Predominant Right Ventricular Dysfunction in Cardiac Sarcoidosis

Clinically significant cardiac sarcoidosis is rare, seen in 5% of patients with sarcoidosis. Predominant right ventricular (RV) dysfunction is seen even less often.

A 59-year-old man presented to the emergency department from his outpatient cardiologist's office with high-grade atrioventricular block and signs of right-sided heart failure. Transthoracic echocardiogram showed normal left ventricular systolic function, with severe RV enlargement and dysfunction. Right heart catheterization revealed mild postcapillary pulmonary hypertension and disproportionate RV dysfunction. A transbronchial biopsy of mediastinal lymph nodes showed noncaseating granulomas, consistent with sarcoidosis.

RV dysfunction in sarcoidosis is most often secondary to lung disease or left heart disease. In general, RV systolic dysfunction is associated with higher all-cause mortality in patients with sarcoidosis.

In patients with cardiac sarcoidosis and predominant RV dysfunction, clinical context along with multimodal imaging, particularly cardiac magnetic resonance imaging, can be useful to reach a diagnosis and provide prognostic value.