C18. Cardiac AA amyloidosis presenting with refractory heart failure complicated by polymorphic ventricular tachycardia: a case report

Abstract

Background Systemic AA amyloidosis is a rare infiltrative disease representing a complication of chronic inflammatory disorders. Cardiac involvement is 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 Summary A 53-years-old male patient with psoriatic arthritis presented with heart failure and nephrotic syndrome. Electrocardiogram revealed first-degree AV block, low voltage QRS complexes, and prolonged QT interval. On echocardiography, a normal systolic function of hypertrophic left ventricle with signs of diastolic dysfunction was present. An abdominal fat pad punch biopsy was able to confirm amyloid deposition. Self-terminated polymorphic ventricular arrhythmia was recorded by 24-hour Holter. Immunosuppressive, anti-inflammatory, anti-arrhythmic, and heart failure agent were promptly instituted, which are ineffective. Discussion Restrictive cardiomyopathy is the hallmark finding in cardiac amyloidosis that caused by the interstitial deposits of amyloid fibrils. Clinically, this manifests in progressive diastolic dysfunction, elevated left ventricular filling pressures, and heart failure with preserved ejection. The presence of rapidly progressed heart and renal failure with characteristic features of ECG should prompt further evaluation to confirm an infiltrative cardiomyopathy. This is highly suggestive that the natural course of cardiac amyloidosis often unpredictable and can be aggressive if left undiagnosed and untreated.