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Cardiac amyloidosis - experience in a tertiary cardiac referral centre
Insights
Cardiac amyloidosis, a rare heart condition, has a poor prognosis, with survival rates declining over time. Specific treatment for underlying amyloidosis significantly improves patient survival outcomes.
Area of Science:
- Cardiology
- Nephrology
- Oncology
Background:
- Amyloidosis is a rare systemic disease involving fibrillar protein deposition in organs.
- Cardiac amyloidosis significantly worsens prognosis, often presenting late with limited treatment options.
Discussion:
- This study reports the largest series of 16 Chinese patients with cardiac amyloidosis.
- Survival rates at 1, 3, and 5 years were 40%, 25%, and 17%, respectively.
- Cardiac-related issues were the primary cause of mortality.
Key Insights:
- Patients with overt heart failure or untreated amyloidosis had a dismal prognosis (2.2 and 3.5 months survival).
- Specific treatment for the underlying cause of amyloidosis led to improved outcomes, with an average survival of 33.4 months.
Outlook:
- Further research is needed to improve early diagnosis and effective treatment strategies for cardiac amyloidosis.
- Developing targeted therapies for amyloidosis can potentially enhance long-term survival and quality of life for affected individuals.
Abstract:
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.
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