B50-41 Systemic Al Amyloidosis Presenting With Gastrointestinal Amyloidosis and Cardiac Amyloidosis: A Rare Critical Care Challenge

Abstract Background Systemic Amyloid light chain (AL) amyloidosis is a rare and progressive protein misfolding disease that can affect multiple organs. When the Gastrointestinal tract is involved, it may present with bleeding, dysmotility, malabsorption, or serious conditions like gastric outlet obstruction and gastric pneumatosis. When coupled with cardiac amyloidosis, the clinical course becomes profoundly complex and life-threatening. Case Presentation A 70-year-old male with insignificant medical history presented with one month of progressive abdominal pain, dysphagia, nausea, vomiting, and dyspnea on exertion. Upon arrival at the emergency department, he was found to be hypotensive and in atrial fibrillation with rapid ventricular response. A CT angiography of the abdomen and pelvis was performed, which revealed gastric pneumatosis, pneumatosis intestinalis, and gastric outlet obstruction. The patient was managed conservatively with bowel rest, broad-spectrum antibiotics, rhythm and rate control for atrial fibrillation. Prompt endoscopy revealed mucosal necrosis, pyloric obstruction, and nodular mucosa in the antrum; biopsy confirmed light chain amyloid deposition. An echocardiogram revealed diastolic dysfunction. Monoclonal light chain assays were obtained and were positive. Subsequently, a bone marrow biopsy revealed multiple myeloma, followed by a cardiac MRI that confirmed cardiac amyloidosis. The patient was started on Bortezomib, Melphalan, and Cyclophosphamide by hematology/oncology for systemic AL amyloidosis secondary to multiple myeloma. Later, he experienced multiple episodes of ventricular fibrillation leading to cardiac arrest, managed with defibrillation, mechanical ventilation, and multiple cycles of resuscitation. Despite extensive cardiac support, the patient ultimately succumbed during a final code event. Discussion This case shows the complications due to systemic AL amyloidosis manifesting as both gastrointestinal and cardiac amyloidosis and how managing them together in a critical care setting can be a challenge. In critically ill patients, unexplained gastric pneumatosis, gastric outlet obstruction, and multiple irregular cardiac rhythms should always prompt physicians to keep systemic amyloidosis in their differentials. AL amyloidosis with gastrointestinal (GI) and cardiac involvement has very poor prognosis. An individualized approach is needed for a patient presenting with both GI and cardiac complications, integrating disease-modifying therapy, conservative management for GI issues, volume management for heart failure, and rhythm control for irregular rhythms. The disease-modifying therapy in AL amyloidosis is targeted at suppressing monoclonal antibodies. Autologous stem cell transplantation (SCT) is the standard of care, but for patients not eligible for SCT, therapy with bortezomib based regimens has been shown to improve survival. This abstract is funded by: NONE