Cardiac sarcoidosis presenting as constrictive pericarditis

Abstract

In patients with cardiac sarcoidosis, the sarcoid granulomas usually involve the myocardium or endocardium. The disease typically presents as heart failure with ventricular arrhythmias, conduction disturbances, or both. Constrictive pericarditis has rarely been described in patients with sarcoidosis: we found only 2 reports of this association. We report the case of a 57-year-old man who presented with clinical and hemodynamic features of constrictive pericarditis, of unclear cause. He was admitted for treatment of recurrent pleural effusion. After a complicated hospital course, he underwent pericardiectomy. His clinical and hemodynamic conditions improved substantially, and he was discharged from the hospital in good condition. The pathologic findings, the patient's clinical course, and his response to pericardiectomy led to our diagnosis of cardiac sarcoidosis presenting as constrictive pericarditis. In addition to the patient's case, we discuss the nature and diagnostic challenges of cardiac sarcoidosis. Increased awareness of this disease is necessary for its early detection, appropriate management, and potential cure.

Keywords: Cardiomyopathies/complications/diagnosis/pathology; diagnosis, differential; diagnostic imaging; myocardium/pathology; pericarditis, constrictive/complications/etiology/surgery; sarcoidosis/complications/diagnosis/pathology; treatment outcome.

Fig. 1.

Right atrial pressure tracing shows rapid x and y descent.

Fig. 2.

Simultaneous recordings of left ventricular (LV) and right ventricular (RV) pressures show ventricular discordance. Note the decrease in RV systolic pressure during expiration (4th beat) and the marked rise at peak inspiration.

Fig. 3.

Photograph of the excised pericardium shows pink-tan to purple-gray thickened fibrous tissue adherent to yellow, lobulated adipose tissue.

Fig. 4.

Photomicrograph of the lymph node biopsy specimen shows nonnecrotizing and granulomatous inflammation and sclerosis (H & E, orig. ×100).

Fig. 5.

Cardiovascular magnetic resonance image shows late enhancement in the anteroseptal and basal inferolateral segments of the left ventricular myocardium (arrows).