Rheumatic heart disease facts

The global burden of disease caused by rheumatic fever and RHD currently falls disproportionately on children and
young adults living in low-income countries and is responsible for about 233,000 deaths annually.

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 schoolchildren in Africa, Asia, the Eastern Mediterranean region, and Latin America show
signs of the disease.
Treatment
Primary prevention of acute rheumatic fever (the prevention of initial attack) is achieved by treatment of acute throat
infections caused by group A streptococcus. This is achieved by up to 10 days of an oral antibiotic (usually penicillin) or a
single intramuscular penicillin injection.
People who have had a previous attack of rheumatic fever are at high risk for a recurrent attack, which worsens the damage
to the heart. Prevention of recurrent attacks of acute rheumatic fever is known as secondary prevention. This involves
regular administration of antibiotics, and has to be continued for many years. Secondary prevention programmes are
currently thought to be more cost effective for prevention of RHD than primary prevention and may be the only feasible
option for low- to middle-income countries in addition to poverty alleviation efforts.
Surgery is often required to repair or replace heart valves in patients with severely damaged valves, the cost of which is very
high and a drain on the limited health resources of poor countries.

Rheumatic heart disease (RHD) is a preventable, treatable form of

cardiovascular disease that affects over 32 million people around the world and
claims 275,000 lives annually. It affects the worlds poorest, most vulnerable
populations and imposes heavy costs on the health systems that can least afford
it.
If left untreated, rheumatic heart disease can lead to heart valve damage, stroke,
heart failure, and death. Treatment of advanced disease requires costly surgery
unavailable in many parts of the world. In endemic countries, prevalence of this
preventable disease is a stark measure of health system failure and inequality.
RHD is the most commonly acquired heart disease in young people under the
age of 25. It most often begins in childhood as strep throat. If left untreated, RHD
may progress to serious heart damage that kills or debilitates adolescents and
young adults, and makes pregnancy hazardous. Although virtually eliminated in
Europe and North America, the disease remains common in Africa, the Middle
East, Central and South Asia, the South Pacific, and in impoverished pockets of
developed nations.

Who is at risk?
The biggest risk factor for developing RHD is having repeated episodes of ARF.
This risk can be reduced by regular antibiotic medication. RHD usually begins in
adolescence but can be diagnosed into adulthood. 80% of people living with
RHD live in developing countries. Others live in vulnerable populations in
developed settings.

Symptoms of RHD
There may be no symptoms in the early stages of RHD, this is sometimes called
the asymptomatic or latent phase. It may be possible for doctors or health
workers to check signs of early disease which people may not notice themselves.
This may include:
Heart murmurs heart through a stethoscope
Changes in the way heart valves work may be seen on an heart scan
(echocardiography)
The first symptoms of RHD are usually from heart failure. These may include:
Fatigue (feeling tired)
Feeling short of breath, this may be worse when exercising or when lying
down.

Treatment
Treatment of RHD usually requires young people to have regular antibiotics
which prevent further attacks of ARF and damage to heart valves. Penicillin is the
most commonly used antibiotic and is often given as an injection every 3 4
weeks.

Medication may also be given to manage symptoms of heart failure or heart

rhythm abnormalities. Anticoagulation medication to reduce the risk of blood clots
may be needed. In some cases of advanced RHD heart surgery may be
required. Information about living with RHD, medical treatment and surgery is
available in the living section of this site.

Medical Care
Medical therapy in rheumatic heart disease includes attempts to prevent rheumatic fever (and thus rheumatic
heart disease). In patients who develop rheumatic heart disease, therapy is directed toward eliminating the
group A streptococcal pharyngitis (if still present), suppressing inflammation from the autoimmune response,
and providing supportive treatment for congestive heart failure. Following the resolution of the acute episode,
subsequent therapy is directed towards preventing recurrent rheumatic heart disease in children and
monitoring for the complications and sequelae of chronic rheumatic heart disease in adults.

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.[8]
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 patients who are allergic to penicillin are also allergic to
cephalosporins.
Do not use tetracyclines or 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:

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.
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.
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 days) is recommended.
In general, antimicrobial therapy is not indicated for pharyngeal carriers of GABHS. Exceptions include the
following:

Outbreaks of rheumatic fever or poststreptococcal glomerulonephritis

Family history of rheumatic fever
During outbreaks of GAS pharyngitis in a closed community
When tonsillectomy is considered for chronic GABHS carriage
When multiple episodes of documented GABHS pharyngitis occur within a family despite appropriate
therapy
Following GAS toxic shock syndrome or necrotizing fasciitis in a household contact

Treatment for patients with rheumatic fever and rheumatic heart disease
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.
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 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.
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, third-degree 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 (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 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.

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.
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.
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, 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). The guidelines for endocarditis prophylaxis in patients with valve damage from rheumatic heart disease
have changed. Antibiotic prophylaxis is no longer recommended. [14]

A study that investigated the difference in clinical manifestations and outcomes between first episode and
recurrent rheumatic fever concluded that subclinical carditis occurred only in patients experiencing the first
episode, and that all deaths occurred in patients with recurrent rheumatic fever, emphasizing the need for
secondary prophylaxis.[17]

Consultations
In addition to cardiology consultation, complications may require cardiothoracic surgery consultation (heart
failure and progressive valve insufficiency) and neurology consultation (chorea, PANDAS).

Diet and activity

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.
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 life-saving.
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.
In the past, due to high rates of recurrent symptoms after annuloplasty or other repair procedures, valve
replacement appeared to be the preferred surgical option. However, modifications of standard repair
techniques, adherence to the importance of good leaflet coaptation and strict quality control with stringent use
of intraoperative transoesophageal echocardiography have all contributed to the improved long-term results. [18]
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