“Malignant” Chronic Constrictive Pericarditis

A 32-year-old woman who suffered from acute myopericarditis after an upper respiratory infection when she was 15 years old was diagnosed 4 years later with restrictive cardiomyopathy. Ten years later, she became symptomatic, with episodes of right-sided heart failure, lower extremity edema, hepatosplenomegaly, and mild ascites. Echocardiography revealed normal right and left ventricular function without tricuspid regurgitation but with severe dilation of both atrial chambers. Cardiac catheterization revealed the presence of the square root sign with equalization of right and left ventricular end-diastolic pressures, together with modest elevation in right ventricular systolic pressure (40 mmHg) and absence of respiratory variations. Finally, contrast-enhanced thoracic computed tomography revealed severe concentric pericardial calcification with partial infiltration of the myocardial free wall of the right ventricle with a maximum width of 10 mm (Figure 1, 2). The patient was finally diagnosed with constrictive pericarditis. Our multidisciplinary team decided to inform the patient to undergo cardiac transplantation.

Figure 1.

Sagittal contrast-enhanced thoracic computed tomography scan showing severe concentric pericardial calcification with partial infiltration of the myocardial free wall of the right ventricle with a maximum width of 10 mm

Figure 2.

Volume rendering of the contrast-enhanced thoracic computed tomography scan. The red arrow shows the severe calcification of the myocardial free wall