1DDX: LECTURE 19 – NOVEMBER 22nd, 2006

RHEUMATIC FEVER AND CARDIAC VALVULAR DISEASES

A 44-year-old Mexican man complains of a progressively worsening cough over the past 5 months.
His cough is associated with shortness of breath on exertion over past year. He must sleep propped
up on 2-3 pillows to breathe (he has two- to three-pillow orthopnea). He notes PND intermittently.
The patient has lived in a very rural area of Mexico for most of his life. His medical history is
significant for several episodes of sore throat as a child.

Physical Examination

Examination of the lungs reveals bilateral basilar crackles but is otherwise clear to percussion and
auscultation. A cardiac exam reveals a prominent first heart sound S1 . A soft I/VI holosystolic
(throughout systole) murmur is heard at the apex. 2nd heart sound S2 is normal. There is loud
opening snap. Soft diastolic murmur is heard best at apex, left axilla. No jugular venous distension
and no peripheral edema. Remainder of physical exam is within normal limits. An ECG shows
notched p waves, but is otherwise normal. A chest radiograph shows bilateral costo-diaphragmatic
angle blunting (accumulation of fluid). The left atrium appears slightly enlarged. The lung fields are
otherwise clear.

What is major pt complaint? Cough (consider resp. and CV system)

DDX?
Cardiac valvular disease?
Congestive heart failure? Probably left heart because of congestion in lungs, also no jugular vein
distension.
Cor pulmonale? (secondary to lung problem)
Angina?
Will come back to this case.

Diseases of the Cardio-vascular system: 4th note package

RHEUMATIC FEVER
• ACUTE SYSTEMIC inflammatory disease that occurs as result of a RECENT Strep infection.
• Symptoms of acute pharyngitis: acute sore throat, sometimes n/v, abdominal pain, difficulty
swallowing
• Can become rheumatic fever or AGN
• Over 50 microbial causes of RF, but we will focus on group A beta-hemolytic Streptococcus
• Treatment is antibiotics: must eradicate Strep to prevent development of RF and damage to heart.

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• Multisystem disorder 1-5 weeks (average 3 weeks) after Group A Strep infxn.
• RF can lead to RHD (Rheumatic heart disease)
• Result from immunologic response
• Note statement “RF licks the joints, but bites the heart”

• Most common in low socio-economic groups, especially if in crowded house

• GAS=group A Streptococcus

DDX LECTURE 19, NOVEMBER 22nd – PAGE 1

• Pharyngeal route necessary for infection. Skin or other routes do not result in RF.
• Latent period: period of 1-5 weeks between Strep throat infection and signs/symptoms of RF.
• Cross reaction between Strep antibodies and cardiac antigens. Pt. Has increased titre of Strep
antibodies following Strep
• Variable onset: may be abrupt or drawn out. MLJP: pain moves.

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Clinically relevant pathology

• If there is an inflammatory process from RF: may have either or both of proliferative process or
exudative process.

1. Myocardial Aschoff body lesions: VERY SPECIFIC: only found on pt. With RF: this is a granuloma.
Most specific pattern of rheumatic inflammation. Occur only in myocardium. As result, get
inflammation of myocardium (myocarditis). Disruption of structure of cardiac layer. Irreversible
scarring results.
2. Endocardial tissue: will have signs of endocarditis. On autopsy, endocarditis is manifested by
vegetation. Surface of valve is not smooth, but is rough. Valves can’t close properly. This will
cause secondary damage. Leaflets become fused, calcified, and the valvular orifice is destroyed
(become much smaller)
3. Pericardial tissue: may also be involved in RF. Pericarditis may develop with accumulation of
inflammatory fluid in pericardial sac. Serofibrinous effusion, calcification follows.

Diagnose RF using Jones Criteria (see notes)

• Major criteria reflect involved systems.
• Minor: arthralgia and arthritis are distinct pathologies. Arthralgia is joint pain that could be the
result of many pathological mechanisms.
• To diagnose, need 2 major, or 1 major and 2 minor criteria. Also need history or lab proof of Strep
infection.

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Pancarditis? This means that all layers of the heart are involved.

Carditis: (all forms: pericarditis, myocarditis, endocarditis, pancarditis)

Chest pain
• With carditis, patient may present with chest pain that is not exercise-linked (DDx with angina).
• Pain from carditis is LOCALIZED: patient can point to the area (usually due to pericardial
involvement). Myocardium doesn’t have pain fibres, neither does endocardium.
• Pain is worse in supine position, because this increases pressure on inflamed pericardium.
Better when patient is sitting and leaning forward.
ECG changes in pericarditis (if pericardium is involved) (add this to list).
• Characterized by ST elevation (also see this with MI: DDX) in all leads: diffuse ST elevation. In
MI, the ST elevation would only be in the leads that are overlying the affected area.
• PR interval will be prolonged if myocardium is involved.
Chest X-ray (add this): If you see accumulation of inflammatory fluid, the heart will be enlarged.
Tachycardia (compensatory: if this is happening, myocardium is involved.)
• Heart function is compromised: beats faster to maintain CO
Arrythmia: due to Aschoff bodies
DDX LECTURE 19, NOVEMBER 22nd – PAGE 2
 Dyspnea
Cardiac enlargement: due to accumulation of inflammatory fluids in pericardial sac.
If endocardium is involved, you will see the appearance of a new murmur.

