RHEUMATIC HEART DISEASE

Worldwide, rheumatic heart disease remains a major health problem. Chronic

rheumatic heart disease is estimated to occur in 5-30 million children and young
adults; 90,000 individuals die from this disease each year. The mortality rate from
this disease remains 1-10%. A comprehensive resource provided by the World
Health Organization (WHO) addresses the diagnosis and treatment
Definition
Rheumatic heart disease is a condition in which permanent damage to heart valves
is caused by a disease process that generally begins with a strep throat caused by
bacteria called Streptococcus, and may eventually cause rheumatic fever. In the
pediatric age group, the sequelae of rheumatic fever consist of mitral, aortic,
tricuspid valve stenosis.
or
Rheumatic heart disease is the most dreaded complication of rheumatic fever. The term "rheumatic heart
disease" refers to the chronic heart valve damage that can occur after a person has had an episode of acute
rheumatic fever. This valve damage can eventually lead to heart failure.
Epidemiology
Mortality/morbidity
Rheumatic heart disease is the major cause of morbidity from rheumatic fever and the major cause of
mitral insufficiency and stenosis in the United States and the world. Variables that correlate with severity
of valve disease include the number of previous attacks of rheumatic fever, the length of time between the
onset of disease and start of therapy, and sex. (The disease is more severe in females than in males.)
Insufficiency from acute rheumatic valve disease resolves in 60-80% of patients who adhere to antibiotic
prophylaxis.
Race
Native Hawaiian and Maori (both of Polynesian descent) have a higher incidence of rheumatic fever (13.4
per 100,000 hospitalized children per year), even with antibiotic prophylaxis of streptococcal pharyngitis.
Otherwise, race (when controlled for socioeconomic variables) has not been documented to influence
disease incidence.
Sex
Rheumatic fever occurs in equal numbers in males and females, but the prognosis is worse for females
than for males.
Age
Rheumatic fever is principally a disease of childhood, with a median age of 10 years, although it also
occurs in adults (20% of cases).

PATHOPHYSIOLOGY

It is believed that certain proteins produced by the group A Streptococci are structually similar to
those found in the heart. The immune system reacts to the presence of bacteria by producing specific
substances, called antibodies, to attack them and stop their spread inside the body. But as these antibodies
attack the bacteria, some of them also attack or cross-react with the tissues in the heart, and this often
results in the manifestation of many RHD symptoms.

Classification

RHD

Mitral valve

aortic valve

tricuspid valve

Regurgitation

Regurgitation

Regurgitation

stenosis
A. MITRAL REGURGITATION:
A condition which is characterized by a regurgitation of blood from the left ventricle into the atrium due
to a problem with the mitral valve.
or
Backflow of blood from the left ventricle into the left atrium, owing to imperfect functioning of the
mitral valve.

Hemodynamics

Chronic compensated stage

In this stage, the LV compensates by allowing greater diastolic filling and developing LV enlargement to
augment forward stroke volume. More importantly, the left atrium dilates in response to the increased
volume. Compensation for the increased volume can occur without resulting in increased pressure in the

pulmonary circulation and the right heart. Left atrial compliance decreases the afterload on the LV,
whereas LV dilation and hypertrophy increases contractility. These important changes keep the overall
afterload on the left heart normal or unchanged. Although the regurgitant fraction may be high, the larger
stroke volume compensates, maintaining a nearly normal forward cardiac output (see the image below).

Acute mitral regurgitation stage

Acute mitral regurgitation causes sudden volume overload of the left atrium and LV. Initially, the
undilated left atrium restricts the regurgitant volume at the expense of increase in both left atrial and LV
end-diastolic pressures.
Although total ventricular stroke volume increases compared to normal, total forward stroke volume
usually decreases, thereby lowering cardiac output. In the acute situation, rapidly increasing left atrial
pressure results in elevated pulmonary venous pressure causing pulmonary congestion and, eventually,
pulmonary edema (see the image below).

Symptoms:
Children with mild to moderate regurgitation usually do not develop any symptoms
and lead a normal life. Children with more severe leakage may experience
symptoms such as:

Shortness of breath
Difficulty breathing, especially during exertion

 Fatigue
Rapid breathing
tachypnoea
Poor feeding
Delayed growth and failure to thrive
Excessive sweating
Fast heart rate
Palpitations
congestive Heart failure
Heart sounds: Upon auscultation, the first heart sound is usually slightly diminished,
whereas the second heart sound is usually split. With more severe mitral regurgitation, a
third heart sound and a mid diastolic low frequency murmur may be present, caused by
increased ventricular filling.
Murmur: Patients with mild mitral regurgitation may reveal no signs other than a
characteristic apical systolic murmur. The sound of the typical mitral regurgitation murmur
is characterized as blowing and high pitched, and it is loudest over the apex with radiation
to the left axilla. The murmur is often pansystolic, beginning immediately after the first
heart sound, and may continue beyond the aortic component of the second heart sound,
thus obscuring the murmur.

