In developed countries, prior cardiac surgery or radiation therapy are major causative factors. A stool alpha-1-antitrypsin level was sent and was elevated, which confirmed the diagnosis of protein-losing enteropathy, a rare but important complication of constrictive pericarditis. This case highlights important diagnostic considerations and management of these complications for the general practitioner. == Table of Contents Summary: == We review a case of protein-losing enteropathy and secondary hypogammaglobulinemia in a patient that developed constrictive pericarditis secondary to an inflammatory myofibroblastic tumor. == Case Presentation == A 14-year-old previously healthy girl presented with a 3-week history of progressive lower extremity edema, abdominal distension, and weight gain. She denied shortness of breath, chest pain or orthopnea and did not have rashes or fevers. She endorsed mild dyspnea on exertion with running or climbing two flights of stairs. She was evaluated by her primary care physician and laboratory evaluation was notable for Rabbit Polyclonal to 5-HT-6 hypoalbuminemia (albumin 2.7, reference 3.7-5.6g/dL), elevated aspartate aminotransferase (AST 58, reference 10-40U/L), and normal urinalysis. An abdominal ultrasound demonstrated an inhomogeneous liver, small bilateral pleural effusions, and ascites. She was admitted to the hospital for further evaluation. Physical exam revealed a normal pulmonary evaluation, Gepotidacin but her cardiac exam was notable for jugular venous distention (10cm), positive Kussmaul sign, 2+ pitting edema extending to the knees, and a liver edge palpable 3cm below Gepotidacin the costal margin. Chest radiographs demonstrated a large soft tissue density along the left mediastinum causing a mass effect on the trachea and left main bronchus (Figure 1A). Further evaluation with a chest/abdomen/pelvis computed tomography (CT) demonstrated constrictive pericarditis, congestive hepatopathy, moderate pleural effusions with ascites, and mediastinal and hilar lymphadenopathy (Figure 1B). There were no splenic, liver, or pulmonary lesions. The echocardiogram revealed dilated atria with dilated hepatic veins and inferior vena cava, which failed to compress during inspiration concerning for elevated right-sided pressure, a finding that can be seen in restrictive cardiomyopathy and constrictive pericarditis (Figure 2). There was dyskinetic interventricular septal motion with abnormal tissue doppler indices supporting diastolic dysfunction with preserved systolic function. Exaggerated septal shifting with respiration, a pathognomonic finding for constrictive pericarditis, was also observed (Figure 2). To better differentiate between constrictive pericarditis and restrictive cardiomyopathy, the patient was referred for cardiac catheterization which revealed elevated but equal end diastolic pressures, with RVEDP=LVEDP=20mmHg (normally 3-8mmHg with RVEDP