stenosis
- Dilated Cardiomyopathy
- Heart Failure, Congestive
- Pediatric Aortic Valve Insufficience
- Pediatric Bacterial Endocarditis
- Pediatric Infective Pericarditis
- Pediatric Mitral Valve Insufficiency
- Pediatric Mitral Valve Prolapse
- Pediatric Septic Arthritis
- Pediatric Valvar Aortic Stenosis
2.1.10 Prevention and Treatment
a) Prevention of rheumatic fever in patients with group A beta hemolytic
streptococci (GABHS) pharyngitis:
For patients with GABHS pharyngitis, a meta-analysis supports a
protective effect against rheumatic fever when penicillin is used following the
diagnosis.Oral (PO) penicillin V remains the drug of choice for treatment of
GABHS pharyngitis, but ampicillin and amoxicillin are equally effective.When
PO penicillin is not feasible or dependable, a single dose of intramuscular
benzathine penicillin G or benzathine/procaine penicillin combination is
therapeutic (8).
For patients who are allergic to penicillin, administer erythromycin or a
first-generation cephalosporin. Other options include clarithromycin for 10 days,
azithromycin for 5 days, or a narrow-spectrum (first-generation) cephalosporin for

10 days. As many as 15% of patients who are allergic to penicillin are also allergic
to cephalosporins (8).
For recurrent group A streptococci (GAS) pharyngitis, a second 10-day
course of the same antibiotic may be repeated. Alternate drugs include narrowspectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin, erythromycin, or
other macrolides.
Control measures for patients with GABHS pharyngitis are as follows:
a) Hospitalized patients: Place hospitalized patients with GABHS pharyngitis
of pneumonia on droplet precautions, as well as standard precautions, until
24 hours after initiation of appropriate antibiotics.
b) Exposed persons: People in contact with patients having documented cases
of streptococcal infection first should undergo appropriate laboratory
testing if they have clinical evidence of GABHS infection and should
undergo antibiotic therapy if infected.
c) School and childcare centers: Children with GABHS infection should not
attend school or childcare centers for the first 24 hours after initiating
antimicrobial therapy
Treatment of the acute inflammatory manifestations of acute rheumatic fever
consists of salicylates and steroids. Aspirin in anti-inflammatory doses effectively
reduces all manifestations of the disease except chorea, and the response is
typically dramatic.If rapid improvement is not observed after 24-36 hours of
therapy, question the diagnosis of rheumatic fever. Attempt to obtain aspirin blood
levels from 20-25 mg/dL, but stable levels may be difficult to achieve during the
inflammatory phase because of variable GI absorption of the drug. Maintain
aspirin at anti-inflammatory doses until the signs and symptoms of acute
rheumatic fever are resolved or residing (6-8 wk) and the acute phase reactants
(APRs) have returned to normal (8).

Anti-inflammatory doses of aspirin may be associated with abnormal liver

function tests and GI toxicity, and adjusting the aspirin dosage may be
necessary.When discontinuing therapy, withdraw aspirin gradually over weeks
while monitoring the APRs for evidence of rebound. Chorea is most frequently
self-limited but may be alleviated or partially controlled with phenobarbital or
diazepam.
If moderate to severe carditis is present as indicated by cardiomegaly, thirddegree heart block or congestive heart failure, substitute PO prednisone for
salicylate therapy. Continue prednisone for 2-6 weeks depending on the severity
of the carditis, and taper prednisone during the final week(s) of therapy. Weaning
prednisone therapy after a shorter period (2-4 weeks) while initiating and
maintaining salicylates for several weeks can minimize adverse effects of the
steroids while preventing rebound of the carditis.
Include digoxin and diuretics, afterload reduction, supplemental oxygen, bed
rest, and sodium and fluid restriction as additional treatment for patients with
acute rheumatic fever and heart failure. The diuretics most commonly used in
conjunction with digoxin for children with heart failure include furosemide and
spironolactone. Initiate digoxin only after checking electrolytes and correcting
hypokalemia.
The total digitalizing dose is 20-30 mcg/kg PO, with 50% of the dose
administered initially, followed by 25% of the dose 12 hours and 24 hours after
the initial dose. Maintenance doses typically are 8-10 mcg/kg/d PO in 2 divided
doses. For older children and adults, the total loading dose is 1.25-1.5 mg PO, and
the maintenance dose is 0.25-0.5 mg PO every day. Therapeutic digoxin levels are
present at trough levels of 1.5-2 ng/mL.
Afterload reduction (using