B. MITRAL STENOSIS
Definition
Mitral valve stenosis results from a pathologic process that narrows the effective mitral valve orifice.
Hemodynamics
Mitral stenosis obstruction to blood flow from LA to LV increase LA pressure hypertrophy of LA
increase pulmonary venous pressure pulmonary congestion dyspnoea
Clinical manifestation
Patients with mitral stenosis may present with exertional dyspnea, fatigue, atrial arrhythmias, embolic
events, angina-like chest pain, hemoptysis, or even right-sided heart failure. Previously asymptomatic or
stable patients may decompensate acutely during exercise, emotional stress

The characteristic findings of MS on auscultation are an accentuated first heart sound, an opening snap,
and a mid-diastolic rumble.
C. AORTIC REGURGITATION
Definition : A condition which is characterized by a backward leak from aorta into the left ventricle
during diastole.
Classification

Mild :difference between systolic pressure in brachial artery and femoral artery is
20 mm of hg
Moderate: pressure difference is 20-40 mm of hg
Severe: pressure difference is 40-60 mm of hg

less than

HEMODYNAMICS
Blood volume in LV increase
blood

increase size of LV

impaired forward flow of

Wide pulse pressure

diastolic pressure goes up on prolong time
increase pulmonary congestion

LV pressure

aortic valve insufficiency results in volume overload on the LV because the LV is

forced to pump the entire diastolic volume received from the left atrium and the
regurgitant volume from the aorta through an incompetent aortic valve. Over time,
such volume overload (ie, increased preload) causes eccentric hypertrophy of the
LV. If on prolong time LV myocardial functioning is failing diastolic pressure goes up
and leads to increase LA pressure leading to pulmonary congestion
CLINICAL MANIFESTION
Palpitation, wide pulse pressure, decrease diastolic BP, prominent carotid pulsation (
corrigans sign), visible arterial pulsation, nodding of head may be present with each
systole due to sudden filling of carotid vessel, arteriolar pulsation may be seen over
the nail beds, uvula, lips, ear lobes.
Difference in systolic pressure between brachial and femoral artery. Cardiac
examination reveals enlarge heart with apex displaced downward and outward,

D. TRICUSPID REGURGITATION
Definition: Refers to the failure of the heart's tricuspid valve to close properly
during systole. As a result, with each heart beat some blood passes from the right
ventricle to the right atrium, the opposite of the normal direction.

Hemodynamics
Systolic leak
volume load of RV, RA increase Increase size of RA, RV
diaplace downward and outward

RA, RV

Clinical features
Systolic and diastolic murmur loud during inspiration, pain right hypochondrium,
fatigue, dyspnoea, pulmonary arterial hypertension, enlarged RA and RV displaced
downward, prominent V waves in the jugular venous pulse, systolic pulsation of
liver, systolic murmur in lower left sternal border increasing intensity with inspiraton

DIAGNOSIS Of RHD

History
Physical examination
Vital
Cardiac examination
Laboratory Studies

Throat

signs

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.
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. 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.
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. Sensitivity for recent infections can be improved by testing for several antibodies. Antibody
titers should be checked at 2-week intervals in order to detect a rising titer.

Imaging Studies

Chest roentgenography
Cardiomegaly, pulmonary congestion, and other findings consistent with heart failure may be seen on
chest radiography. When the patient has fever and respiratory distress, chest radiography helps
differentiate heart failure from rheumatic pneumonia.
Doppler-echocardiogram
In acute rheumatic heart disease, Doppler-echocardiography identifies and quantitates valve insufficiency
and
ventricular
dysfunction.

Other Tests

On ECG, sinus tachycardia most frequently accompanies acute rheumatic heart disease.

Histologic Findings

Pathologic examination of the insufficient valves may reveal verrucous lesions at the line of closure.

Procedures used for diagnosis

Exercise stress testing

o Exercise stress testing can usually be performed in children aged 6 years or older and is
helpful in eliciting symptoms that may not be evident from routine history. Doppler studies
can be helpful in determining whether exercise restrictions are necessary by measuring the
change in aortic valve gradient from rest to immediately after maximal exercise.
o Exercise stress testing may also provide some risk stratification if intervention is delayed
or contemplated. Factors such as heart rate, blood pressure response to exercise (blunted),
exercise duration (reduced), provocable arrhythmias (ventricular ectopy of left ventricular
origin) or ECG ischemic changes, and measured oxygen consumption provide useful data
on which to base decisions regarding timing of intervention.

Cardiac catheterization
o Cardiac catheterization is usually performed in infants, children, and older adolescents in
anticipation of balloon aortic valvuloplasty.
o Other indications for catheterization may include the need for accurate hemodynamic
assessment in patients with multiple levels of obstruction, such as mitral stenosis or
subaortic stenosis in combination with aortic valve stenosis.

MEDICAL MANAGEMENT

Medications: Medications do not rectify the valves but treat the complications and reduce the
consequences. Medications cannot make up the mechanical problems due to narrowing of valves
especially in stenotic cases which essentially require surgery. Medications are generally effective in
valvular regurgitation cases.

