Understanding Rheumatic Heart Disease
Understanding Rheumatic Heart Disease
Objectives :
1. What is ARF And RHD?
2. Diagnosis
3. Jones Criteria & 2015 revision
4. Differential Diagnosis
5. Investigations, Management
6. Rheumatic Valvular Heart Disease
7. Prevention
Done by :
Leader: Hadeel Awartani
Members: Laila Alsabbagh,Khalid Almutairi, Ebtesam
Almutairi ,Hussain Allami
Revised by :
Yazeed Al-Dossare
Resources :
437 slides, 436 team, Davidson 22nd edition & Kumar 8th
edition.
Global Burden :
15.6 Millions Total cases with RHD
3 phases:
• phase 1 : throat infection.
3 Million Have congestive heart failure. • Phase 2 : RHF ( after weeks ).
• Phase 3 : RHD ( after years ).
Incidence
- 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.
1
New cases per year
Pathogenesis
- 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.
Antischkow cells are enlarged macrophages found within granulomas (called aschoff bodies ) associated
with the disease. Larger Antischow cells may coalesce to form multinucleated Aschoff bodies. (See the pic)
* very important to memorize, usually asked about in exams
When the bacteria enter the body it will cause immunological reaction-> the body will produce antibodies against ( M protein )
which is the component of bacteria and similar structure found in heart, skin, joints and brain.
Clinical Features
- 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.
As we mentioned, 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.
**The major problem , we worry about it, because it Destroy the valve and cause permanent damage.
-Murmure in acute attack indicate carditis , HF in acute attack indicate severe reg.
Diagnosis
- No single test to diagnose ARF.
- The symptoms and signs are shared by many inflammatory and infectious diseases
- Accurate diagnosis is important, because:
- Overdiagnosis will result in individuals receiving treatment unnecessarily
- Underdiagnosis may lead to further episodes of ARF causing damage, and the need for valve
surgery, and or premature death
- Diagnosis is primarily clinical (it's a syndrome) and is based on a constellation of signs and symptoms, which
were initially established as the Jones criteria.
- Only about 25% of patients will have a positive culture for group A streptococcus at the time of diagnosis
because there is a latent period between infection and presentation.
- Serological evidence of recent infection with a raised antistreptolysin O (ASO) antibody titre is helpful.
- A presumptive diagnosis of acute rheumatic fever can be made without evidence of preceding streptococcal
infection in cases of isolated chorea or pancarditis, if other causes for these have been excluded.
A- Acute Rheumatic Fever
Diagnostic Criteria
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.
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.
- 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,
Arthritis 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)
DDx of ARF
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.
*to detect subclinical carditis , subclinical means murmur we can’t heart so we use ECG to detect it.
Treatment of ARF
NO specific treatment Now you diagnosed your patient with ARF, what treatment options you can offer
him/her?
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.
The main pathological process in chronic rheumatic heart disease is progressive fibrosis.
Refresh your memory : The heart sound is basically “LUB” which is S1 - This sound is produced by
closure of Atrioventricular valves- + “DUB” which is S2 - This sound is produced by closure of Aortic
and pulmonary valves .
Mitral Regurgitation
May also follow mitral valvotomy or valvuloplasty. Chronic mitral regurgitation causes gradual dilatation of the LA with
little increase in pressure and therefore relatively few symptoms.
- Asymptomatic
- Dyspnea, orthopnea, and
- PND(parxosymal nocturnal dyspnia)
- Displaced PMI*
Clinical features
- Thrill
- Soft S1 “Wall hugging”
- Pansystolic murmur Best heard on the lateral side when lying on
the side radiating to the axilla
Treatment Surgically
*
The point of maximal impulse (PMI) is simply that... the point where there is a maximal impulse against the chest that can be felt.
Most often, this is from the apex or tip of the heart: also referred to as the apical impulse. However, in certain conditions, the apex of
the heart does not cause the PMI.
The normal mitral valve area (MVA) = 4-6 cm2 High LAP left atrial pressure
Clinical features
- Dyspnea due to pulmonary edema
- Fatigue no enough blood bumped to the body to supply it.
