Pulmonary Arterial Thrombosis in a Patient With an Atrial Septal Defect and Eisenmenger Syndrome

Pulmonary hypertension is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. A thromboembolic occlusion of the proximal or distal pulmonary vasculature results in chronic thromboembolic pulmonary hypertension. We report an uncommon case that presented to our hospital with symptoms of dyspnea on exertion over 2 years. The patient had been treated for profound pulmonary thrombosis and right ventricular failure with adequate anticoagulation and sildenafil. Our echocardiography disclosed a large atrial septal defect with severe pulmonary hypertension and right ventricular failure. A diagnosis of Eisenmenger syndrome with pulmonary artery thrombosis was made. Although Eisenmenger syndrome with pulmonary thrombosis is well described in western societies, a huge pulmonary thrombosis is seldom reported in eastern countries. Profound pulmonary thrombosis may obfuscate the actual diagnosis of pulmonary artery hypertension with underlying congenital heart disease. A physical examination and echocardiography are essential in patients with pulmonary hypertension.