Constrictive pericarditis in a patient with Wilson’s cirrhosis: difficulties in differential diagnosis

The clinical and diagnostic differentiation between constrictive pericarditis (CP) and restrictive cardiomyopathy is often difficult because of similar hemodynamic features. We present a 22-year-old male patient with CP, whose differential diagnosis was quite challenging. He had received treatment for Wilson’s disease, had a history of lung tuberculosis, and within the past two years, had undergone thoracotomy and decortication twice. He presented with complaints of dyspnea and swelling in the lower extremities. Computed tomography of the thorax showed pericardium of normal thickness, but pericardial calcification close to the right ventricle. On magnetic resonance imaging, pericardial thickness was 2 mm. Transthoracic echocardiography showed dilatation of the right and left atria. Cardiac catheterization revealed the square-root sign on ventricular diastolic pressure tracings, and a “y descent” on right atrial pressure tracings. Endomyocardial biopsy was performed which showed mild hypertrophic changes, without any signs of inflammatory infiltration, granuloma, amyloid deposition, or fibrosis. Biopsy sample from the pericardial tissue was not suggestive of a specific inflammatory process. He underwent pericardiectomy after which right atrium pressure decreased from 30 mmHg to 15 mmHg and his complaint of exertional dyspnea disappeared.

Keywords: Cardiomyopathies/diagnosis, diagnosis, differential; echocardiography, Doppler; heart catheterization; myocardium/ pathology; pericarditis, constrictive/diagnosis