Right‐Sided Endomyocardial Fibrosis with a Right Atrial Thrombus: Three‐Dimensional Transthoracic Echocardiographic Evaluation

• Published in: Echocardiography (Mount Kisco, N.Y.) N.Y.), 2013-11, Vol.30 (10), p.E322-E325
• Main Authors: Kharwar, Rajiv Bharat, Sethi, Rishi, Narain, Varun Shankar
• Format: Article
• Language: English
• Subjects: Child; Echocardiography; Echocardiography, Three-Dimensional; Endomyocardial Fibrosis - complications; Endomyocardial Fibrosis - diagnostic imaging; endomyocardium; Female; Heart Atria - diagnostic imaging; Heart Diseases - complications; Heart Diseases - diagnostic imaging; Humans; live three‐dimensional transthoracic echocardiography; restrictive cardiomyopathy; Thrombosis - complications; Thrombosis - diagnostic imaging
• ISSN: 0742-2822; 1540-8175
• DOI: 10.1111/echo.12332

Endomyocardial fibrosis is a form of restrictive cardiomyopathy mainly affecting poor children and young adults in geographically restricted areas of Latin America, Africa, and Southeast Asia. The pathophysiological hallmark of the disease is focal or diffuse endocardial thickening involving mainly the inflow, the apices, and the subvalvular region leading to valvular regurgitation, diastolic dysfunction and obliteration of the ventricular apex. Advanced right‐sided disease has slow flow of blood through chambers with propensity of thrombus formation especially in the right atria. Although two‐dimensional transthoracic echocardiography remains the cornerstone for the diagnosis of this disease, the case presented here shows how three‐dimensional transthoracic echocardiography can add substantial information regarding the region of involvement of the right ventricle as well as the various characteristics of the right atrial thrombus. Mini‐ We report a case of a female child presenting to us with complaints of effort dyspnea and gradually increasing abdominal distension of 3 months duration. Two‐dimensional transthoracic echocardiography showed large pericardial effusion, obliterated right ventricular cavity with low pressure tricuspid regurgitation and a large mass in a hugely enlarged right atria. A diagnosis of endomyocardial fibrosis involving the right heart was contemplated. Three‐dimensional transthoracic echocardiography clearly showed the characteristic involvement of the inflow and the apex and sparing of the outflow tract region of the right ventricle. The right atrial mass was confirmed to be a thrombus. Patient was referred for possible corrective surgery.