Isolated right heart failure (RHF) can be caused by right ventricular (RV) compression, mainly described in severe cases of pectus excavatum. Mediastinal masses can determine an extrinsic compression of the RV.
A 62-year-old retired agricultural inspector from Sicily presented in our clinic with worsening signs of RHF. His medical history consisted of echinococcosis familiarity, surgically treated congenital subvalvular aortic stenosis, resulted in a residual moderate aortic insufficiency and permanent atrial fibrillation. Around January 2021 he developed isolated leg swelling. Aortic valvular dysfunction, heart failure and other cardiogenic explanations were excluded.
A year later dyspnea (NYHA class II) appeared. High resolution CT demonstrated fluid collection with peripheric calcifications inside the anterior mediastinum. PET showed uptake in a limited zone of the anterior mediastinum. The patient slowly started developing ascites, hardly manageable with diuretics because of kidney failure. Transthoracic echocardiography (TTE) highlighted mild RV dysfunction (FAC 35%) and right ventricular free wall hypokinesia due to the mediastinal mass extrinsic compression. Transesophageal echocardiography showed a heterogeneous cystic aspect of the mediastinal mass, an oscillating intraventricular mass of the right aortic cuspid (6×4 mm) and a severe mitral annular calcification. In consideration of medical history, endocarditis was considered. Cardiac magnetic resonance recognized a mediastinal mass with echinococcosis features. Echinococcous serology and antibodies were negative but the Quantiferon tested positive. Laboratory tests showed an absolute eosinophilia and a high CD4/CD8 ratio. Because of the patient severely compromised hemodynamic balance the mass was surgically removed and identified as pseudocystic tuberculoma. After the excision, hemodynamic balance was improved and the TTE showed a restored RV function with persistent hypokinesia of the RV free wall and increased right atrium pressure.
In this clinical case RHF was determined by an extracardiac mass which interfered with the hemodynamic balance and the function of the right heart in the absence of any signs of left heart failure. Because of the mass mechanical action on the reduction of RV ejected volumes, a surgical option was the only possible strategy to avoid a following worsening of the heart failure.