Constrictive pericarditis

Definition

Constrictive pericarditis is marked inflammatory, fibrotic thickening and stiffening of the pericardium, often with calcium deposits; the visceral and parietal layers may adhere to each other or to the myocardium. The rigid pericardium impairs ventricular filling, and stroke volume and cardiac output fall. Systolic function is usually maintained.

Classification

• Subacute (early) constriction: weeks to months after an inciting event.
• Transient constriction: resolves again; mostly infectious, after pericardiotomy or idiopathic.
• Chronic constriction: permanent fibrotic, calcified form.
• Effusive-constrictive pericarditis: constriction by the visceral pericardium with a relevant effusion; constrictive hemodynamics persist after the effusion has been evacuated.

Aetiopathogenesis

• High-resource countries: causes similar to acute pericarditis – idiopathic or viral, prior pericardiotomy, prior chest exposure to ionising rays.
• Worldwide: tuberculous pericarditis as the most common cause.
• Others: purulent pericarditis, systemic rheumatic diseases, hemopericardium; some cases occur without preceding acute pericarditis.
• Pathophysiology: diastolic pressures in the ventricles, atria and veins become virtually equal. Systemic venous congestion causes transudation with edema and later ascites; chronically raised hepatic venous pressure can cause cardiac cirrhosis. Arrhythmias, particularly atrial fibrillation, are common.

Clinical features

• signs of peripheral venous congestion: peripheral edema, distended neck veins, hepatomegaly, later ascites; some patients are first investigated for liver cirrhosis
• Kussmaul's sign: rise in venous pressure on inspiration (absent in tamponade)
• pericardial knock: early diastolic extra sound, often best heard during inspiration
• exertional dyspnea and orthopnea; marked fatigue
• pulsus paradoxus uncommon and less pronounced than in tamponade; lungs usually not congested
• occasionally pleural effusion, atrial fibrillation

Diagnosis

• Echocardiography: inspiratory fall in mitral inflow velocity usually above 25 %; exaggerated inspiratory tricuspid inflow; increased tissue Doppler velocity e' at the mitral annulus, especially septal – a septal e' higher than lateral e' (annulus reversus) is highly specific for constriction; respiratory septal shift; expiratory diastolic flow reversal in the hepatic veins; plethoric vena cava. Septal shift combined with increased medial e' or hepatic vein flow reversal reaches a sensitivity of 87 % and a specificity of 91 % (Mayo criteria).
• Cardiac catheterisation (right and left): pulmonary artery occlusion pressure, pulmonary artery diastolic pressure, right ventricular end-diastolic pressure and mean atrial pressure roughly equal at about 10–30 mmHg; accentuated x and y descents; "dip-and-plateau" (square root sign) of the ventricular pressure curve; discordant respiratory variation of the systolic pressures of both ventricles.
• CT and MRI: pericardial thickening above 4 mm with typical hemodynamics confirms the diagnosis; a normal pericardial thickness does not exclude it. MRI additionally assesses inflammatory activity and hemodynamics.
• ECG: usually low voltage, non-specific T-wave changes, atrial fibrillation in some patients.
• Chest X-ray: pericardial calcification, best seen on the lateral view (non-specific).

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Further reading (open access)

1. MSD Manual Professional: Pericarditis
2. StatPearls: Constrictive Pericarditis

Cross-references