sistemik - pedo

8/3/2019 sistemik - pedo

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Congenital heart disease

Congenital heart disease refers to a problem with the heart's structure and function

due to abnormal heart development before birth. Congenital means present at

birth.

Causes

Congenital heart disease (CHD) can describe a number of different problems

affecting the heart. It is the most common type of birth defect. Congenital heart

disease is responsible for more deaths in the first year of life than any other birth

defects. Many of these defects need to be followed carefully. Some heal overtime, others will require treatment.

Congenital heart disease is often divided into two types: cyanotic (blue

discoloration caused by a relative lack of oxygen) and non-cyanotic. The

following lists cover the most common of the congenital heart diseases:

Cyanotic:

Tetralogy of Fallot

Transposition of the great vessels

Tricuspid atresia Total anomalous pulmonary venous return

Truncus arteriosus

Hypoplastic left heart

Pulmonary atresia

Some forms of total anomalous pulmonary venous return

Ebstein's anomaly

Non-cyanotic:

Ventricular septal defect (VSD)

Atrial septal defect (ASD) Patent ductus arteriosus (PDA)

Aortic stenosis

Pulmonic stenosis

Coarctation of the aorta

Atrioventricular canal (endocardial cushion defect)

These problems may occur alone or together. The majority of congenital heart

diseases occurs as an isolated defect and is not associated with other diseases.

However, they can also be a part of various genetic and chromosomal syndromes

such as Down syndrome,trisomy 13, Turner syndrome, Marfan syndrome,

Noonan syndrome, and DiGeorge syndrome.
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No known cause can be identified for most congenital heart defects. Congenital

heart diseases continue to be investigated and researched. Drugs such as retinoic

acid for acne, chemicals, alcohol, and infections (such asrubella) during

pregnancy can contribute to some congenital heart problems.

Symptoms

Symptoms depend on the specific condition. While congenital heart disease is

present at birth, the symptoms may not be immediately obvious. Defects such as

coarctation of the aorta may not cause problems for many years. Other problems,

such as a small ventricular septal defect(VSD), may never cause any problems,

and some people with a VSD have normal physical activity and a normal life

span.

Prevention

Avoid alcohol and other drugs during pregnancy. Doctors should be made aware

that a woman is pregnant before prescribing any medications for her. A blood test

should be done early in the pregnancy to see if the woman is immune to rubella. If

the mother is not immune, she must avoid any possible exposure to rubella and

should be immunized immediately following delivery.

Poorly controlled blood sugar levels in women who have diabetes during

pregnancy are also associated with a high rate of congenital heart defects during

pregnancy.

Experts believe that some prescription and over-the-counter medications and

street drugs used during pregnancy increase the risk of heart defects.

There may be some hereditary factors that play a role in congenital heart disease.

Genetics does appear to play a role in many diseases, and multiple family

members may be affected. Talk to your health care provider about screening.

Expectant mothers should receive good prenatal care. Many congenital defects

can be discovered on routine ultrasound examinations performed by an

obstetrician. The delivery can then be anticipated and the appropriate medicalpersonnel (such as a pediatric cardiologist, a cardiothoracic surgeon, and a

neonatologist) can be present, and ready to help as necessary. Such preparation

can mean the difference between life and death for some babies.
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Rheumatic fever (RF) is a systemic illness that may occur following group A beta

hemolytic streptococcal (GABHS)pharyngitis in children. Rheumatic fever and

its most serious complication, rheumatic heart disease (RHD), are believed to

result from an autoimmune response; however, the exact pathogenesis remainsunclear. Studies in the 1950s during an epidemic on a military base demonstrated

3% incidence of rheumatic fever in adults with streptococcal pharyngitis not

treated with antibiotics.[1] Studies in children during the same period demonstrated

an incidence of only 0.3%. The current incidence of rheumatic fever after

GABHS infection is now thought to have decreased to less than 1%. Cardiac

involvement is reported to occur in 30-70% of patients with their first attack of

rheumatic fever and in 73-90% of patients when all attacks are counted.

Clinical manifestations and time course of acute rheumatic fever are shown in the

image below.