- Palpitation because of arrhythmias from stretching the LA (A-fib is the most common)
- Hemoptysis (10%) due to pulmonary hypertension so the vessels will rupture. B.c of congested lungs
- Hoarseness (Ortner’s syndrome) paralysis of the vocal cords, due to the enlargement of pulmonary artery→ compression of recurrent laryngeal
nerve
- Dysphagia Pressure of LA
- Stroke or peripheral embolization Why ? Left atrium will be enlarged due to stenosis, so the rhythm will be changed from
sinus to atrial and the patient usually develops Atrial Fibrillation. Afib will lead to blood clotting that will form a thrombus which travels to
the brain causing stroke (and sometimes stroke is the main presentation in a patient with Mitral stenosis).
- Cyanosis (Mitral facies2, malar flush (late complication))
- Tapping apex ( S1) Tapping apex beat is present only in mitral stenosis where the left ventricular size and filling is less .
- Parasternal heave It’s precordial impulse that may be felt in patients with cardiac or respiratory
disease. Its either from the pushing of LA or from the hypertrophy of the RV
- Diastolic thrill A vibration felt over the heart during ventricular diastole . it may be caused due to
mitral valve stenosis.
- Accentuated (very obvious) S1
- Accentuated S2
- Opening snap S2 is followed by an opening snap , the distance between S2 and the opening snap
can give an indication as to the severity of the stenosis . the closer the opening snap follows S2 ,
the worst the stenosis .
- Mid-diastolic rumble
Investigations
Management
Patients with minor symptoms should be treated medically. Intervention by balloon valvuloplasty, mitral valvotomy or
mitral valve replacement should be considered if the patient remains symptomatic despite medical treatment or if
pulmonary hypertension develops.):
➢ B-Blockers ,CCB to increase diastole phase , so there will be more blood filling. Slow them down, allow more time for
filling of LV (BVP)
➢ Digoxin ( AF ) Especially if there is HF and hypotension
➢ Warfarin to prevent thrombus formation in the LA
Definitive therapy: you can have up to 3 times but at the end you have to have
➢ Balloon Valvuloplasty MV replacement
➢ Mitral valve replacement
➢ Diuretics to relieve the congestion
2
distinctive facial appearance associated with mitral stenosis . Someone with mitral stenosis may present with rosy cheeks, whilst the rest of the face has a bluish tinge due to cyanosis. ... It is
due to low cardiac output, and therefore low perfusion of the facial skin, caused by the stenosis.
Aortic Regurgitation
This condition is due to disease of the aortic valve cusps or dilatation of the aortic root, The LV dilates and hypertrophies to
compensate for the regurgitation. The stroke volume of the LV may eventually be doubled or tripled, and the major arteries are
then conspicuously pulsatile. As the disease progresses, left ventricular diastolic pressure rises and breathlessness develops.
Early diastolic
murmur
Diastolic murmurs + regurgitation
Aortic valve Replacement (could be done via Transcathter Aortic Valve Replacement)
Treatment
Aortic Stenosis
Triad : VERY IMPORTANT
❏ Angina when the left ventricle enlarges its muscle mass will increase which will increase the oxygen
demand and it cant meet the demand because of the stenosis > chest pain . and its the most common
presentation.
❏ Syncope because there is no enough blood “cardiac output” flow to the brain (low pCO) (exertional)
❏ Dyspnea and PND The worst clinical feature as it indicates HF.
Prognosis: is worse with dyspnea, better in syncope and angina
Without treatment life expectancy is with:
- Angina (5 years)
- Syncope (3 years)
Clinical features - Dyspnea (1 years)
Other clinical features
- Arterial Pulse waveform: Plateau
- Small (Parvus) diminished carotid upstorke Slow rise (Tardus)delayed carotid upstroke Sustained
not displaced PMI
- Systolic thrill S4
- Late peaking of murmur Single S2 : Soft or absent A2
- Paradoxical splitting of S2 (occurs when the splitting is heard during expiration and disappears
*Thickened and fibrotic during inspiration, the opposite of the physiologic split S2)
Aortic valve Replacement (could be done via Transcathter Aortic Valve Replacement)
Treatment why can't we use balloon valvuloplasty like in mitral stenosis ? because the stenosis here is calcification and we
can't ballon calcium very well, where as in mitral stenosis it is fibrosis, and we can ballon fibrotic tissue .
