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Document Details : Title: Clinical experience with cardiac amyloidosis Author(s): GOEMINNE, Ann , MISSAULT, Luc , BAUWENS, Frank , DE PAUW, MIchel , DE SUTTER, Johan Journal: Acta Cardiologica Volume: 58 Issue: 2 Date: April 2003 Pages: 143-147 DOI: 10.2143/AC.58.2.2005267 Abstract : Objective — Amyloidosis is a systemic disease potentially afflicting the heart. In this study we retrospectively studied patients presenting with major cardiac amyloidotic involvement. Methods and results — From 1997 until 2000, eight patients with major cardiac involvement of amyloidosis resulting in heart failure were diagnosed.All patients presented with heart failure. Diagnostic work-up,clinical,electrocardiographic,echocardiographic characteristics and treatment modalities are presented. Seven patients with acquired (both primary AL and postinflammatory AA) amyloidosis out of a total of eight patients died within one year after diagnosis, as a result of intractable cardiac failure due to both systolic and diastolic left ventricular dysfunction despite state-of-the-art medical treatment of heart failure. Only one patient with hereditary amyloidosis undergoing liver transplantation survived. Conclusions — Despite optimal medical cardiac failure treatment, acquired cardiac amyloidosis carries an ominous prognosis probably because patients are already in a very advanced stage of the disease at presentation. |