Pathophysiology

Rheumatic fever develops in children and adolescents following pharyngitis with

GABHS (ie, Streptococcus pyogenes). The organisms attach to the epithelial cells

of the upper respiratory tract and produce a battery of enzymes, which allows

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 elevated leukocyte count. In a small

percent of patients, infection leads to rheumatic fever several weeks after the sore

throat has resolved. Only infections of the pharynx initiate or reactivate rheumaticfever.

Direct contact with oral (PO) or respiratory secretions transmits the organism, and

crowding enhances transmission. 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 prevents the major sequelae.

GABHS organisms are gram-positive cocci, which frequently colonize the skin

and oropharynx. These organisms may cause suppurative diseases (eg,

pharyngitis, impetigo, cellulitis, myositis,pneumonia, puerperal sepsis). GABHS
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organisms also may be associated with nonsuppurative diseases (eg, rheumatic

fever, acute poststreptococcal glomerulonephritis). Group A streptococci (GAS)

elaborate the cytolytic toxins, streptolysins S and O. Of these 2 toxins,

streptolysin O induces persistently high antibody titers that provide a useful

marker of GAS infection and its nonsuppurative complications.

GAS, 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. Surface proteins on the cell wall of the

organism may subserotype GAS. The presence of the M protein is the most

important virulence factor for GAS infection in humans. More than 120 M protein

serotypes or M protein genotypes have been identified,[20] some of which have a

long terminal antigenic domain (ie, epitopes) similar to antigens in various

components of the human heart.

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). Currently, emm

typing is felt to be more discriminating than M typing.[20]

Acute RHD often produces a pancarditis, characterized by endocarditis,

myocarditis, and pericarditis. Endocarditis is manifested as mitral and aortic valve

insufficiency. Severe scarring of the valves develops during a period of months to

years after an episode of acute rheumatic fever, and recurrent episodes may cause

progressive damage to the valves. The mitral valve is affected most commonly

and severely (65-70% of patients); the aortic valve is affected second most

commonly (25%).

The tricuspid valve is deformed in only 10% of patients, almost always in

association with mitral and aortic lesions, and the pulmonary valve is rarely

affected. Severe valve insufficiency during the acute phase may result in

congestive heart failure (CHF) and even death (1% of patients). Whether

myocardial dysfunction during acute rheumatic fever is primarily related to

myocarditis or is secondary to CHF from severe valve insufficiency is not known.

When pericarditis is present, it rarely affects cardiac function or results in

constrictive pericarditis.

Chronic manifestations occur in adults with previous RHD from residual and

progressive valve deformity. RHD is responsible for 99% of mitral valve stenosis

in adults, and it may be associated with atrial fibrillation from chronic mitral

valve disease and atrial enlargement.

Epidemiology


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Frequency

United States

Rheumatic fever is now uncommon among children in the United States.Incidence of rheumatic fever and RHD has decreased in the United States and

other industrialized countries during the past 80 years. Prevalence of RHD in the

United States is now less than 0.05 per 1000 population, with rare regional

outbreaks reported in Tennessee in the 1960s and in Utah, Ohio, and Pennsylvania

in the 1980s. In the early 1900s, incidence was reportedly 5-10 cases per 1000

population. Decreased incidence of rheumatic fever has been attributed to the

introduction of penicillin or a change in the virulence of the streptococci.

International

In contrast to trends in the United States, rheumatic fever and RHD have not

decreased in developing countries. Retrospective studies in developing countries

demonstrate the highest figures for cardiac involvement and the highest

recurrence rates of rheumatic fever. Worldwide, an estimated 5-30 million

children and young adults have chronic RHD, and 90,000 patients die from this

disease each year.

A study using echocardiographic screening in schoolchildren in Cambodia and

Mozambique suggests that RHD prevalence may be as much as 10 times that

detected using clinical examination with echocardiographic verification.[2]

Mortality/Morbidity

RHD is the major cause of morbidity from rheumatic fever and is 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 prognosis for females is worse than for males).

Insufficiency from acute rheumatic valve disease resolves in 70-80% of patients if

they adhere to antibiotic prophylaxis.

Race

Native Hawaiians and Maori (both of Polynesian descent) have a higher incidence

of rheumatic fever. Incidence of rheumatic fever in these patients is 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 the disease incidence.


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Sex

Rheumatic fever occurs in equal numbers in males and females. Females with

rheumatic fever fare worse than males and have a slightly higher incidence of

chorea.