Prophylactic penicillin for prevention of recurrence of rheumatic fever. Penicillin is an antibiotic

that prevents the bacteria from forming a stable cell wall. Without this cell wall the bacteria are
unable to reproduce and the patient's immune system can destroy the infection.
Penicillin G and penicillin V. erythromycin in case of penicillin sensitive patient. An injection of
0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks.penicillin V 250mg
BD or TDS. Benzyl penicillin G 600000 units IM single dose for wt <27 kg and 1.2 million for wt
>27 kg.

1. Angiotensin Converting Enzyme (ACE) Inhibitors: These are used to widen blood vessels, lower
blood pressure and decrease the workload on heart (in valvular regurgitation). Eg ramipril, 1.5mg/day
2. Antiarrhythmics: These maintain a regular heartbeat and reduce the rapid heart rhythms. Thus, the
heart beats less frequently but pumps blood more throughout the body . Inj atropine
Age
(years)
2
3
4
5
6-8
9-12

Dosage in ml (Four Times Daily)

Approximate (kg)
11-14
12-16
14-20
16-23
17-32
23-55

1.5-3.0
2.0-3.0
2.0-4.0
2.5-4.5
2.5-5.0
3.5-5.0

3. Anticoagulants: Valvular problems can lead to blood clots. Anticoagulants help prevent clot
formation inside the heart chambers or on a damaged heart valve. Eg aspirin 3-5 mg/kg/day
4. Antibiotics: Antibiotics are given to the patients suffering from valvular heart disease before any dental
procedure, surgery and IV drug to prevent bacteria from sticking to abnormal heart valves. Amoxicillin 50
mg/kg orally 1 h before procedure. Ampicillin 50 mg/kg I.M./I.V. within 30 min before procedure
5. Beta-blockers: Beta-blockers slow the heart rate so the work of heart is made easier. Eg propanolol 2-4
mg/kg/day in two devided doses orally.
9. Digitalis: Digitalis is prescribed for treatment of arrhythmias, particularly atrial fibrillation. This drug
increases the force of the heart muscles contraction and is helpful if a person has suffered congestive
heart failure. Eg: digoxin 2030 g/kg IV; give 1/2 the initial dose, then 1/3 of dose at 812 h.
10. Diuretics: These drugs help the body rid of excess fluid and salt. Diuretics also reduce swelling and
ease the workload on heart. Eg thiazide 2mg/kg/day BD and Infants under 6 months of age may require
up to 3 mg/kg per day, in 2 divided doses.
Surgical management

Valve repair: This is an open heart surgery in which the surgeon cuts in to repair the damaged
valve. One such surgery is commissurotomy used to rectify mitral stenosis. In this procedure the
narrowed valve leaflets are widened by opening the fused leaflets or commissures with a scalpel.

Prosthetic valve replacement

Commissurotomy or vulvotomy

Percutaneous Balloon Valvuloplasty: This is a nonsurgical catheter based procedure to treat

valvular stenosis. In this procedure, a catheter is threaded up to the heart through an artery in the
groin and guided through the narrowed valve. At the point of narrowed valve, the balloon is gently
inflated to stretch the valve. Once the valve is widened, the balloon is deflated and removed.

Diet
The diet 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.
Activity
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.
Nursing management
A. Assessment
The objective assessment is to collect data on :

Cardiac function

Tolerance to client activity and attitudes toward restrictions on activities

Nutritional Status

Discomfort level

Sleep disruption

Client's ability to overcome problems

The things that can help clients

Knowledge of parents and patients (according to the patient's age) of patient understanding.
Assessment :

Hospital chart

Monitor cardiac complications

Cardiac auscultation, heart sounds with the rhythm of marching weakened diastole

Vital signs

The presence of pain

The existence of joint inflammation

The presence of skin lesions

Nursing Diagnosis :

1.

Reduced cardiac output associated with valve stenosis

goal :

Clients shows a decrease dyspnea

Participating in activities and demonstrate increased tolerance

Intervention :

Monitor blood pressure, pulse apical and peripheral pulse

Monitor cardiac rhythm and the frequency

Sleeping position 450 semifowler

Instruct the client to do stress management techniques (quiet environment, meditation)

Aids client activity as indicated when the client is able

Collaboration and providing oxygenation therapy

2.

Activity intolerance related to decreased cardiac output, oxygenation supply and demand
imbalance
goal :

Verbal Response fatigue is reduced

Carrying out activities within the limits of his ability (pulse activity can not be greater than
90 X / minute, no chest pain)
Intervention :

Energy saving clients during the acute

Maintain a sleep until the results of laboratory and clinical status improved

In line with the better situation, monitor the gradual increase in the level of activity

Create a schedule of activity and rest

Teach to participate in activities.

Teach the children / parents who do not realize that the movement is connected with the
Korean and temporary.
In case of chorea, protect from accidents, bedrest and provide appropriate sedation

program.