Summary 436
ARF : immune-mediated delayed response to infection with specific strains of beta hemolytic streptococci
(manifestations after 2-4 weeks), Antibodies produced cause inflammation in the endocardium,
myocardium and pericardium, as well as the joints, skin, and heart valves.
Clinical features Fever, lethargy, anorexia, joint pain, arthritis, carditis, neurological changes.
- 2 Major manifestations
- 1 Major and 2 Minor manifestations And Evidence of a recent streptococcal
infection.
Diagnosis
- 3 Minor
Evidence of Preceding GAS Infection:
1) Increased ASO titer or Anti-Dnase B titer. 2) positive throat culture.
Major : Minor :
Jones criteria 1. Cardritis 1. Fever
(high/lower 2. Migratory polyarthritis 2. Elevated ESR
risk 3. Sydenham Chorea 3. Polyarthralgia ( major in high risk)
population) 4. Subcutaneous Nodule 4. History of rheumatic fever
5. Erythema Marginatum 5. First degree heart block
➢ WBC.
➢ ESR.
➢ CRP.
➢ Blood cultures, if febrile.
Investigation ➢ ECG to look for Heart block.
➢ Chest X-ray, if clinical or echocardiographic evidence of carditis.
➢ Echocardiogram .
➢ Throat swab: culture for group A streptococcus.
➢ Anti-streptococcal serology: both ASO and anti-DNase B titres
➢ Bed Rest
➢ Salicylates
Treatment ➢ Penicillin
➢ Steroids
➢ Heart Failure Treatment
Management and
Condition Clinical features
treatment
➢ Accentuated S1 &
➢ Dyspnea (pulmonary S2
venous congestion) ➢ Opening snap
Management:
➢ Fatigue ➢ Mid-diastolic
rumble ➢ B-Blockers ,CCB
➢ Palpitation ➢ On X-ray : ➢ Digoxin ( AF )
➢ Hoarseness Straightening of the
Mitral ➢ Dysphagia left heart border, ➢ Warfarin If there is
atrial fibrillation to prevent
stenosis ➢ Stroke or peripheral Double density, stroke.
Kerley B lines or
embolization pulmonary edema. ➢ Balloon
➢ ECG : Valvuloplasty
➢ Cyanosis
LAE, P Mitrale ➢ Mitral valve
➢ Tapping apex RV dominance
Atrial Fibrillation.
replacement
➢ Parasternal heave
➢ Diastolic thrill ➢ Echo Doppler :
Calcification in MV
➢ Asymptotic
Mitral ➢ Dyspnea, orthpnea and PND Management:
regurgitation ➢ Displaced PMI, thril Surgical
➢ Soft S1
➢ Syncope Treatment:
➢ Angina Aortic valve
➢ Dyspnea and PND replacement
➢ Arterial Pulse waveform: Plateau
Aortic ➢ Small (Parvus)
stenosis ➢ Slow rise (Tardus)
➢ Sustained not displaced PMI
(SAD) ➢ Systolic thrill
➢ S4
➢ Late peaking of murmur
➢ Single S2 : Soft or absent A2
➢ Paradoxical splitting of S2
Examine Yourself !!
1. Which one of the following Organisms is the most common cause for Rheumatic Heart Disease?
A. group A beta hemolytic streptococcus
B. group B alpha hemolytic streptococcus
C. staphylococcus aureus
D. E.coli
2. Which one of the following group of people are mostly affected by Rheumatic Heart Disease?
A. Elderly from 40-60
B. Teenagers from 14-18
C. Children from 5-15
D. New born babies
3. Which one of the following Valve is mostly affected by Rheumatic Heart Disease ?
A. Mitral Valve
B. Tricuspid Valve
C. Aortic Valve
D. Pulmonary Valve
5. Which one of the following serological tests is used to detect recent infection by Group A streptococcus?
A. Antistreptolysin O antibodies
B. Antinuclear antibodies
C. Antimitochondrial antibodies
D. Antigliadin antibodies
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Examine Yourself !!
