Autonomic dysfunction in patients with wild-type transthyretin amyloidosis - PubMed
5 hours ago
- #Cardiomyopathy
- #Transthyretin amyloidosis
- #Autonomic dysfunction
- Autonomic dysfunction is well recognized in hereditary transthyretin amyloidosis (ATTRv) but not systematically studied in wild-type transthyretin amyloidosis (ATTRwt).
- ATTRwt primarily presents with cardiomyopathy, and autonomic symptoms may mimic heart failure, leading to inappropriate treatment.
- The study investigated autonomic dysfunction in ATTRwt using standardized questionnaires, orthostatic challenges, Valsalva maneuver, deep breathing, and sudomotor assessment.
- Results showed impaired blood pressure and heart rate regulation in ATTRwt compared to controls during orthostatic challenges.
- 83% of ATTRwt patients had a pathological Valsalva Ratio compared to 30% of controls.
- Autonomic symptoms were infrequently reported in ATTRwt, but detailed assessment revealed cardiovascular autonomic dysfunction.
- The study concluded that autonomic dysfunction contributes to the overall clinical phenotype of ATTRwt.