Endomyocardial fibrosis causing right intraventricular gradient and obliteration

Endomyocardial fibrosis is a cause of restrictive cardiomyopathy and it generally occurs in tropical regions more commonly affecting children and young adults. A 19-year-old male patient presented with edema in the lower extremities and fatigue. Transthoracic echocardiography showed dilated right heart chambers, restrictive physiology in the left ventricle, and increased tissue growth in the right ventricle that caused a 60-mmHg gradient and obliteration. Magnetic resonance imaging confirmed the presence of increased tissue formation in the right ventricular inflow region. Surgical resection was not considered taking into account the functional capacity of the patient (class II), disappearance of symptoms following medical treatment, and the high risk for operative mortality. The presented case may arouse interest in that increased tissue growth in the right ventricle inflow region caused a gradient in the right ventricle, leading to an incorrect diagnosis, at another center, as idiopathic pulmonary hypertension.