The syndrome of inflammatory changes endocardium, a myocardium and a pericardium (the main complaints; physical, laboratory and instrumental criteria of diagnostics): - acute rheumatic fever; - myocarditis, cardiomyopathy; - pericarditis (acute and constrictive)
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Inflammatory Syndromes of the Heart: Endocardium, Myocardium, and Pericardium
I. ACUTE RHEUMATIC FEVER (ARF)
Pathophysiology
ARF is a multisystem autoimmune disease triggered by Group A Streptococcus (Streptococcus pyogenes) pharyngitis. Molecular mimicry between streptococcal antigens and cardiac tissue causes inflammation of all three layers of the heart. The most clinically significant sequela is rheumatic heart disease (RHD) — permanent valvular damage, most commonly mitral stenosis.
(Harrison's, p. 10063)
Main Complaints
- Migratory polyarthritis (most common early complaint)
- Carditis: palpitations, dyspnea, chest discomfort
- Skin rash, subcutaneous nodules
- Chorea (involuntary movements)
- Fever, malaise, fatigue
Diagnostic Criteria — Revised Jones Criteria (2015, AHA)
Requires evidence of preceding GAS infection PLUS:
Two major criteria OR one major + two minor criteria
| Major Criteria | Minor Criteria |
|---|---|
| Carditis (clinical or subclinical/echocardiographic) | Fever (>38.5°C) |
| Polyarthritis | Elevated ESR (≥60 mm/hr) or CRP (≥3 mg/dL) |
| Chorea | Prolonged PR interval on ECG |
| Erythema marginatum | Polyarthralgia (only if arthritis not used as major) |
| Subcutaneous nodules |
For high-risk populations (developing countries), monoarthritis/arthralgia may count as a major criterion.
Physical Examination Findings
- Cardiac auscultation: New murmur (mitral regurgitation — apical blowing holosystolic; Carey-Coombs mid-diastolic murmur in severe carditis)
- Pericardial friction rub (pericarditis component)
- Tachycardia out of proportion to fever
- Signs of heart failure in severe carditis (gallop rhythm, pulmonary crackles)
- Sydenham's chorea: irregular, purposeless movements of hands, face
- Erythema marginatum: transient, map-like rash with central clearing
- Subcutaneous nodules over bony prominences (Aschoff nodules)
Laboratory Criteria
| Test | Finding |
|---|---|
| Throat culture / Rapid Strep antigen | Positive GAS (may be negative by onset of ARF) |
| ASO titer (Anti-streptolysin O) | Elevated (>200 Todd units in adults) |
| Anti-DNase B | Elevated (better for skin infections) |
| ESR | Markedly elevated |
| CRP | Elevated |
| CBC | Leukocytosis, normocytic anemia |
| Troponin / CK-MB | Elevated in carditis |
Instrumental (ECG & Imaging) Criteria
ECG:
- Prolonged PR interval (1st-degree AV block) — most common
- Sinus tachycardia
- ST-T changes (pericarditis component)
Echocardiography:
- Mitral regurgitation (most common valvular lesion in acute phase)
- Aortic regurgitation
- Pericardial effusion
- Subclinical carditis detectable only on echo (pathological MR by Doppler)
Chest X-ray:
- Cardiomegaly in moderate-severe carditis
- Pulmonary congestion in heart failure
II. MYOCARDITIS AND CARDIOMYOPATHY
Pathophysiology
Myocarditis = inflammation of the myocardium. Most commonly viral (Coxsackievirus B, parvovirus B19, CMV, HIV, COVID-19), but also autoimmune, bacterial, drug/toxin-induced. Chronic myocarditis may progress to dilated cardiomyopathy (DCM).
