Overview
Constrictive Pericarditis
1. Overview
Constrictive pericarditis is a condition where fibrotic thickening and calcification of the pericardium impairs diastolic filling of the heart. The rigid pericardium limits cardiac expansion, leading to impaired ventricular filling and symptoms of right-sided heart failure.
Key Features
- Mechanism: Thickened, non-compliant pericardium restricts cardiac filling
- Hemodynamics: Equalization of diastolic pressures across all chambers
- Hallmark Sign: Raised JVP with paradoxical rise on inspiration (Kussmaul's sign)
- Treatment: Surgical pericardiectomy is the definitive treatment
Common Etiologies
| Cause | Frequency | Comments |
|---|---|---|
| Idiopathic/Viral | 40-50% (developed world) | Most common in Western countries |
| Tuberculosis | Variable (>0% globally) | Leading cause worldwide |
| Post-cardiac surgery | 10-20% | Occurs months to years post-surgery |
| Radiation therapy | 5-10% | Often delayed presentation (years) |
| Connective tissue disease | 5% | RA, SLE, scleroderma |
| Post-pericarditis | Variable | After acute or recurrent pericarditis |
Key Clinical Pearl
"Right heart failure out of proportion to left heart failure" is the hallmark clinical presentation
2. Pathophysiology
┌─────────────────────────────────────────────────────────────────────────────┐
│ CONSTRICTIVE PERICARDITIS PATHOPHYSIOLOGY │
├─────────────────────────────────────────────────────────────────────────────┤
│ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ INITIAL INSULT │ │
│ │ • TB, Viral infection, Radiation, Surgery, Connective tissue │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ PERICARDIAL INFLAMMATION │ │
│ │ • Acute pericarditis → Chronic inflammation │ │
│ │ • Fibrin deposition and organization │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ FIBROSIS AND CALCIFICATION │ │
│ │ • Pericardium becomes thickened (>4mm) │ │
│ │ • Loss of elasticity and compliance │ │
│ │ • May develop calcification (especially TB) │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ IMPAIRED DIASTOLIC FILLING │ │
│ │ • Rigid pericardium limits ventricular expansion │ │
│ │ • Early diastole: Normal filling (until pericardium reached) │ │
│ │ • Mid-late diastole: Abrupt halt in filling ("dip and plateau") │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ VENTRICULAR INTERDEPENDENCE │ │
│ │ • Total cardiac volume is FIXED │ │
│ │ • Inspiration: ↑RV filling → ↓LV filling (septum shifts left) │ │
│ │ • Expiration: ↑LV filling → ↓RV filling │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────┬───────────────────────────────────────┐ │
│ ↓ ↓ │ │
│ ┌──────────────┐ ┌───────────────────────────────────────────┐ │ │
│ │ RIGHT HEART │ │ SYSTEMIC CONGESTION │ │ │
│ │ FAILURE │ │ • Elevated CVP/JVP │ │ │
│ │ │ │ • Hepatomegaly, Ascites │ │ │
│ │ │ │ • Peripheral edema │ │ │
│ └──────────────┘ └───────────────────────────────────────────┘ │ │
│ │ │
└─────────────────────────────────────────────────────────────────────────────┘
Key Hemodynamic Concepts
Equalization of Diastolic Pressures:
- LVEDP ≈ RVEDP ≈ RA ≈ LA ≈ Pulmonary wedge pressure
- All within 5 mmHg of each other
Dip and Plateau Pattern (Square Root Sign):
- Rapid early diastolic filling (dip)
- Abrupt cessation when pericardial limit reached (plateau)
- Creates "square root" appearance on pressure tracings
Ventricular Interdependence:
- In normal hearts: inspiration increases RV filling with minor LV change
- In constriction: LV filling DECREASES with inspiration (septum shifts left)
- Key difference from tamponade: ventricular interdependence is exaggerated
3. Clinical Features
History Taking
Symptoms to Elicit:
- Progressive dyspnea on exertion
- Fatigue and exercise intolerance
- Abdominal swelling (ascites)
- Bilateral leg swelling
- Anorexia, early satiety, nausea
- History of pericarditis, TB, cardiac surgery, radiation
Physical Examination Findings
| Sign | Description | Significance |
|---|---|---|
| Raised JVP | Prominent, may be very high | Right heart failure indicator |
| Kussmaul's Sign | JVP rises with inspiration | Highly specific for constriction |
| Pericardial Knock | High-pitched early diastolic sound | Abrupt halt in filling |
| Hepatomegaly | Tender, pulsatile | Hepatic congestion |
| Ascites | Often prominent | May precede peripheral edema |
| Peripheral Edema | Bilateral, pitting | Systemic venous congestion |
| Pulsus Paradoxus | Usually absent or mild (<10 mmHg) | Unlike tamponade |
The Classic Triad
- Elevated JVP with Kussmaul's sign
- Pericardial knock on auscultation
- Hepatomegaly and ascites (often out of proportion to peripheral edema)
JVP Waveform in Constriction
| Waveform | Finding | Explanation |
|---|---|---|
| x descent | Prominent | Rapid ventricular filling |