Age

Rheumatic fever is principally a disease of childhood, with a median age of 10

years; However, GABHS pharyngitis is uncommon in children younger than 3

years, and acute rheumatic fever is extremely rare in these younger children in

industrialized countries. Although less commonly seen in adults compared with

children, rheumatic fever in adults accounts for 20% of cases.

HistoryAcute rheumatic fever (RF) is a systemic disease. Thus, patients may present with

a large variety of symptoms and complaints.

History of an antecedent sore throat 1-5 weeks prior to onset is present in

70% of older children and young adults. Only 20% of younger children

can recall an antecedent sore throat.

Other symptoms on presentation may include fever, rash, headache,

weight loss, epistaxis, fatigue, malaise, diaphoresis, and pallor.

Patients also may have chest pain with orthopnea or abdominal pain and

vomiting. Finally, history may reveal symptoms more specific to rheumatic fever.

o Migratory joint pain

o Nodules under the skin

o Increased irritability and shortened attention span with personality

changes, such as pediatric autoimmune neuropsychiatric disorder

associated with streptococcal infections (PANDAS)

o Motor dysfunction

o History of previous rheumatic fever

Patients with previous rheumatic fever are at a high risk of recurrence.

o

Highest risk of recurrence within 5 years of the initial episodeo Greater risk of recurrence with younger age at the time of the

initial episode

o Generally, recurrent attacks similar to the initial attack (however,

risk of carditis and severity of valve damage increase with each

attack)

Physical

Revised in 1992, the modified Jones criteria provide guidelines for making the

diagnosis of rheumatic fever.[3] The Jones criteria require the presence of 2 major

or 1 major and 2 minor criteria for the diagnosis of rheumatic fever. Having


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evidence of previous group A streptococci (GAS) pharyngitis is also necessary.

These criteria are not absolute, and the diagnosis of rheumatic fever can be made

in patients with only confirmed streptococcal pharyngitis and chorea.

Major diagnostic criteriao Carditis

o Polyarthritis

o Chorea

o Subcutaneous nodules

o Erythema marginatum

Minor diagnostic criteria

o Fever

o Arthralgia

o Prolonged PR interval on electrocardiography

o Elevated acute-phase reactants (APRs), which are erythrocyte

sedimentation rate and C-reactive protein

Three notable exceptions to strict adherence to the Jones criteria

o Chorea: It may occur late and be the only manifestation of

rheumatic fever.

o Indolent carditis: Patients presenting late to medical attention

months after the onset of rheumatic fever may have insufficient

support to fulfill the criteria.

o Newly ill patients with a history of rheumatic fever, especially

rheumatic heart disease (RHD), who have supporting evidence of a

recent GAS infection and who manifest either a single major or

several minor criteria: Distinguishing recurrent carditis frompreexisting significant RHD may be impossible.

Evidence of previous GAS pharyngitis (One of the following must be

present):

o Positive throat culture or rapid streptococcal antigen test

o Elevated or rising streptococcal antibody titer

Major clinical manifestations

o Arthritis

Polyarthritis is the most common symptom and is

frequently the earliest manifestation of acute rheumatic

fever (70-75%).

Characteristically, the arthritis begins in the large joints ofthe lower extremities (ie, knees, ankles) and migrates to

other large joints in the lower or upper extremities (ie,

elbows, wrists).

Affected joints are painful, swollen, warm, erythematous,

and limited in their range of motion. The pain is out of

proportion to clinical findings.

The arthritis reaches maximum severity in 12-24 hours and

persists for 2-6 days (rarely more than 4 wk, but has been

reported to persist 44 d) at each site and is migratory but

not additive.


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The arthritis responds rapidly to aspirin, which decreases

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. Patients suffering multiple attacks may exhibit destructive

arthritis (Jaccoud arthritis).

o Carditis

Pancarditis is the most serious complication and the second

most common complication of rheumatic fever (50%).

In advanced cases, patients may experience of dyspnea,

mild-to-moderate chest discomfort, pleuritic chest pain,

edema, cough, or orthopnea.

