COPD -Chronic Obstructive Pulmonary Disease  Scientific Abstracts   Society of General Internal Medicine: 28th Annual Meeting New Orleans, LA May 11–14, 2005: Out of Chaos: The Critical Role of Generalists, ABSTRACTS OF SUBMISSIONS ACCEPTED FOR PRESENTATIOIN   CLINICAL VIGNETTES   CLINICAL VIGNETTES  LEARNING OBJECTIVES: Recognize that the presenting features of amyloidosis are protean and depend on the organ systems involved. Recognize that cardiac involvement is common in primary amyloidosis and that symptomatic heart involvement carries a poor prognosis. CASE INFORMATION: 66 year old man without PMH presented 8 months of progressive exertional dyspnea and fatigue. Evaluation revealed volume overload with anasarca and signs of biventricular heart failure. Laboratory studies revealed renal insufficiency with a creatinine of 1.8 and 2+ proteinuria. serum protein electrophoresis with immunofixation showed a monoclonal IgA kappa paraprotein; urine protein electrophoresis was unremarkable. Echocardiogram revealed an ejection fraction of 20%. Right ventricular function was moderately reduced and right and left ventricular hypertrophy was noted. ECG showed atrial fibrillation with a ventricular response of 105 BPM and low voltage. Pt. underwent right heart catheterization with endomyocardial biopsy. Tissue stained positive with crystal violet and Congo red with characteristic green birefringence under polarization microscopy. This result together with the paraprotein established the diagnosis of AL amyloidosis. IMPLICATIONS/DISCUSSION: Amyloidosis is a disease characterized by extracellular accumulation of insoluble protein fibrils in various organs and tissues. Classification of amyloidosis is based on the nature of the precursor proteins that form the fibril deposits. AL amyloidosis (primary) is a plasma cell dyscrasia in which clonal plasma cells in the bone marrow produce monoclonal immunoglobulin light chains. Clinical manifestations reflect the organ systems involved with kidney and heart involvement being most common. Clinically evident cardiac involvement occurs in up to 50% of patients with AL amyloid and often leads to significant cardiac dysfunction. Diastolic dysfunction is caused by diffuse infiltration of the left ventricular myocardium with resultant stiffening and impaired relaxation and usually precedes systolic dysfunction. Systolic dysfunction results from replacement of functional myocardial tissue with amyloid protein. The conduction system can be affected by amyloid infiltration as well, leading to conduction disturbances, arrhythmias or sudden death. ECG findings include low voltage, conduction abnormalities such as AV block, atrial fibrillation and pseudoinfarct patterns. This case demonstrates a classic presentation of AL amyloidosis with significant cardiac involvement. AL amyloidosis has a median survival of one to two years but patients with symptomatic heart involvement have a median survival of only six months. LEARNING OBJECTIVES: Recognize the importance of counseling patients who have received previous valve replacements that continued intravenous drug use may disqualify them from lifesaving interventions. CASE INFORMATION: A 44 year-old presented with progressive dyspnea, fevers and substernal tightness. He had a endocarditis requiring porcein valve replacement in 1993. He started using intravenous cocaine and heroin weekly one month prior. He had a fever of 40 C and a holosystolic murmur. Blood cultures grew steptococcus viridans. Transesophageal echocardiography showed aortic valve vegetations on all leaflets and a ring abscess. Toxicology screen was positive for cocaine. Ampicillin and gentamicin were initiated. After six weeks of therapy, the aortic vegetations and blood cultures persisted. Cardiothoracic surgery was consulted, but declined intervention, citing the likely recidivistic drug use and futility of care. IMPLICATIONS/DISCUSSION: Surgical intervention in active intravenous drugs users is an issue of ethical debate. Ninety percent of heroin users will reuse even after successful rehabilitation. Continued intravenous use represents a hundred-fold increased risk of recurrent endocarditis. The apparent futility of surgical intervention in this setting has prompted surgical services to adopt a``one strike and you are out policy.'' This is almost a certain death sentence for patients meeting surgical criteria. The ethics committee was involved but could not establish ethical grounds for challenging surgery's decision. The precedent of withholding organ transplantation from continuing drug users was applied. Physicians should counsel patients who have received previous valve replacements that continued intravenous drug use may disqualify them from lifesaving interventions. Added emphasis on rehabilitation following valve replacement may be the only sure method to avoid this dilemma. 