ACE inhibitor captopril) may be effective in

improving cardiac output, particularly in the presence of mitral and aortic

insufficiency. Start these agents judiciously. Use a small, initial test dose (some

patients have an abnormally large response to these agents), and administer only
after correcting hypovolemia.
When heart failure persists or progresses during an episode of acute
rheumatic fever in spite of aggressive medical therapy, surgery is indicated and
may be life-saving for severe mitral and/or aortic insufficiency.
b)Treatment for patients following rheumatic heart disease (RHD):
Preventive and prophylactic therapy is indicated after rheumatic fever and
acute rheumatic heart disease to prevent further damage to valves.Primary
prophylaxis (initial course of antibiotics administered to eradicate the
streptococcal infection) also serves as the first course of secondary prophylaxis
(prevention of recurrent rheumatic fever and rheumatic heart disease).
An injection of 0.6-1.2 million units of benzathine penicillin G
intramuscularly every 4 weeks is the recommended regimen for secondary
prophylaxis for most US patients. Administer the same dosage every 3 weeks in
areas where rheumatic fever is endemic, in patients with residual carditis, and in
high-risk patients (16).
Although PO penicillin prophylaxis is also effective, data from the World
Health Organization indicate that the recurrence risk of GABHS pharyngitis is
lower when penicillin is administered parentally (16).
The duration of antibiotic prophylaxis is controversial. Continue antibiotic
prophylaxis indefinitely for patients at high risk (eg, health care workers, teachers,
daycare workers) for recurrent GABHS infection. Ideally, one could argue for
continuing prophylaxis indefinitely, because recurrent GABHS infection and
rheumatic fever can occur at any age; however, the American Heart Association
currently recommends that patients with rheumatic fever without carditis receive
prophylactic antibiotics for 5 years or until aged 21 years (16), whichever is
longer.[14] Patients with rheumatic fever and carditis but no valve disease should
receive prophylactic antibiotics for 10 years or well into adulthood, whichever is

longer. Finally, patients with rheumatic fever with carditis and valve disease
should receive antibiotics for at least 10 years or until age 40 years.
Patients with rheumatic heart disease and valve damage require a single
dose of antibiotics 1 hour before surgical and dental procedures to help prevent
bacterial endocarditis. Patients who had rheumatic fever without valve damage do
not need endocarditis prophylaxis. Do not use penicillin, ampicillin, or
amoxicillin for endocarditis prophylaxis in patients already receiving penicillin
for secondary rheumatic fever prophylaxis (relative resistance of PO streptococci
to penicillin and aminopenicillins).Alternate drugs recommended by the American
Heart Association for these patients include PO clindamycin (20 mg/kg in
children, 600 mg in adults) and PO azithromycin or clarithromycin (15 mg/kg in
children, 500 mg in adults) (16).
The diet for such patients should be nutritious and without restrictions
except in the patient with congestive heart failure. In these patients, fluid and
sodium intake should be restricted. Potassium supplementation may be necessary
if steroids or diuretics are used.Initially, patients should be placed on bed rest,
followed by a period of indoor activity before being permitted to return to school.
Full activity should not be allowed until the acute phase reactants have returned to
normal levels
When heart failure persists or worsens after aggressive medical therapy for
acute rheumatic heart disease, surgery to decrease valve insufficiency may be lifesaving. Forty percent of patients with acute rheumatic heart disease subsequently
develop mitral stenosis as adults. In patients with critical stenosis, mitral
valvulotomy, percutaneous balloon valvuloplasty, or mitral valve replacement
may be indicated.

2.2. Congestive Heart Failure

2.2.1. Definition
Congestive Heart failure (CHF) is a complex syndrome, with several
definitions, the commonest being an abnormality of cardiac function whereby
heart in unable to pump at a rate commensurate with the requirement of the
metabolizing tissues, or does so only at elevated filling pressures. In case of
children, this requirement includes growth and development (10).

2.2.2. Etiology
Etiology of congestive heart failure depending on the age of the child.
Fetus: severe anemia, tachycardia supraventricular, tachycardia ventricular, AV
Block total
Neonatus premature: fluid overload, PDA, VSD,Cor pulmonale, hypertension
Neonatus: Cardiomyopathy asphyxia, COA, Myocarditis virus
Baby: VSD, Hemangioma, Cardiomyopathy metabolic, acute hypertension,
Tachycardia supraventricular, Kawasaki disease
Children

Teenagers:

Rheumatic

fever,

Endocarditis,

glomerulonefritis,

myocarditis, tirotoksikosis, hemokromtosis-hemosiderosis, cancer treatment,

cystic fibrosis, cardiomyopathy (10).
2.2.3. Classification
Part of defining heart failure is defining a spectrum of severity. The wellestablished New York Heart Association (NYHA) Heart Failure Classification is
not applicable to most of the pediatric population. The ROSS Heart Failure
Classification was developed to provide a global assessment of heart failure
severity in infants, and has subsequently been modified to apply to all pediatric
ages. The modified Ross Classification incorporates feeding difficulties, growth
problems, and symptoms of exercise intolerance into a numeric score comparable
with the NYHA classification for adults More recently.