Upon physical examination, carditis is most commonly

revealed by a new murmur and tachycardia that is out of

proportion to the fever. New or changing murmurstraditionally have been considered necessary for a diagnosis

of rheumatic valvulitis. The murmurs of acute rheumatic

fever are from valve regurgitation, and the murmurs of

chronic rheumatic fever are from valve stenosis.

Frequently examine patients in whom the diagnosis of

acute rheumatic fever is made due to the progressive nature

of the disease. Some cardiologists have proposed that

evidence of new mitral regurgitation from Doppler

echocardiography, even in the absence of accompanying

auscultatory findings, may be sufficient for making the

diagnosis of carditis, particularly if the echocardiography

findings resolve along with other manifestations of

rheumatic fever. This criterion for carditis is not uniformly

accepted and remains specifically excluded in the 1992

revised Jones criteria because of insufficient data at the

time of publication.

Congestive heart failure (CHF) may develop secondary to

severe valve insufficiency or myocarditis. Physical findings

associated with heart failure include tachypnea, orthopnea,

jugular venous distention, rales, hepatomegaly, a gallop

rhythm, and peripheral swelling and edema. A pericardialfriction rub indicates that pericarditis is present. Increased

cardiac dullness to percussion, muffled heart sounds, and a

paradoxical pulse are consistent with pericardial effusion

and impending pericardial tamponade. Confirm this clinical

emergency with ECG, and evacuate the effusion by

pericardiocentesis if it is producing hemodynamic

compromise.

o Chorea: In the absence of a family history of Huntington chorea or

findings consistent with systemic lupus erythematosus, the

diagnosis of acute rheumatic fever is almost certain. A long latency

period exists between streptococcal pharyngitis (1-6 mo) and the


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onset of chorea, and a history of an antecedent sore throat

frequently is not obtained. Patients with chorea often do not

demonstrate other Jones criteria. Chorea is slightly more common

in females than males. Chorea is also known as rheumatic chorea,

Sydenham chorea, chorea minor, and St Vitus dance.o Poststreptococcal movement disorders

Described poststreptococcal movement disorders have

included pediatric autoimmune neuropsychiatric disorder

associated with streptococcal infections (PANDAS) and

Tourette syndrome.

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, incidents

have been reported in which symptoms wax and wane for

several years. The PANDAS disorder appears to have a relapsing-

remitting symptom complex characterized by obsessive-

compulsive personality disorder. Patients with Sydenham

chorea and obsessive-compulsive symptoms tend to show

aggressive, contamination, and somatic obsessions and

checking, cleaning, and repeating compulsions. Neurologic

abnormalities include cognitive defects and motoric

hyperactivity. The symptoms may also include emotional

lability, separation anxiety, and oppositional behaviors, and

they are prepubertal in onset.

Some have proposed that the streptococcal infection

triggers 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

and causes the symptom complex.

o Erythema marginatum: This characteristic rash, also known as

erythema annulare, occurs in 5-13% of patients with acute

rheumatic fever. Erythema marginatum begins as 1-cm to 3-cm

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 raisedmargins and central clearing. The rash may fade and reappear

within hours and is exacerbated by heat. Thus, if the lesions are not

observed easily, 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. Erythema marginatum (shown in the

image below) has also been reported in association with sepsis,

drug reactions, and glomerulonephritis.


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Erythema marginatum, the

characteristic rash of acute rheumatic fever.

o Subcutaneous nodules: Subcutaneous nodules are now an

infrequent manifestation of rheumatic fever. The frequency has

declined during 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, scalp, and

spinous processes of the lumbar and thoracic vertebrae (attached to

the tendon sheath). The nodules are firm, nontender, and free from

attachments to the overlying skin, and they range from a few

millimeters to 1-2 cm. The nodules number from 1 to dozens, with

a mean of 3-4. Histologically, the nodules contain areas resembling

the Aschoff bodies observed in the heart. Subcutaneous nodules

generally occur several weeks into the disease and resolve within a

month. They are strongly associated with severe rheumatic carditis,

and in the absence of carditis, question the diagnosis of

subcutaneous nodules.

Other clinical manifestations

o Abdominal pain: Abdominal pain usually occurs at the onset of

acute rheumatic fever, resembles other conditions with acutemicrovascular mesenteric inflammation, and may mimic acute

appendicitis.

o Arthralgias: Patients may report arthralgias upon presentation. In

the history, determining if the patient has taken aspirin or

nonsteroidal anti-inflammatory drugs (NSAIDs) is important

because these may suppress the full manifestations of the disease.