| y descent | Prominent, steep | Rapid early diastolic filling |
| Overall | "M" or "W" pattern | Combined prominent x and y |
4. Diagnosis
Diagnostic Approach
Clinical Suspicion:
- Right heart failure with normal or near-normal LVEF
- History of pericarditis, TB, cardiac surgery, or radiation
- JVP elevated with Kussmaul's sign
Investigations
ECG Findings:
- Low voltage QRS complexes
- Non-specific ST-T changes
- Atrial fibrillation (in 25-50%)
Chest X-Ray:
- Pericardial calcification (especially TB) - "eggshell" heart
- Normal or slightly enlarged cardiac silhouette
- Clear lung fields
Echocardiography:
| Finding | Description |
|---|---|
| Septal bounce | Abnormal septal motion with respiration |
| Respiratory variation | >5% variation in mitral inflow |
| Thickened pericardium | May be visible (not always) |
| Dilated IVC | Non-collapsing with inspiration |
| Preserved LVEF | Usually >0% |
CT/MRI:
| Modality | Findings |
|---|---|
| CT | Pericardial calcification, thickening >mm |
| MRI | Pericardial thickness, active inflammation, septal shift |
Cardiac Catheterization (Gold Standard for Hemodynamics):
| Finding | Description |
|---|---|
| Diastolic equalization | LVEDP ≈ RVEDP ≈ PA diastolic ≈ PCWP |
| Square root sign | Dip-and-plateau pattern |
| Elevated RA pressure | With preserved LV function |
| Discordant pressure changes | RV and LV peak pressures move in opposite directions |
Diagnostic Criteria
Major Criteria:
- Elevated JVP with Kussmaul's sign
- Pericardial thickening/calcification on imaging
- Characteristic hemodynamics on catheterization
5. Management Algorithm
┌─────────────────────────────────────────────────────────────────────────────┐
│ CONSTRICTIVE PERICARDITIS MANAGEMENT ALGORITHM │
├─────────────────────────────────────────────────────────────────────────────┤
│ │
│ SUSPECTED CONSTRICTIVE PERICARDITIS │
│ (Right heart failure, Raised JVP, Kussmaul's sign) │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ CONFIRM DIAGNOSIS │ │
│ │ • Echo: Septal bounce, respiratory variation, dilated IVC │ │
│ │ • CT/MRI: Pericardial thickening >4mm, calcification │ │
│ │ • Catheterization: Diastolic equalization, square root sign │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ EXCLUDE RESTRICTIVE CARDIOMYOPATHY │ │
│ │ • MRI: Myocardial characteristics, pericardial thickness │ │
│ │ • Catheterization: Discordant vs concordant pressure changes │ │
│ │ • Advanced echo: Strain imaging, tissue Doppler │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ IDENTIFY UNDERLYING CAUSE │ │
│ │ • TB testing (if endemic area or risk factors) │ │
│ │ • Autoimmune workup (ANA, RF) │ │
│ │ • History: Prior surgery, radiation, pericarditis │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌──────────────────────────────────────┐ │
│ │ TRANSIENT/REVERSIBLE CAUSE? │ │
│ └──────────────────────────────────────┘ │
│ ↓ YES ↓ NO │
│ ┌──────────────────────────┐ ┌──────────────────────────────────────┐ │
│ │ MEDICAL THERAPY TRIAL │ │ SURGICAL ASSESSMENT │ │
│ │ • Anti-inflammatory │ │ • Cardiothoracic surgery referral │ │
│ │ • Diuretics for symptoms│ │ • Operative risk assessment │ │
│ │ • Treat underlying cause│ │ • Consider timing │ │
│ │ • Monitor for 2-3 months│ │ │ │
│ └──────────────────────────┘ └──────────────────────────────────────┘ │
│ ↓ No improvement ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ PERICARDIECTOMY │ │
│ │ • Radical excision of pericardium (phrenic to phrenic) │ │
│ │ • Median sternotomy approach preferred │ │
│ │ • May require cardiopulmonary bypass │ │
│ │ • Post-op: Hemodynamic improvement often gradual │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ ↓ │
│ ┌─────────────────────────────────────────────────────────────────────┐ │
│ │ FOLLOW-UP │ │
│ │ • Clinical assessment at 1, 3, 6 months post-surgery │ │
│ │ • Echo to assess improvement in filling │ │
│ │ • Continue management of underlying cause │ │
│ │ • Monitor for recurrence (especially radiation-induced) │ │
│ └─────────────────────────────────────────────────────────────────────┘ │
│ │
└─────────────────────────────────────────────────────────────────────────────┘
Medical Management
Symptomatic Treatment:
- Diuretics: For congestion relief (use cautiously - preload dependent)
- Sodium restriction: Dietary modification
Cause-Specific Treatment:
- TB: Full anti-tuberculous therapy (6-9 months)
- Inflammatory: NSAIDs, colchicine (may help early/transient cases)
- Autoimmune: Immunosuppression as indicated
Surgical Treatment: Pericardiectomy
Indications:
- Symptomatic constrictive pericarditis
- Failed medical management
- Established fibrosis/calcification
Surgical Approach:
- Median sternotomy (preferred)
- Radical excision from phrenic nerve to phrenic nerve
