Inflammation can lead to a thickened, fibrotic, adherent pericardium that restricts diastolic filling and produces chronically elevated venous pressures. In the past, tuberculosis was the most common cause of constrictive pericarditis, but the process now more often occurs after radiation therapy, cardiac surgery, or viral pericarditis; histoplasmosis is another uncommon cause.
The principal symptoms are slowly progressive dyspnea, fatigue, and weakness. Chronic edema, hepatic congestion, and ascites are usually present. The examination reveals these signs and a characteristically elevated jugular venous pressure with a rapid y descent. Kussmaul’s sign - an increase in jugular venous pressure during inspiration - occurs in constrictive pericarditis and restrictive cardiomyopathy. Pulsus paradoxus is unusual. Atrial fibrillation is common.
The chest x-ray may show normal heart size or cardiomegaly. Pericardial calcification is best seen on the lateral view and is common. Echocardiography can demonstrate a thick pericardium and small chambers. CT scans and MRI are helpful in revealing pericardial thickening and may be more sensitive than echocardiography.
The primary differential diagnoses are restrictive cardiomyopathy and tamponade. The former distinction can be difficult and is best made by evaluating left ventricular function (more consistently depressed in cardiomyopathy), measuring hemodynamics (which show more complete equalization of diastolic pressures in all four chambers in constrictive pericarditis), and demonstrating pericardial thickening and calcification.
Initial treatment consists of gentle diuresis. Surgical removal of the pericardium, which should be complete, is usually required in symptomatic patients but is associated with a relatively high mortality rate.
Asher CR et al: Diastolic heart failure: restrictive cardiomyopathy, constrictive pericarditis, and Cardiac tamponade: clinical and echocardiographic evaluation. Cardiol Rev 2002;10:218.
Hoit BD: Management of effusive and constrictive pericardial heart disease. Circulation 2002;105:2939.
Myers RB et al: Constrictive pericarditis: clinical and pathophysiologic characteristics. Am Heart J 1999;138:219.
Revision date: June 11, 2011
Last revised: by Jorge P. Ribeiro, MD