None of these measures has been validated as surrogate clinical end points
in large numbers of children or patients with congenital heart disease, but
neurohormonal activation and deteriorating clinical status have been shown to
correlate with increasing class (11).
Ross Heart Failure Classification For Children
Class I
Asymptomatic
Class II
Mild tachypnea or diaphoresis with feeding in infant
Class III

Dyspnea on exertion in older children

Marked tachypnea or diaphoresis with feeding in infant
Marked dispnea on exertion

Class IV

Prolonge feeding times with growth failure

Symptome such as tachypnea, retraction, grunting, or diaphoresis
at rest

New York Heart Association (NYHA) Functional Classification

Class
Patient Symptoms
Class I
No limitation of physical activity. Ordinary physical activity does not
cause undue fatigue, palpitation (feeling heart beats), or dyspnea
Class

(shortness of breath).
II Slight limitation of physical activity. Comfortable at rest, but ordinary

(Mild)
physical activity results in fatigue, palpitation, or dyspnea.
Class III Marked limitation of physical activity. Comfortable at rest, but less
(Moderate) than ordinary activity causes fatigue, palpitation, or dyspnea.
Class IV Unable to carry out any physical activity without discomfort.
(Severe)

Symptoms of cardiac insufficiency at rest. If any physical activity is

undertaken, discomfort is increased.

2.2.4. Pathophysiology

When the ventricular end-diastolic volume increase, a healthy heart will

increase up to a maximum cardiac output and cardiac output is achieved can not
be enlarged again (Frank-Starling principle). Increase in stroke volume achieved
in this manner due to the strain of myocardial fibers, but also raise the wall strain
and increase myocardial oxygen consumption.
Heart failure is not a clinical situation involving only one body system but rather a
clinical syndrome due to heart abnormalities so that the heart is unable to pump
meet metabolic needs of the body. Heart failure is characterized with a
hemodynamic response, kidneys, nervous and hormonal real as well as a
pathological state of a decrease in heart function. One of abnormal hemodynamic
response is an increase in filling pressure of the heart or preload.Response to the
heart causing some compensation mechanism which aims to improve blood
volume, the volume of the heart chamber,resistance peripheral blood and cardiac
muscle hypertrophy.
This condition also causes activation of the body's compensatory
mechanisms that acute form of hoarding water and salt by the kidneys and
nervous system adrenergic activation. Important to distinguish between the heart's
ability to pump with the contractility of the heart muscle. In some circumstances
found excessive load causing failure the heart as a pump without depression of the
heart muscle are intrinsic. On the contrary may also occur depression intrinsic
cardiac muscle but is not clinically visible signs of heart failure due to cardiac
load light. At the beginning of heart failure due to low cardiac output, in the body
of an increase in activity of the sympathetic nervous system and the reninangiotensin-aldosterone system, as well as the release of arginine vasopressin is
everything a compensatory mechanism to maintain blood pressure adequate.
The decline will be followed by a decrease in ventricular contractility
cardiac output which further decrease in blood pressure and a decrease in effective
arterial blood volume.This will stimulate the neurohumoral compensatory
mechanisms.
Vasoconstriction and water retention will temporarily increase blood pressure
while the increase preload and increase cardiac contractility through Starling law.

If this situation is not resolved soon, afterload elevation, elevation of preload and
cardiac muscle hypertrophy will further increase the burden of the heart, causing
heart failure who are not compensated. Dilated ventricle systolic dysfunction
(decreased ejection fraction) and fluid retention increases the ventricular volume
(dilatation). Dilated cardiac mechanically inefficient will cause ventricular
dysfunction.Congestive heart failure occurs stagnation of blood flow, systemic
embolization of the mural thrombus, and refractory ventricular dysrhythmias.
Mechanisms underlying heart failure include impaired ability cardiac
contractility, which causes cardiac output is lower than normal cardiac output. The
concept of cardiac output described by the equation CO = HR x SV where cardiac
output is a function of heart rate multiplied by the stroke volume. Reduced cardiac
output resulted in the sympathetic nervous system will increase heart rate to
maintain normal cardiac output. If the compensation mechanism to maintain
adequate tissue perfusion, the stroke volume must adjust to maintain cardiac
output. But in heart failure all this happened so that the disturbances in cardiac
output that is pumped by the ventricles is inadequate (11).

