What is the appropriate diagnostic workup and management for constrictive pericarditis?

Constrictive Pericarditis: Diagnostic Workup and Management

Diagnostic Workup

Begin with transthoracic echocardiography as the first-line imaging modality in all patients with suspected constrictive pericarditis, looking specifically for septal bounce, respiratory variation of mitral E velocity >25%, and ventricular interdependence. 1

Initial Imaging Studies

• Obtain chest X-ray (frontal and lateral views) to identify pericardial calcifications, which suggest chronic disease but are absent in up to 20% of cases 1
• Perform CT and/or cardiac MRI as second-line imaging to assess pericardial thickness (normal <3.0 mm), detect calcifications, evaluate degree of pericardial inflammation via contrast enhancement, and measure extent of involvement 1, 2
• Normal pericardial thickness does not exclude constrictive pericarditis, as 18-20% of surgically-proven cases have thickness <3 mm 1, 2

Hemodynamic Assessment

• Proceed to cardiac catheterization when non-invasive methods are inconclusive, looking for the "dip and plateau" (square root sign) in ventricular pressure curves and equalization of left and right ventricular end-diastolic pressures within 5 mmHg 1
• Monitor intrapericardial and right heart pressures during pericardiocentesis to identify effusive-constrictive pericarditis, diagnosed when right atrial pressure fails to fall by 50% or below 10 mmHg after drainage 1

Key Diagnostic Features

The 2015 ESC Guidelines emphasize specific echocardiographic findings 1:

• Septal bounce and respiratory variation of mitral peak E velocity >25%
• E/A ratio >2 with short deceleration time
• Tissue Doppler peak e' >8.0 cm/s (helps differentiate from restrictive cardiomyopathy)
• Dilated inferior vena cava and hepatic veins with restricted respiratory fluctuations

Management Algorithm

Risk Stratification: Transient vs. Chronic Constriction

Immediately assess for features of chronicity versus potentially reversible disease upon diagnosis. 3

Chronic disease indicators (mandate surgery): 1, 3

• Cachexia, atrial fibrillation, hepatic dysfunction
• Pericardial calcification on imaging
• Symptoms present for prolonged duration

Transient constriction indicators (trial conservative management): 1, 3

• Elevated CRP indicating active inflammation
• Pericardial contrast enhancement on CT/CMR suggesting ongoing inflammation
• Recent onset pericarditis with mild effusion
• Hemodynamically stable presentation

Treatment by Clinical Scenario

For Hemodynamically Stable Patients Without Chronic Features

Initiate a 2-3 month trial of conservative management with anti-inflammatory therapy before recommending pericardiectomy. 1, 3, 2

• Use loop diuretics for volume overload and edema control 3, 4
• Consider empiric anti-inflammatory therapy with NSAIDs, colchicine, or glucocorticoids if active inflammation is present 3, 4
• Perform CT/CMR surveillance to monitor for resolution of pericardial inflammation 1
• CMR showing late gadolinium enhancement (LGE) pericardial thickness ≥3 mm has 86% sensitivity and 80% specificity for predicting reversible constriction 2

For Tuberculous Pericarditis

Administer rifampicin-based antituberculosis therapy for 6 months, which reduces progression to constriction from >80% to <10%. 3

• Add adjunctive prednisolone for 6 weeks in HIV-negative patients, reducing constrictive pericarditis incidence by 46% 3
• Avoid steroids in HIV-positive patients due to increased risk of HIV-associated malignancies 3

For Chronic Permanent Constrictive Pericarditis

Perform pericardiectomy for symptomatic patients (NYHA class III-IV) with persistent constriction after conservative management or those with chronic disease features. 1, 3, 4

• Complete pericardiectomy via midline sternotomy is the preferred approach, removing both parietal and visceral pericardium 3, 2, 4
• Operative mortality ranges from 6-12% 3, 2
• Early pericardiectomy before development of myocardial atrophy or fibrosis improves survival 3
• Use lateral thoracotomy for suppurative pericarditis to avoid sternal infection 4

For Effusive-Constrictive Pericarditis

Treat with the same approach as chronic constriction once identified, requiring visceral pericardiectomy. 1, 3

• This variant presents with both effusion and constriction, diagnosed when right atrial pressure remains elevated after pericardiocentesis 1
• Visceral pericardiectomy is technically challenging and should only be performed at experienced centers 1

Medical Therapy Alone (When Surgery Contraindicated)

Reserve medical therapy with loop diuretics for patients who are high surgical risk or have contraindications to pericardiectomy. 3, 2

• Medical therapy provides only temporary symptom relief and does not address the underlying constrictive process 3

Prognostic Factors

Poor surgical outcomes are predicted by: 3

• Prior mediastinal radiation
• Worse renal function and lower serum sodium
• Higher pulmonary artery systolic pressure
• Abnormal left ventricular systolic function (indicating myocardial fibrosis/atrophy)
• Older age and Child-Pugh score ≥7

Pericardial calcification does not impact survival 3

Critical Pitfalls to Avoid

• Do not delay surgery in appropriate candidates, as progression leads to myocardial atrophy and fibrosis, which significantly increases operative mortality and reduces benefit from pericardiectomy 1, 3
• Do not use vasodilators or diuretics aggressively in acute presentations without first establishing the diagnosis, as they can worsen hemodynamics 1
• Do not assume normal pericardial thickness excludes the diagnosis, as 18-20% of cases have normal thickness 1, 2
• Do not mistake constrictive pericarditis for primary liver disease, as ascites, hepatomegaly, and liver dysfunction are common presenting features that can mimic cirrhosis 1, 5
• Do not diagnose as HFpEF without investigating further when septal bounce/shudder is present on echocardiography, as constrictive pericarditis is potentially curable 6