Arthralgia cannot be considered a minor manifestation if arthritis is

present.

o Epistaxis: Epistaxis may be associated with severe protracted

rheumatic carditis.

o Fever: Fevers greater than 39C with no characteristic pattern arepresent initially in almost every patient with acute rheumatic fever.

The fever may be low grade (38-38.5C) in children with mild

carditis or absent in patients with pure chorea. The fever decreases

without antipyretic therapy in approximately 1 week, but low-

grade fevers persist for 2-3 weeks.

o Rheumatic pneumonia: Patients present with the same signs as an

infectious pneumonia. Differentiate rheumatic pneumonia from

respiratory distress related to CHF.

Causes
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Rheumatic fever is believed to result from an autoimmune response; however, the

exact pathogenesis remains unclear.

Rheumatic fever only develops in children and adolescents following

group A beta hemolytic streptococcal (GABHS) pharyngitis, and onlyinfections of the pharynx initiate or reactivate rheumatic fever.

At least some rheumatogenic strains of GAS have antigenic domains

similar to antigens in components of the human heart, and some authors

have proposed that anti-M antibodies against the streptococci may cross-

react with heart tissue, causing the pancarditis that is observed in

rheumatic fever. So-called molecular mimicry between streptococcal and

human proteins is felt to involve both the B and T cells of peripheral

blood, with infiltration of the heart by T cells. Some believe that an

increased production of inflammatory cytokines is the final mechanism of

the autoimmune reaction that causes damage to cardiac tissue in RHD. An

insufficiency of interleukin-4 (IL-4)producing cells in the valve tissuemay also contribute to the valve lesions.

Streptococcal antigens, which are structurally similar to those in the heart,

include hyaluronate in the bacterial capsule, cell wall polysaccharides

(similar to glycoproteins in heart valves), and membrane antigens that

share epitopes with the sarcolemma and smooth muscle.

Medical Care

Prevention of rheumatic fever in patients with group A beta

hemolytic streptococci (GABHS) pharyngitis

For patients with GABHS pharyngitis, a meta-analysis supported a protective

effect against rheumatic fever (RF) when penicillin is used following the

diagnosis.[4]

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.

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 penicillin-allergic patients

are also allergic to cephalosporins.

Do not use tetracyclines and sulfonamides to treat GABHS pharyngitis.

For recurrent group A streptococci (GAS) pharyngitis, a second 10-day

course of the same antibiotic may be repeated. Alternate drugs include

narrow-spectrum cephalosporins, amoxicillin-clavulanate, dicloxacillin,

erythromycin, or other macrolides.

Control measures for patients with GABHS pharyngitis are as follows:


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o 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.

o Exposed persons: People in contact with patients havingdocumented 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.

o School and childcare centers: Children with GABHS infection

should not attend school or childcare centers for the first 24 hours

after initiating antimicrobial therapy.

GABHS carriage is difficult to eradicate with conventional penicillin

therapy. Thus, PO clindamycin (20 mg/kg/d PO in 3 divided doses for 10

d) is recommended.

In general, antimicrobial therapy is not indicated for pharyngeal carriers ofGABHS. Exceptions include the following:

o Outbreaks of rheumatic fever or poststreptococcal

glomerulonephritis

o Family history of rheumatic fever

o During outbreaks of GAS pharyngitis in a closed community

o When tonsillectomy is considered for chronic GABHS carriage

o When multiple episodes of documented GABHS pharyngitis occur

within a family despite appropriate therapy

o Following GAS toxic shock syndrome or necrotizing fasciitis in a

household contact

Treatment for patients with rheumatic fever

Therapy is directed towards eliminating the GABHS pharyngitis (if still present),

suppressing inflammation from the autoimmune response, and providing

supportive treatment of congestive heart failure (CHF).

Treat residual GABHS pharyngitis as outlined above, if still present.

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 typically is dramatic.

o If rapid improvement is not observed after 24-36 hours of therapy,

question the diagnosis of rheumatic fever.

o 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.


