RHEUMATIC HEART

DISEASE

PRESENTED BY
Ms. DEBIKA DAS, M.Sc. (N)
NEUROSCIENCE NURSING
Definition
Acute rheumatic fever usually affects children (most commonly between 5 and 15
years) or young adults . - The most common cause of acquired heart disease in
childhood and adolescence.
The condition is triggered by an immune-mediated delayed response
(manifestations appearing after a period of 2-4 weeks) to infection with specific
strains of group A (beta hemolytic) streptococci, which have antigens that may
cross-react with cardiac myosin and sarcolemmal membrane protein. - Antibodies
produced against the streptococcal antigens cause inflammation in the
endocardium, myocardium and pericardium, as well as the joints and skin, but the
major effect on health is due to damage to heart valves. - Histologically, fibrinoid
degeneration is seen in the collagen of connective tissues.
*(VERY IMP) Aschoff nodules, are pathognomonic and occur only in the heart. They
are composed of multinucleated giant cells surrounded by macrophages and T
lymphocytes, and are not seen until the subacute or chronic phases of rheumatic
carditis.
Acute rheumatic fever is a multisystem disease that usually presents with
 fever,
 anorexia,
 lethargy and
 joint pain
2–3 weeks after an episode of streptococcal pharyngitis. There may, however, be no
history of sore throat.
 Arthritis* occurs in approximately 75% of patients.
 Other features include rashes, carditis** and neurological changes.
Clinical features are not specific. for example: leukemia, sickle cell anemia and viral
infections can cause joint pain and swelling. So you should diagnose the patient
carefully. It’s a multisystem disorder that will affect:
• 1) CNS : mainly basal ganglia
• 2) Joints and skin : will recover later
• 3) Heart : permanent damage to the valves. *not serious because it doesn’t leave
permanent damage.
Definition
It is a non-suppurative sequela of
Group A, ß-hemolytic
streptococcal (GABHS) throat
infection. The disease involves
the joint (hence the word
“rheumatic”), heart, skin,
subcutaneous tissue and the
brain. It is considered as acute
when the illness lasts for less
than 6 months or chronic, if it is
grater than 6 months in
duration.
• In cases of established rheumatic heart disease or prior rheumatic
fever, a diagnosis of acute rheumatic fever can be made based only on
the presence of multiple minor criteria and evidence of preceding
group A streptococcus pharyngitis.
1992 Modified jones criteria
• Major criteria* Migratory polyarthritis Carditis Erythema marginatum
Sydenham chorea Subcutaneous nodules Minor criteria Arthralgia
(Not arthritis!) First degree heart block Fever Elevated inflammatory
markers (ESR, CRP)
2015 Revision of jones criteria

• pulations. 1. In accordance with the degree of prevalence of ARF/RHD in the population: - low risk
populations have been defined as those with ARF incidence < 2:100000 school-age children or all
age prevalence of RHD of < 1:1000 population per year - Children not from low risk population
have been considered to be at moderate or high risk. - 2. Advocated the use of Echocardiography
in all cases of confirmed or suspected ARF or RHD, to diagnose valvulitis( subclinical carditis) and
has been included as a major criterion to diagnose carditis. 3. Aseptic monoarthritis has been
included as a major criteria in moderate or high risk population. 4. Polyarthralgia has been
recognized as a major manifestation for moderate or high risk population 5. Fever: low risk
population: >38.5 c, ESR >60 and or CRP > 3mg/dl, while moderate or high risk population: fever
>38 and ESR >30 and or CRP > 3mg/dl for A firm diagnosis requires: 2 Major manifestations or 1
Major and 2 Minor manifestations And Evidence of a recent streptococcal infection. Or 3 Minor.
Evidence of Preceding GAS “Group A strept.”Infection: 1) *Increased or rising ASO titer or Anti-
Dnase B titer. 2) *A positive throat culture. 1992 Modified jones criteria It is based upon two or
more major manifestations, or one major and two or more minor manifestations, along with
evidence of preceding streptococcal infection. * Will be discussed in more detail later on. 2
• A firm diagnosis requires: 2 Major manifestations or 1 Major and 2
Minor manifestations And Evidence of a recent streptococcal
infection. Or 3 Minor. Evidence of Preceding GAS “Group A
strept.”Infection: 1) *Increased or rising ASO titer or Anti-Dnase B
titer. 2) *A positive throat culture.
A ‘pancarditis’ involves the endocardium, myocardium and pericardium to varying
degrees. Its incidence declines with aging, ranging from 90% at 3 years to around 30% in
adolescence. - Occurs in 50-70% of cases. - The only manifestation of ARF that leaves
serious permanent damage. - May be subclinical (without murmurs so we should use
Echo). - Murmurs of MR or AR may occur in acute stage, while mitral stenosis occur in
late stage - Cardiomegaly and CHF may occur. (in the acute phase)
- This is the most common major manifestation and occurs early when streptococcal
antibody titres are high (present in 35-66% ,Earliest manifestation of ARF). - An acute
painful asymmetric and migratory inflammation (Migrating, “Fleeting”polyarthritis) of
the large joints typically affects the knees, ankles, shoulders,elbows. The joints are
involved in quick succession and are usually red, swollen and tender. Rarely affects the
spine. - Duration short < 1 week. - Rapid improvement with salicylates.(Aspirin) The pain
characteristically responds to aspirin; if not, the diagnosis is in doubt. - Does not progress
to chronic disease. (Doesn’t have any late manifestations as the RHD)
- Also known as Saint Vitus's Dance, occur in 10-30%, female predominance. - This is a
late neurological manifestation (extrapyramidal manifestation) that appears at least 3
months after the episode of acute rheumatic fever, when all the other signs may have
disappeared (Delayed manifestation of ARF). - Emotional lability may be the first feature
and is typically followed by purposeless, involuntary, choreiform movements of the
hands,face, neck, trunk, and limbs.. Speech may be explosive and halting (clumsiness,
deterioration of handwriting,emotional lability or grimacing of face). - Spontaneous
recovery usually occurs within a few months. Approximately one-quarter of affected
patients will go on to develop chronic rheumatic valve disease..
Subcutaneous nodules occur in 10% of patients. - They are small (0.5–2.0 cm), firm non-
tender and painless. - Best felt over extensor surfaces of bone or tendons (Occur over
extensor surfaces of joints, on bony prominences, tendons, spine). Key words (firm non
tender and its on extensor surfaces of joints) - They typically appear more than 3 weeks
after the onset of other manifestations and therefore help to confirm rather than make
the diagnosis - Short lived: last for few days. - Associated with severe carditis. (When you
have it suspect Carditis)
Erythema marginatum occurs in less than 6% of patients - The lesions start as red
macules that fade in the centre(pale center) but remain red at the edges, round or
irregular serpiginous (having a wavy margin) borders, non-pruritic, transient rash. - The
resulting red rings or ‘margins’ may coalesce or overlap. - Occur mainly on the trunk and
proximal extremities but not the face. - Less common, but highly specific manifestation
of ARF. - Associated with carditis
 SIGNS & SYMPTOMS
Polyarthritis & fever Carditis Chorea
 Septic Arthritis  Innocent  Systemic Lupus
(including murmur erythematosus
disseminated  Mitral valve  Drug intoxication
gonococcal prolapse  Wilson’s disease
infection)  Congenital  Tic disorder
 Connective tissue Heart disease  Choreoathetoid
and other  Infective cerebral palsy
autoimmune endocarditis  Encephalitis
disease  Hypertrophic  Familial chorea
 Viral arthropathy cardiomyopathy (Including
 Reactive  Myocarditis: Huntington’s)
arthropathy viral or  Intracranial tumor
 Lyme disease idiopathic  Lyme disease
 Investigations