- Cardiopulmonary bypass may be needed
Outcomes:
- Perioperative mortality: 5-15%
- Long-term survival: 70-80% at 10 years
- Higher mortality with radiation-induced constriction
6. Differentiating Constriction from Restriction
The Critical Distinction
| Feature | Constrictive Pericarditis | Restrictive Cardiomyopathy |
|---|---|---|
| Pericardium | Thickened/Calcified | Normal |
| Myocardium | Normal | Abnormal (infiltrative) |
| Pericardial knock | Present | Absent |
| Kussmaul's sign | Present | May be present |
| Septal bounce | Present | Absent |
| Treatment | Surgery (pericardiectomy) | Medical (limited options) |
Echo Findings
| Finding | Constriction | Restriction |
|---|---|---|
| Septal motion | Bounce/shift with respiration | Normal |
| Mitral inflow variation | >5% | <15% |
| Tissue Doppler e' | Normal or increased | Decreased |
| Annulus reversus | Present | Absent |
Catheterization Findings
| Finding | Constriction | Restriction |
|---|---|---|
| LV-RV pressure | Discordant with respiration | Concordant |
| RVEDP/LVEDP | Usually <1/3 LVEDP | RVEDP often >/3 LVEDP |
| Pulmonary systolic | Usually <50 mmHg | May be >0 mmHg |
MRI Features
| Finding | Constriction | Restriction |
|---|---|---|
| Pericardial thickness | >mm | Normal |
| Late gadolinium | Pericardial enhancement | Myocardial enhancement |
| Septal motion | Abnormal | Normal |
7. Prognosis
Natural History
- Without treatment: Progressive deterioration
- Mortality related to degree of cardiac cachexia and hepatic dysfunction
- Earlier surgery associated with better outcomes
Surgical Outcomes
| Factor | Impact on Outcome |
|---|---|
| Etiology | Radiation = worst prognosis |
| NYHA class | Higher class = higher mortality |
| Symptom duration | Longer = worse outcome |
| Atrial fibrillation | Associated with worse outcome |
| Hepatic dysfunction | Pre-op liver disease = higher risk |
Post-Pericardiectomy
| Outcome | Expected |
|---|---|
| Symptomatic improvement | 80-90% |
| Hemodynamic improvement | May be gradual (weeks to months) |
| 10-year survival | 70-80% (lower for radiation) |
| Recurrence | Rare if complete pericardiectomy |
8. Complications
Disease Complications
| Complication | Presentation | Management |
|---|---|---|
| Cardiac cirrhosis | Hepatomegaly, ascites, abnormal LFTs | Urgent pericardiectomy |
| Protein-losing enteropathy | Hypoalbuminemia, edema | Surgery if stable |
| Atrial fibrillation | Palpitations, embolic events | Rate/rhythm control, anticoagulation |
| Cardiac cachexia | Weight loss, muscle wasting | Nutritional support |
Surgical Complications
| Complication | Incidence | Notes |
|---|---|---|
| Perioperative mortality | 5-15% | Higher in radiation-induced |
| Low cardiac output | Variable | May need inotropic support |
| Bleeding | Variable | Especially with calcification |
| Phrenic nerve injury | Rare | Careful surgical technique |
| Incomplete relief | 10-20% | May need re-operation |
9. Special Considerations
Tuberculous Pericarditis
- Leading cause globally
- Requires 6-9 months anti-TB therapy
- Adjunctive corticosteroids controversial (may reduce constriction)
- Surgery if constriction develops despite treatment
Radiation-Induced
- Occurs years to decades after radiation
- Worst surgical outcomes (myocardial fibrosis may coexist)
- CAD often coexists
- Perioperative mortality up to 20%
Post-Cardiac Surgery
- Occurs in 0.2-0.3% of cardiac surgery patients
- Median presentation: 2-3 years post-surgery
- May be transient (respond to anti-inflammatory therapy)
- Trial of colchicine/NSAIDs reasonable before surgery
Transient Constrictive Pericarditis
- May occur after acute pericarditis
- Can resolve spontaneously or with anti-inflammatory therapy
- Trial of medical therapy for 2-3 months if suspected
- Surgery if no improvement
10. Key Clinical Pearls
Exam-Focused Points
- Kussmaul's Sign: JVP rises (paradoxically) with inspiration - highly specific
- Pericardial Knock: High-pitched early diastolic sound (abrupt halt in filling)
- Square Root Sign: Dip-and-plateau pattern on pressure tracings
- Septal Bounce: Characteristic echo finding with respiratory variation
- Discordant Pressures: LV and RV peak pressures move oppositely (vs concordant in restriction)
- TB is #1 Cause Globally: But idiopathic/viral common in developed countries
- Treatment is Surgery: Pericardiectomy is definitive - medical therapy is symptomatic only
- Radiation = Worst Prognosis: Highest surgical mortality
Common Exam Scenarios
- Right heart failure with normal LVEF and calcified pericardium
- Post-TB patient with raised JVP and hepatomegaly
- Comparison between constriction and restriction
- Interpretation of catheterization pressure tracings
11. Patient Explanation
What is Constrictive Pericarditis?