(She said that we will not be examined on ECG changes???)

(DDX between pleural friction rub and pericardial friction rub? On the chest, it may come from either
structure. Get patient to hold breath. If the sound disappears, it is due to pleural friction rub.
Constant? Due to pericardial friction rub.)

Polyarthritis:
• Often the most common symptom that patient will report.
• Migratory! Signs and symptoms fibral illness. May see increase in body temperature. Large joints
are involved first.
• Key word in contrast to rheumatoid arthritis? MIGRATORY! Pain disappears from one joint, goes
to another.

Chorea:
• CNS disorder: involuntary, purposeless, non-repetitive movements. May have trouble writing,
drawing, handiwork.
• Symptoms worsened by excitement, effort, fatigue, subside during sleep. Speech disturbance
possible too.

(Page 5)

Chorea, continued:
• Kids may be teased d/t grimacing (can’t control)
• This will subside without any neurological complications.
• No path changes in NS.
• Self-limiting! Temporary impairment of motor functions. Will not affect intellectual capacity.

Subcutaneous nodules:
Same granulomas (proliferative process) that are found in the heart: these are under skin.

Erythema Marginatum:
Very specific to this condition.
Never occurs on the face.

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Minor Criteria: see notes

Prognosis: depends on recurrences of rheumatic fever. Most common in first 5 years.

Except for carditis: all symptoms will subside without complications.

“Silent Infection”: Patient has sore throat d/t Strep infection, but it is not diagnosed. Patient may not
pay attention to sore throat: it may go silent for years, then murmur will present.

Diseases of the Cardio-vascular system: 5th note package

DDX LECTURE 19, NOVEMBER 22nd – PAGE 3
When the endocardium is involved, valves are impacted.
How many orifices in the heart?
• 2 A/V valves (mitral, tricuspid)
• Pulmonary artery (pulmonic valve)
• Aorta (aortic valve)

Pathologies are most common on mitral and aortic valves

MITRAL STENOSIS
Obstructive lesion in mitral valve caused by adhesions on leaflets of valve.
Consequence of RF (one reason)
Less blood flow between LA and LV as result.

Vegetations  Scar tissue  Calcification  will not open properly.

During atrial systole, not all blood empties into ventricle due to stenosis. During atrial diastole, new
blood comes in to atrium from pulmonary vein. Leads to dilation of left atrium. Over time, the
myocardium of left atrium dilates to its limit. Pressure begins to build in atrium (can’t stretch
anymore): blood backs up into lungs  congestion in pulmonary tree. Eventually, right ventricle is
also involved. Congestive heart failure.

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First signs of congestive heart failure? Shortness of breath due to congestion in pulmonary tree. Will
see this first on exertion.

Symptoms of mitral stenosis:

See notes.
Fatigue and dyspnea on physical exertion: due to congestion
Hemoptysis: from rupture of small vessels in pulmonary tree due to increased pressure. May be
sudden elevation of pressure (uncontrolled fibrillation)
Hoarseness: because left recurrent laryngeal nerve is compressed by dilated atrium.
Thrombi will occur due to stasis in left atrium. During fibrillation, thrombus may embolize and lodge in
the systemic circulation: brain, kidney, spleen and extremities.

Evidence of right-sided failure:

• JVP (Jugular venous pressure)
• Edema
• Enlargement of liver

Physical examination:
• Malar rash
• Arrythmias (irregular pulse)
• S1 will be accentuated, and you should be listening for a murmur. What kind? Diastolic, will
hear it in 5th intercostal space, at apex of heart (7-9cms from mid-sternal line)
• Hear it in diastole (note, this is ventricular diastole), because the valve is not able to open
properly. The orifice is narrowed, and the sound is heard.

DDX LECTURE 19, NOVEMBER 22nd – PAGE 4

KEY CHARACTERISTIC FEATURE OF MITRAL STENOSIS: DIASTOLIC MURMUR AT APEX
Also very localized to this area (also characteristic)
Best heard in left lateral decubitus position. Does not radiate!
Low pitched sound: use the bell of the stethescope.

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Normal size of orifice is 4-6cms. Don’t hear murmur until it has diminished to 1.5-2cm.

(Treatment of mitral stenosis: not on exam)

MITRAL INSUFFICIENCY OR MITRAL REGURGITATION

• The mitral valve isn’t closing properly. Should be closed completely during systole of ventricle. In
patients with mitral insufficiency, this doesn’t happen. Blood under high pressure regurgitates
back into the atrium on systole.
• Pressure in LA increases again, as in mitral valve stenosis.
• More blood is going into LV (no problem with the opening of valve). Leads to hypertrophy of LV.
• Can go for years without problem: heart compensates. Eventually, there are problems: heart
failure.

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• Some patients are asymptomatic. Others have symptoms of LV failure.

• May feel thrill and lift at apex.
• LV is responsible for apical impulse. Will note lateral, inferior displacement of apical impulse.
• Listen for murmur on systole, at apex (5th intercostal, 7-9cms from midline)
• Loud, blowing murmur that radiates to the left axilla.

DDX LECTURE 19, NOVEMBER 22nd – PAGE 5