2.2.5. Diagnosis

Thorough history taking and physical examination, including an

assessment of the upper-extremity and lower-extremity blood pressures, are
crucial in the evaluation of an infant or child with congestive heart failure.
Regardless of the etiology, the first manifestation of congestive heart failure is
usually tachycardia. An obvious exception to this finding occurs in congestive
heart failure due to a primary bradyarrhythmia or complete heart block.
As the severity of congestive heart failure increases, signs of venous
congestion usually ensue. Left-sided heart failure is generally associated with
signs of pulmonary venous congestion, whereas right-sided heart failure is
associated with signs of systemic venous congestion. Marked failure of either
ventricle, however, can affect the function of the other, leading to systemic and
pulmonary venous congestion.
Later stages of congestive heart failure are characterized by signs and
symptoms of low cardiac output. Generally, congestive heart failure with normal
cardiac output is called compensated congestive heart failure, and congestive heart
failure with inadequate cardiac output is considered decompensated.
Signs of congestive heart failure vary with the age of the child.Signs of
pulmonary venous congestion in an infant generally include tachypnea,
respiratory distress (retractions), grunting, and difficulty with feeding. Often,
children with congestive heart failure have diaphoresis during feedings, which is
possibly related to a catecholamine surge that occurs when they are challenged
with eating while in respiratory distress.
Right-sided venous congestion is characterized by hepatosplenomegaly
and, less frequently, by edema or ascites. Jugular venous distention is not a
reliable indicator of systemic venous congestion in infants, because the jugular
veins are difficult to observe. In addition, the distance from the right atrium to the
angle of the jaw may be no more than 8-10 cm, even when the individual is sitting
upright.
Uncompensated congestive heart failure in an infant primarily manifests as
a failure to thrive. In severe cases, failure to thrive may be followed by signs of
renal and hepatic failure.

In older children, left-sided venous congestion causes tachypnea,

respiratory distress, and wheezing. Right-sided congestion may result in
hepatosplenomegaly, jugular venous distention, edema, ascites, and/or pleural
effusions.
Older children with uncompensated congestive heart failure may have
fatigue or lower-than-usual energy levels. Patients may complain of cool
extremities, abdominal pain, nausea/vomiting, exercise intolerance, dizziness, or
syncope.
Clinical findings may include hypotension, cool extremities with poor
peripheral perfusion, a thready pulse, and decreased urine output. Chemical
evidence of renal and liver dysfunction may be present, as well as a diminished
level of consciousness. Children with uncompensated congestive heart failure,
particularly older children, generally have a lower cardiac output than that which
most experienced clinicians would estimate on the basis of the clinical signs. (17)
Signs and symptoms of congestive heart failure include the following:

Tachycardia

Venous congestion - Right-sided (hepatomegaly, ascites, abdominal pain,

pleural effusion, edema, jugular venous distention); left-sided (tachypnea,
retractions, nasal flaring or grunting, rales, pulmonary edema)

Low cardiac output - Fatigue or low energy, pallor, sweating, cool

extremities,

nausea/vomiting,

poor

growth,

dizziness,

altered

consciousness, and syncope

Appropriate laboratory testing includes assessment of the following:
oxygen saturation, complete blood count (CBC), hemoglobin concentration,
electrolyte levels, calcium level, cardiac biomarkers, blood urea nitrogen (BUN)
level, creatinine level, and renal and hepatic function. The CBC count can reveal
signs of anemia or infection.

Brain natriuretic peptide (BNP) or N -terminal prohormone BNP (NTproBNP) levels are elevated as a result of ventricular dilation. Elevation of serum
BNP level is particularly useful in distinguishing patients with congestive heart
failure from those with a primary respiratory process. BNP levels of more than
100 pg/mL are associated with congestive heart failure in adults and children.
Normal levels may be slightly higher in neonates. Serial measurements of BNP
levels in children with primary myocardial dysfunction and acute decompensated
heart failure in which levels are persistently elevated and/or there is a lesser
degree of decline in the first week of presentation are adverse prognostic factors.
Cardiac troponin may be elevated in cases of myocarditis or after ischemic
injury due to coronary anomaly or cardiomyopathy, as well as in noncardiac
conditions in which cardiac perfusion may be compromised (sepsis).
The evaluation of serum electrolyte levels in the patient with congestive
heart failure may demonstrate hyponatremia secondary to water retention.
Elevated potassium levels may represent renal compromise or even tissue
destruction due to low cardiac output. Significant tissue hypoxia increases serum
lactate concentration and depletes the serum bicarbonate level. In more chronic
congestive heart failure states, reduced renal blood flow may be expressed as
increased BUN and creatinine levels.
12-lead electrocardiogram (ECG) may reveal evidence of structural or
coronary artery disease or a complete atrioventricular block or arrhythmia.
Pulse oximetry, as well as a hyperoxia test in newborns, may be useful.
The systemic saturation on room air is a more reliable measure of oxygenation
than are observations for cyanosis alone, which are often misleading. The partial
pressure of arterial oxygen (PaO2) when the patient is receiving 100% oxygen
(hyperoxia test) may help in distinguishing intracardiac mixing malformations
from pulmonary disease in the setting of hypoxia. Blood gas abnormalities may
show respiratory alkalosis in mild forms of congestive heart failure or metabolic
acidosis in patients with evidence of low cardiac output or ductal-dependent
congenital heart disease (11).