8. Which one of the following is seen under the microscope and confirm the diagnosis of ARF ?
A. Aschoff Nodules
B. Lewy Body
C. Caseous Necrosis
D. Mallory Body
9. In order to treat patient with Streptococcal pharyngitis , which of the following is the best for him ?
A. penicillin
B. Ciftriaxone
C. Ciprofloxacin
D. Gentmycin
10. Which one of the following is the main pathological process in Chronic
Rheumatic Heart Disease ?
A. Calcification
B. Necrosis
C. Amyloidosis
D. Progressive Fibrosis
11. Which one of the following is Clinical feature for Aortic Regurgitation ?
A. Collapsing pulse
B. Syncope
C. Dyspnea
D. Angina
12. Which one of the following is Clinical feature for Mitral Regurgitation ?
A. Early diastolic murmur
B. Mid systolic click
C. Pansystolic murmur
D. Systolic Thrill
16. Syncope , Angina and Dyspnea are characters for which disease ?
A. Aortic Regurgitation
B. Aortic Stenosis
C. Mitral Stenosis
D. Mitral Regurgitation
19. Which one of the following valve is LESS common affected by Rheumatic Heart Disease?
A. Aortic valve
B. Mitral valve
C. Tricuspid
D. Pulmonary
20. Which one of the following is Clinical feature for Mitral Stenosis?
A. Opening Snap
B. Mid diastolic rumble
C. Soft S1
D. A&B
Answer key :
1-A 2-C 3-A 4-D 5-A 6-D 7-C 8-A 9-A 10-D 11-A 12-C 13-B 14-D 15-A 16-B 17-B 18-D 19-D 20-D
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Questions
1.Which one of the following Organisms is the most common cause for Rheumatic Heart Disease ?
A-group A beta hemolytic streptococcus
B-group B alpha hemolytic streptococcus
C-staphylococcus aureus
D-E.coli
2. Which one of the following group of people are mostly affected by Rheumatic Heart Disease ?
A-Elderly from 40-60
B-Teenagers from 14-18
C-Children from 5-15
D-New born babies
3. Which one of the following Valve is mostly affected by Rheumatic Heart Disease ?
A- Mitral Valve
B- Tricuspid Valve
C- Aortic Valve
D- Pulmonary Valve
5. Which one of the following serological tests is used to detect recent infection by Group A streptococcus?
A-Antistreptolysin O antibodies
B-Antinuclear antibodies
C-Antimitochondrial antibodies
D-Antigliadin antibodies
9.In order to treat patient with Streptococcal pharyngitis , which of the following is the best for him ?
A-penicillin
B-Ceftriaxone
C- Ciprofloxacin
D- Gentamicin
10.Which one of the following is the main pathological process in Chronic Rheumatic Heart Disease ?
A-Calcification
B-Necrosis
C-Amyloidosis
D- Progressive Fibrosis
11. Which one of the following is Clinical feature for Aortic Regurgitation ?
A- Collapsing pulse
B- Syncope
C-Dyspnea
D- Angina
12. Which one of the following is Clinical feature for Mitral Regurgitation ?
A-Early diastolic murmur
B-Mid systolic click
C-Pansystolic murmur
D- Systolic Thrill
19. Which one of the following valve is LESS common affected by Rheumatic Heart Disease?
A-Aortic valve
B- Mitral valve
C-Tricuspid
D-Pulmonary
20. Which one of the following is Clinical feature for Mitral Stenosis?
A-Opening Snap
B-Mid diastolic rumble
C- Soft S1
D-A & B
Answer key :
1-A 2-C 3-A 4-D 5-A 6-D 7-C 8-A 9-A 10-D-11-A 12-C 13-B 14-D 15-A 16-B 17-B 18-D 19-D 20-D