(Management of Myocarditis and Pericarditis, p. 68)
Main Complaints
- Chest pain (often sharp, pleuritic, especially when pericarditis coexists)
- Dyspnea (exertional or at rest in heart failure)
- Palpitations, syncope (arrhythmias)
- Fatigue, reduced exercise tolerance
- Prodromal flu-like illness (fever, myalgia, URI symptoms) 1–4 weeks prior
- Fulminant myocarditis: rapid hemodynamic collapse
Physical Examination
- Tachycardia (disproportionate to fever)
- S3/S4 gallop rhythm
- Mitral regurgitation murmur (annular dilation)
- Signs of low cardiac output: hypotension, cool extremities
- Jugular venous distension, peripheral edema (right heart failure)
- Pericardial friction rub (if myopericarditis)
- Low-grade fever
Laboratory Criteria
| Test | Finding |
|---|---|
| Troponin I/T | Elevated (myocardial necrosis) |
| CK-MB | Elevated |
| BNP / NT-proBNP | Elevated (HF marker) |
| ESR, CRP | Elevated (inflammation) |
| CBC | Leukocytosis, eosinophilia (eosinophilic myocarditis) |
| Viral serology / PCR | Coxsackievirus, parvovirus B19, CMV, HIV |
| ANA, ANCA, anti-ds-DNA | Autoimmune causes |
| Endomyocardial biopsy | Gold standard — lymphocytic infiltration (Dallas criteria); recommended in intermediate/high-risk cases |
Instrumental Criteria
ECG:
- Sinus tachycardia (most common)
- ST elevation (diffuse, concave/saddle-shaped) or ST-T changes
- New-onset LBBB or RBBB
- PR depression
- Ventricular ectopy, non-sustained VT
- AV block (giant cell myocarditis, sarcoidosis, Lyme)
Echocardiography:
- Reduced LV ejection fraction (LVEF)
- Global or regional wall motion abnormalities
- LV dilation (dilated cardiomyopathy pattern)
- Diastolic dysfunction
- Pericardial effusion (if myopericarditis)
- Sometimes: hyperechogenic myocardium, thickened walls (inflammatory edema)
Cardiac MRI (CMR) — the non-invasive gold standard:
- Lake Louise Criteria: T2-weighted edema + late gadolinium enhancement (LGE) with non-ischemic (epicardial/midwall) pattern
- Distinguishes myocarditis from ischemic cardiomyopathy
Chest X-ray:
- Cardiomegaly
- Pulmonary vascular congestion / pulmonary edema
Cardiomyopathy (Brief Summary)
Chronic myocardial disease resulting from myocarditis, genetic, or systemic causes:
| Type | Key Feature |
|---|---|
| Dilated (DCM) | Enlarged, poorly contracting LV; EF <40% |
| Hypertrophic (HCM) | Asymmetric septal hypertrophy, dynamic outflow obstruction |
| Restrictive (RCM) | Normal size, stiff walls, diastolic dysfunction |
| Arrhythmogenic (ARVC) | RV fatty infiltration, VT, sudden death |
III. PERICARDITIS — ACUTE AND CONSTRICTIVE
Pathophysiology
Acute pericarditis = inflammation of the pericardial sac. Most common cause: idiopathic/viral. Other causes: bacterial (purulent), TB, autoimmune (SLE, RA), post-MI (Dressler's), uremia, malignancy, drugs.
Constrictive pericarditis = fibrous thickening and calcification of the pericardium restricting diastolic filling (often sequela of TB, radiation, purulent, or recurrent pericarditis).
(Management of Myocarditis and Pericarditis, p. 68)
Main Complaints — Acute Pericarditis
- Sharp, pleuritic chest pain — key feature; worse lying flat, relieved sitting forward (tripod position)
- Radiation to trapezius ridge (pathognomonic for pericarditis)
- Dyspnea (from pain, effusion, or tamponade)
- Low-grade fever
- If cardiac tamponade develops: severe dyspnea, hypotension, elevated JVP
Main Complaints — Constrictive Pericarditis
- Progressive dyspnea and exercise intolerance
- Lower limb edema, ascites (right-sided failure dominates)
- Fatigue, weight loss (protein-losing enteropathy in severe cases)
Physical Examination
Acute pericarditis:
- Pericardial friction rub — pathognomonic; best heard at left sternal border, leaning forward; scratchy, "to-and-fro," 3-component (atrial systole, ventricular systole, diastole)
- Tachycardia
- Tamponade: Beck's triad (hypotension + elevated JVP + muffled heart sounds), pulsus paradoxus (>10 mmHg fall in SBP on inspiration)