"Your heart is surrounded by a thin, flexible sac called the pericardium. In constrictive pericarditis, this sac has become thick and stiff - often due to a past infection, inflammation, or sometimes for unknown reasons.
The stiff sac acts like a rigid shell around your heart. This prevents your heart from expanding properly when it fills with blood. Think of it like trying to fill a balloon inside a glass jar - once the balloon touches the jar, it can't expand anymore.
This leads to blood backing up, particularly to your liver and legs, causing fluid buildup and swelling."
What Are the Symptoms?
"The main symptoms include:
- Swelling in your legs and belly
- Shortness of breath especially with activity
- Tiredness and feeling weak
- Loss of appetite as fluid builds up around your stomach
You might notice that your belly swells more than your legs - this is typical of this condition."
How is it Treated?
"The main treatment is surgery to remove the thickened pericardium (pericardiectomy). This frees your heart to expand properly again.
Medications (water pills/diuretics) can help reduce fluid buildup temporarily, but surgery is usually needed for long-term improvement.
After surgery, most people feel significant improvement, though it may take several weeks to months to notice the full benefit as your heart adjusts to working normally again."
12. Evidence & Guidelines
Key Guidelines
| Guideline | Organization | Year | Key Points |
|---|---|---|---|
| ESC Pericardial Diseases | European Society of Cardiology | 2015/2023 | Diagnostic criteria, management |
| AHA Scientific Statement | American Heart Association | 2020 | Multimodality imaging |
| STS Guidelines | Society of Thoracic Surgeons | 2019 | Surgical approach |
Key Evidence
Imaging Studies:
- Feng et al., JACC 2011: MRI criteria for distinguishing constriction from restriction
- Welch et al., Circulation 2010: Echo criteria for diagnosis
Surgical Outcomes:
- George et al., J Thorac Cardiovasc Surg 2007: Long-term outcomes after pericardiectomy
- Bertog et al., Circulation 2004: Perioperative predictors of outcome
Evidence-Based Recommendations
| Recommendation | Evidence Level |
|---|---|
| Echo as first-line imaging | Strong |
| CT/MRI for pericardial assessment | Strong |
| Cardiac catheterization for hemodynamics | Strong |
| Pericardiectomy for symptomatic constriction | Strong |
| Anti-inflammatory trial for transient constriction | Moderate |
13. References
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Adler Y, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases. Eur Heart J. 2015;36(42):2921-2964.
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Syed FF, Schaff HV, Oh JK. Constrictive pericarditis—a curable diastolic heart failure. Nat Rev Cardiol. 2014;11(9):530-544.
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Welch TD, et al. Echocardiographic diagnosis of constrictive pericarditis: Mayo Clinic criteria. Circ Cardiovasc Imaging. 2010;3(3):305-313.
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Bertog SC, et al. Constrictive pericarditis: etiology and cause-specific survival after pericardiectomy. J Am Coll Cardiol. 2004;43(8):1445-1452.
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George TJ, et al. Long-term outcomes of pericardiectomy for constrictive pericarditis. J Thorac Cardiovasc Surg. 2007;134(6):1528-1533.
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Feng D, et al. Cardiac magnetic resonance imaging pericardial late gadolinium enhancement and elevated inflammatory markers can predict the reversibility of constrictive pericarditis after antiinflammatory therapy. J Am Coll Cardiol. 2011;57(8):1502-1508.
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Mayosi BM, et al. Tuberculous pericarditis. Circulation. 2005;112(23):3608-3616.