Radiography and Echocardiography

In the presence of congestive heart failure, the cardiac silhouette is usually
enlarged on the chest radiograph. As with BNP elevation, cardiac enlargement
may help to distinguish patients with congestive heart failure from those with
respiratory disease. However, exceptions may include restrictive cardiomyopathy,
venous obstruction (total anomalous pulmonary venous obstruction), and diastolic
dysfunction due to high ventilator mean airway pressures, displaying a normal
cardiac size on chest radiographs. Increased pulmonary blood flow may be
present, along with pulmonary edema or venous congestion (44). (See the image
below.)

Chest radiograph shows signs of congestive heart failure (CHF).

Echocardiography is indicated in any child with unexplained congestive
heart failure to assess cardiac function and identify potential cardiovascular
causes, particularly anatomic lesions and cardiomyopathy. On the other hand,
congestive heart failure itself is not an echocardiographic diagnosis; therefore, the
underlying etiology is best identified by means of detailed history taking and
physical examination and often by means of chest radiography. When oral
sedation is performed for echocardiography, note that children with a low cardiac
output can depend on endogenous catecholamine levels to maintain tissue

perfusion. Sedation can cause withdrawal of the endogenous catecholamine drive,

resulting in cardiac decompensation.

2.2.6. Treatment
The management of congestive heart failure (CHF) is difficult and
sometimes dangerous without knowledge of the underlying cause. Consequently,
the first priority is acquiring a good understanding of the etiology. The goals of
medical therapy for congestive heart failure include the following:

Reducing the preload

Enhancing cardiac contractility

Reducing the afterload

Improving oxygen delivery

Enhancing nutrition
As previously discussed, the causes of congestive heart failure vary, and

they appear in different patients to variable degrees. Thus, the medical

management of congestive heart failure in children should be tailored to the
specific details of each case (11).
Pharmacologic Therapy
Preload reduction can be achieved with oral (PO) or intravenous (IV)
diuretics (eg, furosemide, thiazides, metolazone). Venous dilators (eg,
nitroglycerin) can be administered, but their use is less common in pediatric
practice. Contractility can be supported with IV agents (eg, dopamine) or mixed
agents (eg, dobutamine, inamrinone, milrinone). Digoxin appears to have some
benefit in congestive heart failure, but the exact mechanism is unclear.

Afterload reduction is obtained orally through administration of

angiotensin-converting enzyme (ACE) inhibitors or intravenously through
administration of other agents, such as hydralazine, nitroprusside, and alprostadil.
Pharmaceutical agents used in the treatment of congestive heart failure are
summarized in the Table below.
Table. Pharmaceutical Agents Used in the Treatment of Congestive Heart Failure

Agent
Pediatric Dose
Preload Reduction
Furosemide
1 mg/kg/dose PO or IV
Hydrochlorothiazide 2 mg/kg/d PO divided bid
Metolazone

0.2 mg/kg/dose PO

Comment
May increase to qid
May increase to qid
Used with loop diuretic,
may increase to bid

Inotropic
Preterm infants: 0.005 mg/kg/d
PO divided bid or 75% of this
Digoxin

dose IV; age 10 y: 0.005 ...

mg/kg/d PO qd or 75% of this
dose IV
5-10 mcg/kg/min IV (usual

Dopamine

dosage; maximal dosage may

be up to 28 mcg/kg/min)

Dobutamine

5-10 mcg/kg/min IV

Epinephrine

0.01-0.03 mcg/kg/min IV

Milrinone

0.3-1 mcg/kg/min IV

Gradually titrate upward to

desired effect
Gradually titrate upward to
desired effect
Not to exceed

0.1-0.3

mcg/kg/min
Typically used

without

loading dose, especially in

unstable

patients

Load: 50 mcg/kg IV over

15 min

Afterload Reduction
0.1-0.5 mg/kg/d PO divided
Captopril
...
q8h
Adults: 2.5-5 mg/day PO
qd/bid
Enalapril

0.1 mg/kg/d PO divided qd/bid, slowly

not to exceed 0.5 mg/kg/d

initially;
at

1-

titrate

to

2-wk

intervals; target dose is 1020 mg PO bid; not to

exceed 40 mg/day
Adults: Usual dosage is

Lisinopril

Not established

10mg PO qd (range, 2.5-10

mg)

Initial dose for hypertension

Losartan

is 0.1 mg/kg/day PO; dosage

Adults: 25-100 mg/d PO qd

for treatment of CHF is not

or divided bid

established in children
Nitroprusside

0.5-10 mcg/kg/min IV

Nitroglycerin

0.1-0.5 mcg/kg/min IV

May

need

to

monitor

cyanide level
Vasodilator
Initiate
with

0.01

mcg/kg/min

Nesiritide

0.01-0.03 mcg/kg/min IV
May

cause

hypotension
Alprostadil*

0.03-0.1 mcg/kg/min IV

...

dose-related

Beta-Blockade
Adults: 12.5-25 mg PO bid
Limited

data

suggest

therapeutic dosage range of

Carvedilol

0.2-0.4 mg/kg/dose PO bid;

initiate with lower dose and Initiate with 3.125 mg PO
gradually increase dose q2- bid
3wk to therapeutic range

Metoprolol
Not established
Selective Aldosterone Antagonists
Spironolactone

Adults: 25-100 mg PO qd

1-3.3 mg/kg/day PO in single

or divided doses

Adults: 12.5-50 mg PO qd;

reduce dose to 25 mg qod
if hyperkalemia occurs
25-50 mg PO qd

Eplerenone
Not established
*Prostaglandin E1 (PGE1).

