From the Guidelines
Amyloid deposition in the cardiac tissue of dialysis patients is a serious complication that primarily occurs due to beta-2 microglobulin accumulation, especially in patients on long-term hemodialysis, and management focuses on kidney transplantation as the definitive treatment, which can halt or slow amyloid deposition, as recommended by the most recent guidelines 1.
Background
Amyloid deposition in dialysis patients, also known as dialysis-related amyloidosis (DRA), is a condition where amyloid fibrils deposit in cardiac structures, potentially leading to cardiomyopathy, arrhythmias, and heart failure. This condition is primarily caused by the accumulation of beta-2 microglobulin, especially in patients on long-term hemodialysis 1.
Diagnosis and Monitoring
Regular cardiac monitoring with echocardiography and cardiac MRI is essential for early detection, recommended annually for patients on dialysis for more than 5 years. This monitoring can help identify cardiac involvement and allow for timely intervention 1.
Treatment
For patients unable to receive transplants, high-flux dialysis membranes or hemodiafiltration should be used to better clear beta-2 microglobulin. Supportive care includes managing heart failure symptoms with careful use of diuretics, ACE inhibitors, and beta-blockers, though these medications must be adjusted for dialysis patients 1.
Underlying Mechanism
The underlying mechanism involves inadequate clearance of beta-2 microglobulin during conventional dialysis, leading to its accumulation and transformation into amyloid fibrils that deposit in various tissues, including the heart, causing structural and functional damage 1.
Recent Guidelines
Recent guidelines recommend a comprehensive multidisciplinary care approach for patients with cardiac amyloidosis, including careful evaluation and management of cardiac involvement, as well as consideration of treatment options such as kidney transplantation and high-flux dialysis membranes 1.
- Key points to consider:
- Kidney transplantation is the definitive treatment for dialysis-related amyloidosis.
- High-flux dialysis membranes or hemodiafiltration can help clear beta-2 microglobulin in patients unable to receive transplants.
- Regular cardiac monitoring is essential for early detection and timely intervention.
- Supportive care includes managing heart failure symptoms with careful use of medications.
- Recent guidelines recommend a comprehensive multidisciplinary care approach for patients with cardiac amyloidosis.
From the Research
Amyloid Deposition in Cardia in Dialysis Patients
- Amyloidosis is a condition where amyloid fibrils deposit in various organs, including the heart, leading to cardiac amyloidosis 2, 3.
- Cardiac amyloidosis is a life-threatening and progressive disease that can be hereditary or occur as a consequence of the ageing process 2.
- Dialysis-related amyloidosis (DRA) is a disorder caused by excessive accumulation of β-2 microglobulin (B2M) and is considered a "disappearing act" or complication in many countries 4.
- However, the number of patients requiring interventions for treatment of DRA is postulated to increase due to the growing number of patients living with end-stage kidney disease (ESKD) 4.
- Early recognition and management of cardiac amyloidosis are crucial, as recent advances in diagnosis and treatment have improved patient outcomes 5, 6, 3.
- Clinicians should aggressively screen at-risk groups, identify early signs of disease, and initiate treatment to prevent significant end-organ amyloid deposition 5.
- Treatment options for cardiac amyloidosis include heart transplantation, which should be considered in carefully selected patients with end-stage cardiac amyloidosis 5, 6.