• White blood cells count. - Erythrocyte sedimentation rate (ESR). - C-reactive

protein (CRP). - Blood cultures, if febrile. - Electrocardiogram (if prolonged
P-R interval or other rhythm abnormality, repeat in 2 weeks and again in 2
months, if still abnormal). To look for Heart block (minor criteria) - Chest X-
ray, if clinical or echocardiographic evidence of carditis. - Echocardiogram
Very important* (consider repeating after 1 month, if negative) - Throat
swab (preferably before giving antibiotics): culture for group A
streptococcus. - Anti-streptococcal serology: both ASO and anti-DNase B
titres, if available (repeat 10-14 days later if first test not confirmatory)
*Remember that if you’re suspecting ARF or if you’ve confirmed it, you
should do an echocardiography to confirm or refuse the diagnosis of
rheumatic carditis.
 TREATMENT OF ARF

• 1. Bed Rest : It’s important, as it lessens joint pain and reduces cardiac workload.
The duration should be guided by symptoms, along with temperature, leucocyte
count and ESR, and should be continued until these have settled. 2. Salicylates :
Like Aspirin, this usually relieves the symptoms of arthritis rapidly and a response
within 24 hours helps confirm the diagnosis. We should monitor the patient for
toxicity (usually he will have tinnitus and vomiting) 3. Penicillin : Like Procaine
Penicillin 4 million units/day x10 days. (Know the dose) if the patient is penicillin-
allergic, erythromycin or a cephalosporin can be used. 4. Steroids (Prednisolone):
2 mg/kg/day taper over 6 weeks Produces more rapid symptomatic relief than
aspirin and is indicated in cases with carditis or severe arthritis (Given when
there is severe carditis). There is no evidence that long-term steroids are
beneficial. 5. Heart Failure Treatment : Like diuretics and ACEI. If heart failure
develops, and does not respond to medical treatment, valve replacement may be
necessary and is often associated with a dramatic decline in rheumatic activity.
• Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) :
Primary prevention is important by improving socio-economical status - Patients
are susceptible to further attacks of rheumatic fever if another streptococcal
infection occurs, and long-term prophylaxis with penicillin should be given as
benzathine penicillin Long acting penicillin (1.2 million U IM monthly), if
compliance is in doubt, or oral phenoxymethylpenicillin (250 mg twice daily). -
Sulfadiazine or erythromycin may be used if the patient is allergic to penicillin;
sulphonamides prevent infection but are not effective in the eradication of group
A streptococci. - Further attacks of rheumatic fever are unusual after the age of
21, when treatment may be stopped. However, it should be extended if an attack
has occurred in the last 5 years, or if the patient lives in an area of high
prevalence or has an occupation (e.g. teaching) with high exposure to
streptococcal infection.In those with residual heart disease, prophylaxis should
continue until 10 years after the last episode or 40 years of age, whichever is
later. Long-term antibiotic prophylaxis prevents another attack of acute
rheumatic fever but does not protect against infective endocarditis.
“Summarised in the picture”