RHEUMATIC HEART

DISEASE
DR.ABID ALI
Diagnosis
Jones criteria

(1) five major manifestations

(2) four minor manifestations, and
(3) supporting evidence
of an antecedent group A streptococcal infection
Diagnosis
• streptococcal pharyngitis, 1 to 5 weeks (average, 3 weeks) before the
onset of
• symptoms, is common. The latent period may be as long as 2 to 6
months (average, 4
• months) in cases of isolated chorea
Diagnosis
• Carditis
• Carditis occurs in 50% of patients. Signs of carditis include some or all of the following.
• 1. Tachycardia (out of proportion to the degree of fever) is common; its absence makes
• the diagnosis of myocarditis unlikely.
• 2. A heart murmur of mitral regurgitation (MR) or aortic regurgitation (AR) (or both) is
• almost always present. Although the American Heart Association’s Jones criteria
• recommend not to make the diagnosis of acute rheumatic carditis without audible
• murmurs of MR or AR, this is debatable. Significant echocardiographic abnormalities
• may be present in the absence of heart murmur.
MR AND RHD
• a hemodynamically insignificant echocardiographic finding of MR alone
is
• considered insufficient to diagnose myocarditis. Gross prolapse of the
mitral valve or the
• presence of posterolateral (not central) MR jet by color-flow mapping
may be significant.
• (With chronic rheumatic MR, fusion of the leaflets and cordae and
contracture of these
• structures occur, and the regurgitation jets tends to become more
central.)
Carditis
• abnormal echocardiographic findings may include pericardial effusion, increased left
• ventricular (LV) dimension, or impaired LV function.
• 3. Pericarditis (with friction rub, pericardial effusion, chest pain, and
• electrocardiographic [ECG] changes) may be present. Pericarditis does not occur without
• mitral valve involvement in rheumatic fever. Pericardial effusion is usually of small
• amount and almost never causes cardiac tamponade.
• 4. Cardiomegaly on chest radiograph is indicative of severity of rheumatic carditis (or
• valvulitis) or congestive heart failure (CHF).
• 5. Signs of CHF (gallop rhythm, distant heart sounds, cardiomegaly) are indications of
• severe cardiac dysfunction.
Erythema marginatum
ASO titers
• Antistreptolysin 0 (ASO) titer is well standardized and therefore is the most widely
• used test. It is elevated in 80% of patients with acute rheumatic fever and in 20% of
• normal individuals. Only 67% of patients with isolated chorea have an elevated ASO
• titer. ASO titers of at least 333 Todd units in children and 250 Todd units in adults
• are considered elevated. A single low ASO titer does not exclude acute rheumatic
• fever. If three antistreptococcal antibody tests (antistreptolysin O,
• antideoxyribonuclease B, and antihyaluronidase tests) are obtained, a titer for at
• least one antibody test is elevated in more than 95% of patients.
• Other Clinical Features
• 1. Abdominal pain, a rapid sleeping heart rate, tachycardia out of
proportion to fever,
• malaise, anemia, epistaxis, and precordial pain are relatively common
but not specific.
• 2. A positive family history of rheumatic fever also may heighten the
suspicion.
• The following tips help in applying the Jones criteria:
• a. Two major manifestations are always stronger than one major plus two minor
• manifestations.
• b. Arthralgia or a prolonged PR interval cannot be used as a minor
manifestation when
• using arthritis and carditis, respectively, as major manifestations.
• c. The absence of evidence of an antecedent group A streptococcal infection is a
• warning that acute rheumatic fever is unlikely (except when chorea is present).
• Exceptions to the Jones criteria include the following three specific
situations:
• a. Chorea may occur as the only manifestation of rheumatic fever.
• b. Indolent carditis may be the only manifestation in patients who
come to medical
• attention months after the onset of rheumatic fever.
• c. Occasionally, patients with rheumatic fever recurrences may not
fulfill the Jones
• criteria
• Prognosis
• The presence or absence of permanent cardiac damage determines the prognosis. The
• development of residual heart disease is influenced by the following three factors.
• 1. Cardiac status at the start of treatment: The more severe the cardiac involvement at
the
• time the patient is first seen, the greater the incidence of residual heart disease.
• 2. Recurrence of rheumatic fever: The severity of valvular involvement increases with
• each recurrence.
• 3. Regression of heart disease: Evidence of cardiac involvement at the first attack may
• disappear in 10% to 25% of patients 10 years after the initial attack. Valvular disease
• resolves more frequently when prophylaxis is followed.
Valve involvement
• Among rheumatic heart disease, mitral valve involvement occurs in
about
• three fourths and aortic valve involvement in about one fourth of the
cases. Stenosis and
• regurgitation of the same valve usually occur together. Isolated aortic
stenosis (AS) of
• rheumatic origin without mitral valve involvement is extremely rare.
Rheumatic
• involvement of the tricuspid and pulmonary valves almost never
occurs.
Prevention