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o Anti-inflammatory doses of aspirin may be associated with

abnormal liver function tests and GI toxicity, and adjusting the

aspirin dosage may be necessary.

o When discontinuing therapy, withdraw aspirin gradually over

weeks while monitoring the APRs for evidence of rebound. Choreamost frequently is self-limited but may be alleviated or partially

controlled with phenobarbital or diazepam.

If moderate to severe carditis is present as indicated by cardiomegaly,

third-degree heart block, or CHF, add PO prednisone to salicylate therapy.

o Continue prednisone for 2-6 weeks depending on the severity of

the carditis, and taper prednisone during the last week of therapy.

o Discontinuing prednisone therapy after 2-4 weeks, while

maintaining salicylates for an additional 2-4 weeks, can minimize

adverse effects.

Include digoxin and diuretics, afterload reduction, supplemental oxygen,

bed rest, and sodium and fluid restriction as additional treatment forpatients with acute rheumatic fever and CHF. The diuretics most

commonly used in conjunction with digoxin for children with CHF

include furosemide and spironolactone.

o Initiate digoxin only after checking electrolytes and correcting

abnormalities in serum potassium.

o The total loading dose is 20-30 mcg/kg PO every day, with 50% of

the dose administered initially, followed by 25% of the dose 8

hours and 16 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, andthe 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 (ie, 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 worsens during the acute phase after

aggressive medical therapy, surgery is indicated to decrease valve

insufficiency.

Treatment for patients following rheumatic heart disease (RHD)

Preventive and prophylactic therapy is indicated after rheumatic fever and RHD 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 RHD).

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


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weeks in areas where rheumatic fever is endemic, in patients with residual

carditis, and in high-risk patients.

o 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 administeredparentally.

o 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, continue 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,

whichever is longer.[5] Patients with rheumatic fever with carditis

but no valve disease should receive prophylactic antibiotics for 10years or well into adulthood, whichever is longer. Finally, patients

with rheumatic fever with carditis and valve disease should receive

antibiotics at least 10 years or until aged 40 years.

o Patients with RHD 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). Additional guidelines for endocarditis prophylaxis in

patients who are allergic to penicillin or who are unable to receive

PO antibiotics are discussed in the Bacterial Endocarditis article.

o A recent study investigated the difference in clinical manifestations

and outcomes between first episode and recurrent rheumatic fever.[6] The study concluded that subclinical carditis occurred only in

patients experiencing the first episode, and that all deaths occurredin patients with recurrent rheumatic fever, emphasizing the need

for secondary prophylaxis.

Diet

Advise nutritious diet without restrictions except in patients with CHF, who

should follow a fluid-restricted and sodium-restricted diet. Potassium

supplementation may be necessary because of the mineralocorticoid effect of

corticosteroid and the diuretics, if used.
http://emedicine.medscape.com/article/896540-overviewhttp://emedicine.medscape.com/article/896540-overview


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Medication Summary

Treatment and prevention of group A streptococci (GAS) pharyngitis outlined

here are based on the current recommendations of the Committee on Infectious

Disease (American Academy of Pediatrics). Medical therapy is directed toward

elimination of GAS pharyngitis (if still present), suppression of inflammation

from the autoimmune response, and supportive treatment of congestive heart

failure (CHF). Attempts are being made to produce vaccines against GAS

infection, but the vaccines will not be available for years.

Antibiotics for endocarditis prophylaxis are administered to patients with certain

cardiac conditions, such as carditis caused by rheumatic fever, before procedures

that may causebacteremia are performed. For more information, see Antibiotic

Prophylactic Regimens for Endocarditis.

Antibiotics

Class Summary

The roles for antibiotics are to (1) initially treat GABHS pharyngitis, (2) prevent

recurrent streptococcal pharyngitis, rheumatic fever (RF), and rheumatic heart

disease (RHD), and (3) provide prophylaxis against bacterial endocarditis.

View full drug information

Penicillin VK (Beepen-VK, Pen.Vee K, V-Cillin K, Veetids)

DOC for treatment of GABHS pharyngitis. Although ampicillin or amoxicillin

may be used instead, they have no microbiologic advantage. Do not use

tetracyclines and sulfonamides to treat GABHS pharyngitis. For recurrent

GABHS pharyngitis, a second 10-d course of same antibiotic may be repeated.