Managing Acute Congestive Heart Failure in Child

Long-standing but unrecognized congestive heart failure may present
acutely; similarly, an acute presentation may represent an acute onset of acquired
cardiac

disease

(myocarditis

or

arrhythmia).

Management

of

acute

decompensation involves treatment of presenting symptoms and adjustment or

initiation of long-term therapy.
In older children with acute congestive heart failure, admit to the ICU for
diuresis with IV furosemide. For patients with significant hypotension, IV
dopamine (5-10 mcg/kg/min) or milrinone (0.3-1 mcg/kg/min) infusion is
appropriate until stabilization is achieved. Older children may require the
placement of a central venous or pulmonary artery catheter to monitor venous
pressure and cardiac output during stabilization.

Nitrates (nitroprusside, nitroglycerin) or nesiritide may be useful in patients with

elevated pulmonary capillary wedge pressure and pulmonary congestion due to
their venous dilating effects. Nesiritide carries the additional theoretical benefits
of reversing deleterious neurohumoral responses and increasing natriuresis. Small
studies have been conducted to measure hemodynamic effects of nesiritide in
children with dilated cardiomyopathy. However, nesiritide has demonstrated no
mortality advantage compared with nitroglycerin for acute decompensated heart
failure in a large adult trial. (10)

CHAPTER III
CASE REPORT
3.1 Objective
The objective of this paper is to report a case of a 12 years old and 6
months old boy with a diagnosis of CHF ec Rheumatoid Heart Disease.
3.2 Case
AT a 12 years and 6 months years old boy, BW 25 kg and a BH 130 cm,
admitted to emergency room in Haji Adam Malik General Hospital Medan on 3 rd
June 2015 at 04.00 pm with a complain tiredness.

3.3 History of Disease

AT was admitted to Haji Adam Malik General Hospital Medan
complaining fatigue since +/- 1 year prior to admitted to the hospital and have
been worsening for 3 months. Patient experienced fatigue while doing daily
activities without exercise. Patient reported paroxysmal nocturnal dyspnea and
sleeping with raised pillow to reduce symptom. Patient has been limiting his
normal routine due to shortness of breath. There is no history of such symptom
experienced. History of fever and sore throat were reported. Patient also reported
multiple joint pain. Patient has been also suffering weight loss for the past 12
months. Patient was referred from Lubuk Pakam Hospital and was diagnosed with
rheumatic heart disease.
Previous illness: Throat pain a year ago is found. Tiredness and dyspnea during
childhood is denied by the parents of patient. Tiredness during breast feeding was
not found.
History of medication: Spironolactone, furosemide
History of family: Unclear
History of parents medication: Unclear
History of Pregnancy: The age of mother was 38 years old during pregnancy.
The gestation was 36 weeks.
History of Birth: Birth was assisted by a midwife. The patient was born
pervaginal and cried immediately after birth. Body weight at birth was 2800 gram,
body length at birth was 50 cm, and head circumference was unclear.
History of feeding: 12 months of being breast fed.
History of immunization: Incomplete immunization.
History of growth and development: The patients mother reported that AT grew
normally. AT had developed talking, crawling, and walking skills on time.

Physical Examination:

Present Status: Level of consciousness: alert. Body temperature: 37C,

BW: 25 kg, BH: 130 cm. BW/A: 53.4%, BL/A: 89.5%, BW/BL: 78,3%
anemic (-), ikteric (+), dyspnea (+), cyanosis (-), edema (-).

Eye: light reflex +/+, conjunctiva palpebra

inferior pale (-/-)

Ear: within normal range
Nose: O2 nasal canule, breathing through

nostrils visible
Mouth: within normal range

L
o
c
a
li
z
e

d Status
- Head: Eye: eye light reflect +/+, conjunctiva palpebral inferior pale -/-,
ear/nose/mouth: within normal range.
- Neck:
Jugular Vein Pressure: +2 cm H2o
- Thorax:
Symetrical fusiformis, Retractions (-), HR: 116 x/i, regular, systolic
murmur (+) grade IV/6 ICR III-IV linea left midclavicularis, ictus
palpable. RR: 24x/i, Ronchi -/-

- Abdomen:
Soepel, Peristaltic (+) N, Hepar: Palpable 2cm below the costal
margin, Lien: unpalpable
- Extremities:
Pulse: 112x/i, regular, adequate pressure and volume, warm, CRT <
3, edema pretibial (-), blood pressure: 100/70 mmHg, SaO2 : 98%
Radiography

Results: CTR 56% Aorta dilatation (-), Pulmonal dilatation (-), Apex downward,
Cardio thoracic waist straightened, Congestion (+), Infiltrat (-)Conclusion :
Cardiomegaly with congestion, straightened cardio thoracic waist.