Constrictive pericarditis:
- Elevated JVP with Kussmaul's sign (JVP rises on inspiration — paradoxical)
- Pericardial knock — early diastolic sound (S3 equivalent, louder than S3, earlier timing)
- Hepatomegaly, ascites, peripheral edema
- Pulsus paradoxus (less common than tamponade)
- Friedreich's sign: rapid y-descent of JVP
Laboratory Criteria
| Test | Acute | Constrictive |
|---|---|---|
| ESR, CRP | Elevated | Usually normal or mildly elevated |
| Troponin | Elevated in myopericarditis | Normal |
| CBC | Leukocytosis | May show secondary polycythemia or hypoalbuminemia |
| Pericardial fluid analysis | Exudate (protein, LDH, glucose, cytology, culture) | N/A (no effusion) |
| ANA, RF, ANCA | Positive in autoimmune | — |
| TSH | Rule out hypothyroid | — |
| Creatinine/BUN | Uremic cause | — |
| Blood cultures, Quantiferon-TB | Infectious causes | TB workup |
Instrumental Criteria
ECG — Acute Pericarditis (4 Classical Stages):
| Stage | Timing | ECG Changes |
|---|---|---|
| I | Days 1–2 | Diffuse saddle-shaped ST elevation in all leads except aVR, V1 (concave up); PR depression |
| II | Days 3–7 | ST returns to baseline; PR depression |
| III | 1–3 weeks | Diffuse T-wave inversions |
| IV | Weeks–months | Normalization |
PR depression in II, aVF, V4–V6 with PR elevation in aVR is highly specific for pericarditis.
ECG — Constrictive Pericarditis:
- Low voltage
- Non-specific ST-T changes
- Atrial fibrillation (common)
Echocardiography:
| Finding | Acute Pericarditis | Constrictive Pericarditis |
|---|---|---|
| Pericardial effusion | Present (variable) | Absent/minimal |
| Pericardial thickening | May be present | Characteristic (>4 mm) |
| LV/RV function | Usually normal | Normal EF, diastolic restriction |
| Respiratory variation in E-wave | Absent | Exaggerated (>25%) — key sign of interdependence |
| IVC | Normal | Dilated, non-collapsing |
| Septal bounce | Absent | Present (interventricular dependence) |
| Tamponade signs | RV collapse in diastole, RA collapse | Not applicable |
CT/MRI:
- CT: pericardial calcification (classic in constrictive, especially TB), thickening
- CMR: inflammation (pericardial LGE in acute); thickened pericardium in constrictive
Chest X-ray:
- Acute: enlarged cardiac silhouette (if large effusion — "water bottle heart")
- Constrictive: pericardial calcification (best on lateral view), normal heart size
Cardiac Catheterization (constrictive):
- Equalization of diastolic pressures across chambers (RVEDP = LVEDP = RVDP = LVDP ~20 mmHg)
- "Square root sign" (dip-and-plateau) in ventricular pressure tracing
- Discordance of LV/RV pressure during respiration
Summary Comparison Table
| Feature | ARF | Myocarditis | Acute Pericarditis | Constrictive Pericarditis |
|---|---|---|---|---|
| Etiology | GAS pharyngitis | Viral, autoimmune | Viral, idiopathic, TB | Prior pericarditis, TB, radiation |
| Key complaint | Migratory arthritis + carditis | Chest pain, dyspnea, arrhythmia | Sharp positional chest pain | Progressive right HF |
| Key exam sign | Carey-Coombs murmur | S3 gallop | Friction rub | Kussmaul's sign + pericardial knock |
| ECG hallmark | Prolonged PR | ST changes, arrhythmias | Diffuse concave ST↑, PR↓ | Low voltage, AF |
| Key lab | ASO titer, CRP | Troponin, BNP, biopsy | CRP, troponin (if myopericarditis) | Normal or mildly elevated CRP |
| Key imaging | Echo (MR, effusion) | CMR (LGE midwall) | Echo (effusion), ECG stages | Echo (septal bounce, respiratory variation), CT (calcification) |
| Unique sign | Aschoff nodules (biopsy) | Non-ischemic LGE on CMR | PR depression on ECG | Equalization of diastolic pressures on cath |
Sources: Harrison's Principles of Internal Medicine, 21st Ed. (p. 10063); Management of Myocarditis and Pericarditis — ESC Guidelines (p. 68); supplemented by ACC/AHA 2015 Revised Jones Criteria and ESC 2015 Pericardial Diseases Guidelines.
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