Cardiology > Constrictive Pericarditis  

Background

Constrictive pericarditis is a chronic inflammatory disorder characterized by thickening, fibrosis, and often calcification of the pericardium, which restricts diastolic filling of the heart. This leads to symptoms of right-sided heart failure with preserved systolic function. The rigid pericardium prevents normal cardiac expansion during diastole, causing elevated and equalized diastolic pressures in all chambers. 

II) Classification/Types 

By Pathology: 

• • Idiopathic or Viral (most common in developed countries) 

• • Post-surgical or Post-radiation 

• • Tuberculous (most common in developing countries) 

• • Uremic, neoplastic, or autoimmune-related 

By Morphology: 

• • Chronic fibrosing pericarditis 

• • Calcific constrictive pericarditis 

By Coexisting Pathology: 

• • Effusive-constrictive pericarditis: Constriction occurs with concurrent pericardial effusion. 

III) Pathophysiology 

Pericardial injury from various causes initiates inflammation, leading to fibrin deposition, collagen fibrosis, and occasionally calcification. The resultant noncompliant pericardium restricts ventricular filling during mid-to-late diastole, leading to elevated venous pressures, decreased stroke volume, and dissociation of intrathoracic pressure transmission (ventricular interdependence). 

IV) Epidemiology 

• • Age: Typically seen in middle-aged and older adults 

• • Geography: 

• • • Developed countries: Idiopathic, viral, post-cardiac surgery, radiation 

• • • Developing countries: Tuberculosis is the leading cause 

• • • Sex: Slight male predominance 

Etiology

I) Causes 

• • • Idiopathic/viral (presumed viral pericarditis) 

• • • Post-cardiac surgery (e.g., CABG, valve replacement) 

• • • Radiation therapy (especially >35 Gy to mediastinum) 

• • • Tuberculosis 

• • • Autoimmune disease (SLE, RA, sarcoidosis) 

• • • Uremia 

• • • Neoplasia (e.g., breast, lung, lymphoma) 

• • • Post-infectious (bacterial, fungal) 

II) Risk Factors 

• • Prior cardiac surgery or radiation 

• • Tuberculosis exposure or history 

• • Autoimmune disease 

• • Malignancy 

• • Chronic kidney disease 

Clinical Presentation

I) History (Symptoms) 

• • Progressive fatigue and exercise intolerance 

• • Abdominal distension (ascites), early satiety 

• • Lower extremity edema 

• • Dyspnea, orthopnea 

• • Weight loss, cachexia in advanced disease 

• • History of pericarditis, TB, or chest radiation 

II) Physical Exam (Signs) 

Vital Signs: 

• • May be normal or show low pulse pressure 

Neck: 

• • Elevated jugular venous pressure (JVP) with prominent y descent 

• • Kussmaul’s sign: JVP rises with inspiration 

Cardiac: 

• • Pericardial knock: Early diastolic sound 

• • Muffled heart sounds 

Abdomen: 

• • Ascites, hepatomegaly, pulsatile liver 

Peripheral: 

• • Pitting edema, cool extremities 

Differential Diagnosis (DDx)

• • Restrictive cardiomyopathy 

• • Right-sided heart failure (e.g., pulmonary hypertension) 

• • Budd-Chiari syndrome 

• • Cirrhosis 

• • Cardiac tamponade 

• • Severe tricuspid regurgitation 

Diagnostic Tests

Initial Tests 

ECG: 

• • Low voltage QRS 

• • Nonspecific ST-T changes 

• • Atrial fibrillation (in advanced disease) 

Chest X-ray: 

• • Pericardial calcifications 

• • Small or normal heart size 

• • Pleural effusions 

Echocardiogram (TTE): 

• • Septal bounce (interventricular dependence) 

• • Biatrial enlargement 

• • Preserved EF 

• • Dilated IVC with reduced respiratory variation 

• • Pericardial thickening (may be limited by acoustic windows) 

Cardiac CT or MRI: 

• • Best modality to assess pericardial thickening (>4 mm), calcification 

• • Pericardial enhancement (active inflammation) 

Cardiac Catheterization (Definitive): 

• • Equalization of diastolic pressures in all chambers 

• • “Square root sign” or dip-and-plateau pattern in ventricular pressure tracing 

• • Discordance of LV and RV systolic pressures with respiration 

Laboratory Tests: 

• • ESR/CRP: May be elevated if active inflammation 

• • ANA, RF if autoimmune suspected 

• • TB testing (PPD, interferon gamma release assay) 

Treatment

I) Medical Management 

Trial of Anti-inflammatory Therapy (if active inflammation suspected): 

• • NSAIDs + Colchicine 

• • Corticosteroids in autoimmune or refractory cases 

• • May reverse early constrictive physiology 

Diuretics: 

• • Symptomatic relief of volume overload 

• • Use cautiously to avoid preload reduction and low cardiac output 

II) Interventional/Surgical 

Pericardiectomy (Definitive treatment): 

• • Surgical removal of pericardium 

• • Indicated in chronic constriction with persistent symptoms 

• • Requires median sternotomy or thoracotomy 

• • Operative mortality ~6–12%, higher in radiation or TB-related cases 

Patient Education, Screening, Vaccines

Education: 

• • Explain chronic nature and treatment options 

• • Need for lifelong follow-up after surgery 

• • Importance of early reporting of recurrent symptoms 

Lifestyle: 

• • Sodium restriction if fluid retention 

• • Monitor daily weights 

Vaccinations: 

• • Influenza 

• • Pneumococcal 

• • COVID-19 (especially in those immunocompromised or post-op) 

Consults/Referrals

• • Cardiology: For diagnostic evaluation and management 

• • Cardiothoracic Surgery: For pericardiectomy evaluation 

• • Infectious Disease: If TB or fungal etiology suspected 

• • Rheumatology: For autoimmune workup 

• • Nephrology: If uremic etiology suspected 

Follow-Up

• • Serial imaging (echo or MRI) if under conservative management 

• • Monitor volume status and adjust diuretics as needed 

• • Post-pericardiectomy: Long-term cardiac monitoring, re-evaluation of symptoms 

• • Prognosis: Improved symptoms after pericardiectomy in 80–90%, though functional recovery may take months