ECG

Results: Sinus Rhythm, HR 113x/i, P wave unpredictable, Interval PR

unpredictable, Normo Axis, QRS duration 0.04s, segment ST depression, T wave
invertion V3, V4, and Left Ventricular Hypertrophy.
Conclusion: Sinus rhythm, inferior anterolateral myocardial ischemic, Left
ventricular hypertrophy.
Laboratory Findings:
3rd July 2015
Complete blood count:
Test

Result

Unit

Referal

Hemoglobin

15.20

g%

12.0-14.4

Erythrocyte

6.64

106/mm3

4.75-4.85

Leucocyte

15.85

103/mm3

4.5-11.0

Thrombocyte

44.90

103/mm3

150-450

Hematocrite

31.7

36-42

Eosinophil

5.70

1-6

Basophil

0.400

0-1

Neutrophil

63.50

37-80

Lymphocyte

17.70

20-40

Monocyte

12.70

2-8

Neutrophil

10.06

103/L

2.7-6.5

absolute
Lymphocyte

2.80

103/L

1.5-3.7

absolute
Monocyte absolute

2.02

103/L

0.2-0.4

Basophyl absolute

0.06

103/L

0-0.1

MCV

67.60

fL

75-87

MCH

22.90

pg

25-31

MCHC

33.90

g%

33-35

Result
1.81
0.73
204
40
24

Unit
mg/dL
mg/dL
U/L
U/L
U/L

Referal
<1
0-0.2
<300
<38
<41

Clinical chemistry
Test
Total bilirubin
Direct bilirubin
Alcali fosfatase
AST/SGOT
ALT/SGPT
Electrolyte

Test
Natrium
kalium
Chloride

Result
138
3.8
100

Unit
mEq/L
mEq/L
mEq/L

Referal
135-155
3.6-5.5
96-106

Immunoserology Test:
-

ASTO < 200

CRP qualitative (+)

Diagnosis: CHF NYHA I-II ec RHD

Therapy:
-

O2 2-4 litre (if needed)

Inj Ceftriaxone 1gr/12 hrs/IV

Inj Furosemide 25mg/12 hrs/IV

Spironolactone 2x25 mg

Benzatine Penicilin 1,2 million units IM

Further Investigation Plan:

-

ASTO

Electrolyte

Electrocardiography

Echocardiography

Consult Cardiology Department

Complete blood count

Follow Up:
4th July 2015
S
O
A
P
o
Dyspnea (+), Sensorium: alert, T: 37,6 C, CHF NYHA II-III -O2 2 litre/I (if
sore throat (-)

BW: 25 kg BH: 136cm arm ec RHD

needed)

circimflex: 18cm

-inj

Head: eye reflect +/+, conj

1gr/12 hrs,IV

palpebral inferior pale -/-,

- inj furosemide

mouth/nose/ear: normal.

25mg/12 hrs/IV

ceftriaxone

Neck: JVP R+2 H2O

-Spironolactone

Thorax: symetris fusiformis,

2x25mg

retraction (-) HR: 112x/i,

- liquid food diet

systolic murmur (+) grade

2x200cc

IV/6 ICR III-IV linea left

-benzatine

midclavicularis,

penicilin

open

out

ictus and strong lifted. RR:

1,2

million units

24x/i, Ronchi -/-.

Abdomen: Seopel, Normal
peristaltic,

Hepar

palpable

2cm below the costal margin,

Lien: unpalpable.
Extremities:
regular,

Pulse: 112x/i,

adequate

pressure

and volume, warm, CRT <

3, edema pretibial (-), BP :
100/70 mmhg, SaO2 : 98%

5th July 2015

S
O
A
P
o
Dyspnea (-), Sensorium : alert, T : 37,1 C, CHF NYHA II-III -O2 2 litre/I (if
sore throat (-)

BB: 25kg, TB: 136 arm ec RHD

needed)

circumflex : 18cm

-inj

Head : eye reflect +/+, conj

1gr/12 hrs,IV

palpebral inferior pale -/-,

- inj furosemide

mouth/nose/ear: normal.

