Document Type : Case Report
Department of Cardiology and Vascular Medicine, Faculty of Medicine, Airlangga University – Dr. Soetomo General Hospital, Surabaya, Indonesia.
Department of Internal Medicine, Faculty of Medicine, Airlangga University – Dr. Soetomo General Hospital, Surabaya, Indonesia.
Systemic AA amyloidosis is a rare infiltrative disease representing a complication of chronic inflammatory disorders. Cardiac involvement is extremely rare and is associated with a poor prognosis. Early recognition is imperative as appropriate measures and treatment of the underlying disease may prevent death from refractory heart failure and fatal arrhythmias.
Case Report: A 53-year-old male patient with psoriatic arthritis presented with heart failure and nephrotic syndrome. Electrocardiography revealed a first-degree atrioventricular block, low-voltage QRS complexes, and a prolonged QT interval. Echocardiography revealed a hypertrophic left ventricle with normal systolic function and signs of diastolic dysfunction, as well as right ventricular dysfunction and mild pericardial effusion. An abdominal fat pad punch biopsy confirmed amyloidosis deposition. A 24-hour Holter study recorded self-terminated polymorphic ventricular arrhythmias. Immunosuppressive, anti-inflammatory, antiarrhythmic, and heart failure agents were promptly instituted, which proved ineffective.
Conclusions: Significant cardiac involvement in systemic AA amyloidosis is infrequent and complicated by fatal arrhythmias not described before. This report highlights the significance of the early recognition and aggressive control of the inflammatory response. (Iranian Heart Journal 2022; 23(2): 120-129)