Alternate drugs include narrow-spectrum cephalosporins, amoxicillin-clavulanate,

dicloxacillin, erythromycin, or other macrolides.

Penicillin benzathine (Bicillin L-A) or penicillin procaine(Crysticillin A.S., Wycillin)

Used when PO administration of penicillin is not feasible or dependable. IM

therapy with penicillin is painful, but discomfort may be minimized if penicillin G

is brought to room temperature before injection or combination of benzathine

penicillin G and procaine penicillin G is used. Initial course of antibiotics

administered to eradicate streptococcal infection also serves as first course of

prophylaxis. An injection of benzathine penicillin G IM q4wk is recommended

regimen for secondary prevention for most United States patients. Administer
http://emedicine.medscape.com/article/961169-overviewhttp://web.archive.org/web/20060209093103/http/master.emedicine.com/drugs/Endocarditis_Prophylaxis.htmhttp://web.archive.org/web/20060209093103/http/master.emedicine.com/drugs/Endocarditis_Prophylaxis.htmhttp://reference.medscape.com/drug/pen-vee-k-penicillin-v-penicillin-vk-342483#1http://reference.medscape.com/drug/pen-vee-k-penicillin-v-penicillin-vk-342483#1http://emedicine.medscape.com/article/961169-overviewhttp://web.archive.org/web/20060209093103/http/master.emedicine.com/drugs/Endocarditis_Prophylaxis.htmhttp://web.archive.org/web/20060209093103/http/master.emedicine.com/drugs/Endocarditis_Prophylaxis.htmhttp://reference.medscape.com/drug/pen-vee-k-penicillin-v-penicillin-vk-342483#1http://reference.medscape.com/drug/pen-vee-k-penicillin-v-penicillin-vk-342483#1


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same dosage q3wk in areas where RF is endemic, in patients with residual

carditis, and in high-risk patients.


Erythromycin (E.E.S., E-Mycin, Eryc, Ery-Tab, Erythrocin)

Used for patients who are allergic to penicillin. Other options include

clarithromycin, azithromycin, or a narrow-spectrum cephalosporin (ie,

cephalexin). As many as 15% of penicillin-allergic patients are also allergic to

cephalosporins.


Clarithromycin (Biaxin)

Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin.


Azithromycin (Zithromax)



Cephalexin (Keflex, Biocef, Keftab)



Amoxicillin (Amoxil, Biomox, Trimox)

DOC used for bacterial endocarditis prophylaxis. Administered as single PO dose

1 h before dental work or surgery.

Anti-inflammatory agents
http://reference.medscape.com/drug/ees-eryped-erythromycin-ethylsuccinate-999596#1http://reference.medscape.com/drug/ees-eryped-erythromycin-ethylsuccinate-999596#1http://reference.medscape.com/drug/biaxin-xl-clarithromycin-342524#1http://reference.medscape.com/drug/biaxin-xl-clarithromycin-342524#1http://reference.medscape.com/drug/zithromax-azithromycin-342523#1http://reference.medscape.com/drug/zithromax-azithromycin-342523#1http://reference.medscape.com/drug/keflex-cephalexin-342490#1http://reference.medscape.com/drug/keflex-cephalexin-342490#1http://reference.medscape.com/drug/amoxil-moxatag-amoxicillin-342473#1http://reference.medscape.com/drug/amoxil-moxatag-amoxicillin-342473#1http://reference.medscape.com/drug/ees-eryped-erythromycin-ethylsuccinate-999596#1http://reference.medscape.com/drug/ees-eryped-erythromycin-ethylsuccinate-999596#1http://reference.medscape.com/drug/biaxin-xl-clarithromycin-342524#1http://reference.medscape.com/drug/biaxin-xl-clarithromycin-342524#1http://reference.medscape.com/drug/zithromax-azithromycin-342523#1http://reference.medscape.com/drug/zithromax-azithromycin-342523#1http://reference.medscape.com/drug/keflex-cephalexin-342490#1http://reference.medscape.com/drug/keflex-cephalexin-342490#1http://reference.medscape.com/drug/amoxil-moxatag-amoxicillin-342473#1http://reference.medscape.com/drug/amoxil-moxatag-amoxicillin-342473#1


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Class Summary

Manifestations of acute rheumatic fever (including carditis) typically respond

rapidly to therapy with anti-inflammatory agents. Aspirin, in anti-inflammatory

doses, is DOC. Prednisone is added when evidence of worsening carditis andheart failure is noted.


Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Begin administration immediately after diagnosis of RF. Initiation of therapy may

mask manifestations of disease.


Prednisone (Deltasone, Orasone)

If moderate-to-severe carditis is present as indicated by cardiomegaly, CHF, or

third-degree heart block, use 2 mg/kg/d PO prednisone in addition to or in lieu of

salicylate therapy. Continue prednisone for 2-4 wk depending on severity of

carditis and taper during last week of therapy. Discontinuing prednisone therapy

after 2 wk while adding or maintaining salicylates for additional 2-4 wk may

minimize adverse effects.

Therapy for congestive heart failure

Class Summary

Heart failure in RHD probably is related in part to severe insufficiency of the

mitral and aortic valves and in part to pancarditis. Therapy traditionally has

consisted of an inotropic agent (digitalis) in combination with diuretics

(furosemide, spironolactone) and afterload reduction (captopril).


Digoxin (Lanoxin, Lanoxicaps)

Inotropic agent widely used in past. Its efficacy in CHF is under review. Potential

for toxicity is present. Therapeutic levels and clinical effects are observed more

quickly if loading doses of digitalis are administered before routine maintenance

doses. Acts directly on cardiac muscle, increasing myocardial systolic

contractions. Indirect actions result in increased carotid sinus nerve activity and
http://reference.medscape.com/drug/zorprin-bayer-buffered-aspirin-343279#1http://reference.medscape.com/drug/zorprin-bayer-buffered-aspirin-343279#1http://reference.medscape.com/drug/prednisone-intensol-342747#1http://reference.medscape.com/drug/prednisone-intensol-342747#1http://reference.medscape.com/drug/lanoxin-digoxin-342432#1http://reference.medscape.com/drug/lanoxin-digoxin-342432#1http://reference.medscape.com/drug/zorprin-bayer-buffered-aspirin-343279#1http://reference.medscape.com/drug/zorprin-bayer-buffered-aspirin-343279#1http://reference.medscape.com/drug/prednisone-intensol-342747#1http://reference.medscape.com/drug/prednisone-intensol-342747#1http://reference.medscape.com/drug/lanoxin-digoxin-342432#1http://reference.medscape.com/drug/lanoxin-digoxin-342432#1


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enhanced sympathetic withdrawal for any given increase in mean arterial

pressure. Therapeutic digoxin levels are present at trough levels of 1.5-2 ng/mL.


Captopril (Capoten)

Systemic afterload reduction may be helpful in improving cardiac output,

particularly in setting of mitral and aortic valve insufficiency. Some patients have

unusually large hypotensive response. Use small starting dose, particularly with

hypovolemia.


Furosemide (Lasix)

Diuretics frequently are used in conjunction with inotropic agents for patients

with CHF. When used aggressively, may result in hypokalemia and hypovolemia.

Risk of hearing loss in premature infants.

Increases excretion of water by interfering with chloride-binding cotransport

system, which, in turn, inhibits sodium and chloride reabsorption in ascending

loop of Henle and distal renal tubule.


Spironolactone (Aldactone)

Used in conjunction with furosemide as potassium-sparing diuretic.

Competes with aldosterone for receptor sites in distal renal tubules, increasing

water excretion while retaining potassium and hydrogen ions.
http://reference.medscape.com/drug/capoten-captoril-captopril-342315#1http://reference.medscape.com/drug/capoten-captoril-captopril-342315#1http://reference.medscape.com/drug/lasix-furosemide-342423#1http://reference.medscape.com/drug/lasix-furosemide-342423#1http://reference.medscape.com/drug/aldactone-spironolactone-342407#1http://reference.medscape.com/drug/aldactone-spironolactone-342407#1http://reference.medscape.com/drug/capoten-captoril-captopril-342315#1http://reference.medscape.com/drug/capoten-captoril-captopril-342315#1http://reference.medscape.com/drug/lasix-furosemide-342423#1http://reference.medscape.com/drug/lasix-furosemide-342423#1http://reference.medscape.com/drug/aldactone-spironolactone-342407#1http://reference.medscape.com/drug/aldactone-spironolactone-342407#1

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