A CASE OF PREGNANCY, HYPOXIA, AND TOO MUCH PROTEIN. R.A. Andrews 1 , E.L. Salerno 2 ; 1 University of Connecticut, Farmington, CT; 2 University of Connecticut, Columbia, CT (Tracking ID #76994) LEARNING OBJECTIVES: 1) Discuss diagnosis, eitiology, and management of pulmonary alveolar proteinosis. CASE INFORMATION: A.G. is a 29 year old female 15 weeks into her fourth pregnancy who presents for diagnosis and treatment of severe hypoxia. Ten weeks prior to admission, she begins having progressive dyspnea on exertion, non-productive cough, weakness, fatigue, nausea, and diarrhea, but no fevers and occassional chills. Two days prior to admission she is referred to the emergency room for severe hypoxia. Patient denies problems with previous pregnancies and is HIV negative. Past medical history and social history are essentially unremarkable. At presentation, she is afebrile with a blood gas on room air that shows a PaO2 of 64. Cardiovascular exam is benign, but diffuse course rales are heard on lung exam. The patient has perioral and acral cyanosis and clubbing. Chest xray shows a diffuse patchy alveolar infiltrate. HCT is 49.3 with normal chemistries and negative D-dimer. Patient is placed on a non-rebreather mask at 100% and started on broad spectrum antibiotics. On day two, she is transferred to intensive care with a PaO2 of 54 on 100% oxygen. Video-assisted thorascopic biopsy is obtained providing the diagnosis of pulmonary alveolar proteinosis. Patient then undergoes whole-lung lavage with improvement in chest xray and PaO2 saturation. IMPLICATIONS/DISCUSSION: Pulmonary alveolar proteinosis (PAP) is a non-inflammatory disease of the lungs affecting men more than women (2:1) typically between 30 and 50 years of age. The primary defect is either a failure to degrade, or increased production, of surfactant causing an accumulation of amorphous proteinaceous material within the alveoli. The exact eitiology remains unclear. Patients generally present with dyspnea on exertion, 30% are asymptomatic. Common symptoms are dry cough, weight loss, malaise and fatigue. Rales are present in 50% of cases. Typical xray findings reveal perihilar alveolar infiltrates with typical``bat wing'' appearance. Diagnosis is made from BAL (bronchioalveolar lavage) or lung biopsy. Lavage produces a milky effluent composed of amorphous granular material that stains PAS (periodic acid-Schiff) positive. On biopsy, inflammatory cells are rare and there is no fibrosis. Alveoli are congested with proteinaceous material and dysfunctional macrophages. Standard of care is whole-lung lavage with 30±40% of patients resolving with only one lavage while the majority of patients require repeat lavage at 6±12 month intervals. Newer treatments include high doses of GM-CSF (granulocyte macrophage-colony stimulating factor) and possibly bone marrow transplant. Prognosis of PAP has been shown to be a 74% 5-year survival. LEARNING OBJECTIVES: 1. Recognize the long term complications after heart surgery for congenital defect in childhood. 2. Appreciate that patients living in precarious conditions need careful management that goes beyond an initial impression, although the risk of somatizing exists. CASE INFORMATION: A 25 year old male asylum-seeker, living in Switzerland for a month, with a history of surgical repair of a pentalogy of Fallot 20 years before with no subsequent complications, presented with breathing-related chest pain responsive to paracetamol, exertional dyspnoea and orthopnoea ± on the same day that five of his compatriots living in the same asylum-seeker centre located in a public bomb shelter with the same symptoms! He was in good general health, his blood pressure was 93/66 mmHg, and he had a regular heart rate of 100 beats/min and a respiratory rate of 25/min. His chest was slightly asymmetrical, with slight arching of the left hemithorax, the sternotomy scar appeared normal, the apical impulse was widened and pulsations were apparent across the whole sternal and left parasternal area (Harzer's sign). Auscultation revealed a holosystolic, non-radiating crescendo murmur of 3/6 in the left parasternal area. Diastole was free and peripheral arteries were normal. Laboratory tests were within normal limits, excluding acute conditions such as pulmonary embolism or cardiac ischaemia. The ECG revealed sinus tachycardia, a vertical frontal axis of the QRS complex and complete right bundle branch block. A transthoracic echocardiogram revealed a voluminous mass with an echo-dense border and an echo-poor centre adjacent to the free wall of the right ventricle, no vascularized (free of internal blood flow), exerting an obvious compression on the right ventricle, the intraventricular pressure being estimated at 80 mmHg based on the high-velocity severe tricuspid regurgitation. Magnetic resonance imaging and computed transverse tomography confirmed the presence of a hypodense mass, in close contact with the pericardium (Fig. 1 & 2) . The severe compression of the right ventricle prompted the surgical removal of this mass. The mass was adhering to the rib cage and the free wall of the right ventricle. Bacteriological investigation revealed the presence of an abscess with coagulase-negative staphylococci. Postoperative progress was uneventful, and the medium-term outcome favourable. No abnormal cavity was visualized during transthoracic echocardiography at the 3-month postoperative control. IMPLICATIONS/DISCUSSION: While infections are known to occur after sternotomy, usually involving coagulase-negative staphylococci, the formation of an abscess in the anterior mediastinum several years after the intervention appears to be exceptional. This diagnosis came to mind only after the more common postoperative complications, e.g. pseudoaneurysm or pericardial haematoma, and the wide range of possible diagnoses covering tumours, infections and malformations of the anterior mediastinum had been considered. Although the signs and symptoms were not very specific and potentially psychogenic in a patient living in precarious conditions, we were inclined to consider a wide range of differential diagnoses, given the patient's history.