25mg/12 hrs/IV

Neck : JVP R+ 2cm H2O

-Spironolactone

Thorax: symetris fusiformis,

2x25mg

retraction (-) HR: 110x/i,

- liquid food diet

ceftriaxone

systolic murmur (+) grade

2x200cc

IV/6 ICR III-IV linea left

midclavicularis,

open

out

ictus and strong lifted. RR:

26x/i, Ronchi -/-.
Abdomen: Seopel, Normal
peristaltic,

Hepar

palpable

2cm below the costal margin,

Lien: unpalpable.
Extremities:
adequate

Pulse

110x/i

pressure

and

volume, warm, CRT < 3,

edema pretibial (-), BP :
110/70 mmhg,

6th July 2015

S
Dyspnea(-),

O
A
P
Sensorium : alert, T : 37,1 oC, CHF NYHA II-III -O2 2 litre/I (if

sore throat (-)

BB: 25kg, TB: 136 arm ec RHD

needed)

circumflex : 18cm

-inj

Head : eye reflect +/+, conj

1gr/12 hrs,IV

palpebral inferior pale -/-,

- inj furosemide

mouth/nose/ear: normal.

25mg/12 hrs/IV

Neck : JVP R+ 2cm H20

-Captopril

Thorax: symetris fusiformis,

3x6,25mg

retraction (-) HR: 110x/i,

- liquid food diet

systolic murmur (+) grade

2x200cc

IV/6 ICR III-IV linea left

midclavicularis,

open

out

ceftriaxone

ictus and strong lifted. RR:

24x/i, Ronchi -/-.
Abdomen: Soepel, Normal
peristaltic,

Hepar

palpable

2cm below the costal margin,

Lien: unpalpable.
Extremities:
adequate

Pulse
pressure

110x/i
and

volume, warm, CRT < 3,

edema pretibial (-), BP :
110/70 mmhg,

CHAPTER IV
DISCUSSION

Case

Theory

Patient has a history of sore throat, and -Rheumatic fever develops in some
has been experiencing pain in his joints

children and adolescents following

pahryngitis.
-Based on Jones Criteria which was
revised

in

1992,to

diagnose

Rheumatic fever patient has to have

either 2 major or 1 major 2 minor
symptoms.
Symptoms may include:
-Fever
-Swollen, tender, red and extremely
painful joints--particularly the knees,
ankles, elbows, or wrists
-Nodules over swollen joints
-Red, raised, lattice-like rash, usually
on the chest, back, and abdomen
-Shortness of breath, chest discomfort
-Uncontrolled movements of arms,
Patient had experienced dyspnea

legs, or facial muscles

-Weakness and shortness of breath

Accumulation of extra fluid in body

and in heart and lungs makes it difficult
for the heart to pump out all the blood
leading to constant accumulation of
blood in lungs leading to dyspnea

Based on Chest X-Ray The Patient has - Depending on the cause of heart
Cardiomegaly

failure, the chest radiograph may show

cardiomegaly,

defined

as

cardiothoracic ratio of greater than 0.5

on the posterior film.
The patient treated with furosemide,
- Benzatine Penicilline G is given
Spironolactone, ceftriaxone, Benzatine

to

Penicilin

rheumatic fever
Ceftriaxone is an antibiotic used

prevent

recurrence

of

to treat a wide variety of

bacterial

infection.

medication

is

cephalosporin

This

known

as

antibiotic.

It

works by stopping the growth of

bacteria. This drug may also be
used before dental procedures in
patients

with

certain

heart

condition such as actificial heart

valves

to

prevent

serious

infection after heart(bacterial

-

endocarditis).
A conservative approach to the
treatment of CHF involves fluid
restriction and fluid removal
from

the

body. Furosemide

helps in the removal of fluid

-

from the body

Spironolactone is a selective
antagonist aldosterone used in
CHF treatment.

CHAPTER V
SUMMARY
AT, a 12 years and 6 months years old boy, with a BW 25 kg and a BH 130
cm, was admitted to emergency room in Haji Adam Malik General Hospital
Medan on 3rd July 2015 at 04.00 pm with a complain of fatigue and have been
worsening for 3 months. Patient experienced fatigue while doing daily activities
without exercise. Patient reported paroxysmal nocturnal dyspnea and has been
sleeping with raised pillow to reduce symptom. Patient has been limiting his
normal routine due to shortness of breath. There is no history of such symptom
experienced before. History of fever and sore throat were reported. Patient also
reported multiple joint pain. Patient has been also suffering weight loss for the

past 12 months. Patient was referred from Lubuk Pakam Hospital and was
diagnosed with rheumatic heart disease. Patient has also reported that he had
throat pain a year ago. According to his parents he never had any tiredness during
his childhood and breast feeding. Level of consciousness: alert. Body temperature:
37C, BW: 25 kg, BH: 130 cm. BW/A: 53.4%, BL/A: 89.5%, BW/BL: 78.3%
anemic (-), ikteric (+), dyspnea (+), cyanosis (-), edema (-). Patient treated with
Benzatinr Penicilin, furosemide, spironolactone and captopril.

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