• The prevention, control and elimination or eradication of rheumatic

heart disease is increasingly being recognized as an important
developmental issue

• Secondary prevention of rheumatic fever and rheumatic heart disease

is among the policy options for Member States in WHO’s Global
Action Plan for the Prevention and Control of Noncommunicable
Diseases 2013–2020.
Prevention

• There are three levels of prevention for rheumatic heart disease:

reducing the risk factors for rheumatic fever (primordial prevention);
primary prevention of rheumatic fever and rheumatic heart disease;
and secondary prevention (prophylaxis) of rheumatic fever and
rheumatic heart disease.

• Group A streptococcal infection accounts for 20–40% of cases of

pharyngitis in children. Compared with culture of throat swabs, rapid
antigen-detection tests
Prevention

• Secondary prophylaxis through the administration every 3–4 weeks of

injections of benzathine benzylpenicillin to patients with a previous
history of rheumatic fever and/or rheumatic heart disease is effective
at preventing streptococcal pharyngitis and a recurrence of rheumatic
fever. It requires case finding, referral, registration, administration of
penicillin injections and regular follow-up. Establishment of registries
of known patients has proven to be effective in reducing morbidity
and mortality, and these sources should be included in existing
national disease surveillance mechanisms, where available.
Prevention

• the main strategies for prevention, control and elimination include:

improving standards of living; expanding access to appropriate care;
ensuring a consistent supply of quality-assured antibiotics for primary
and secondary prevention; and planning, development and
implementing feasible programmes for prevention and control of
rheumatic heart disease, supported by adequate monitoring and
surveillance, as an integrated component of national health systems
responses.
Future Research

• Potential future research areas may include: better understanding of

disease epidemiology and case detection; further elucidation of the
pathogenic mechanisms of disease, aiming to identify new pathways
amenable to therapeutic intervention and to inform vaccine
development and application; development of a safe and effective
group A streptococcal vaccine; and development of a long-acting
formulation of penicillin that might improve adherence to secondary
prophylactic regimens
Barriers in Prevention

• The main barriers to prevention, control and elimination of rheumatic

heart disease are: the neglect of rheumatic fever and rheumatic heart
disease in national health policies and budgets in countries in which
rheumatic heart disease is endemic; the paucity of data to enable
targeting of prevention efforts; poor primary and secondary prevention
and access to primary health care; inadequate numbers and training of
health workers at all levels; limited understanding of rheumatic fever
and/or rheumatic heart disease in affected communities; and inaction
on the social determinants of the disease and inequities in health.
Barriers in prevention

• supply of quality-assured benzathine benzylpenicillin for secondary

prophylaxis is a significant challenge in some settings. The continued
availability of some essential medicines including benzathine
benzylpenicillin appears to be further threatened because prices have
become so low that it seems no longer commercially interesting for
manufacturers to supply them.
• Progression of Borderline RHD
• (Top) A 2-cm mitral regurgitant jet is seen in at least 1 view,,mitral
regurgitation is seen in 2 or more views, and a pan-systolic jet is
seen and measures >3 m/s. (Bottom) Two years later, the same
features are noted, but in addition there are new signs of restricted
posterior leaflet motion and anterior mitral valve leaflet thickness
>3 mm. This echocardiogram meets the criteria for definite
rheumatic heart disease (RHD) (pathological mitral regurgitation
with 2 morphological criteria)
 David A. Watkins et al. JACC 2018;72:1397-1416

2018 American College of Cardiology Foundation

 Parasternal Long-Axis Echocardiography Images of a Child With Borderline RHD

David A. Watkins et al. JACC 2018;72:1397-1416

2018 American College of Cardiology Foundation

 David A. Watkins et al. JACC 2018;72:1397-1416

2018 American College of Cardiology Foundation

 David A. Watkins et al. JACC 2018;72:1397-1416

2018 American College of Cardiology Foundation