Cardiac amyloidosis - experience in a tertiary cardiac referral centre.

Amyloidosis is an uncommon systemic disease characterized by deposition of insoluble fibrillar protein in different organs and the prognosis is poor if the heart is involved. Experience with management of cardiac amyloidosis is difficult because of its rare occurrence, late presentation and ineffective treatment. Since 1995, we have encountered and prospectively followed up 16 cases of cardiac amyloidosis in our cardiac centre. We believe this is the largest series of cardiac amyloidosis reported in Chinese patients. The 1-year, 3-year and 5-year survival rates were 40%, 25% and 17%, respectively. The major cause of death was cardiac-related. Those patients with overt heart failure or with untreated amyloidosis had a dismal prognosis (mean survival of 2.2 months and 3.5 months, respectively). Those who received specific treatment for the underlying amyloidosis had a better outcome with an average survival of 33.4 months.