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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Endothelial Function Is Preserved in Patients with Wild-Type Transthyretin Amyloid Cardiomyopathy
Yu Hashimoto1, Takayuki Yamaji1, Toshiro Kitagawa1
1Department of Cardiovascular Medicine, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima 734-8551, Japan.
Insights
Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) patients show preserved endothelial function, potentially due to compensatory mechanisms. This contrasts with typical heart failure endothelial dysfunction, suggesting unique vascular adaptations in ATTRwt-CM.
Area of Science:
- Cardiology
- Vascular Biology
- Cardiomyopathy Research
Background:
- Heart failure (HF) is linked to endothelial dysfunction, impacting cardiac health.
- Wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) lacks data on vascular function.
- Understanding vascular function in ATTRwt-CM is crucial for its pathophysiology.
Purpose of the Study:
- To assess endothelial and vascular smooth muscle function in ATTRwt-CM patients.
- To evaluate arterial stiffness in individuals with ATTRwt-CM.
- To compare vascular parameters between ATTRwt-CM patients and matched controls.
Main Methods:
- Flow-mediated vasodilation (FMD) for endothelial function.
- Nitroglycerine-induced vasodilation (NID) for vascular smooth muscle function.
- Brachial artery intima-media thickness (bIMT) and pulse wave velocity (baPWV) for arterial stiffness.
- Comparison of 22 ATTRwt-CM patients with 22 matched controls.
Main Results:
- ATTRwt-CM patients exhibited significantly greater FMD (5.4% vs. 3.5%, p=0.038).
- N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were higher in ATTRwt-CM patients (2202 vs. 470 pg/mL, p<0.001).
- No significant differences were found in NID, bIMT, or baPWV between groups.
- A positive correlation was observed between NT-proBNP and FMD in ATTRwt-CM patients (r=0.485, p=0.022).
Conclusions:
- Endothelial function is preserved in patients with ATTRwt-CM.
- Elevated NT-proBNP may indicate compensatory endothelial function in ATTRwt-CM.
- Vascular smooth muscle function and arterial stiffness do not differ significantly from controls.
Abstract:
Heart failure (HF) is associated with endothelial dysfunction. Vascular function per se plays an important role in cardiac function, whether it is a cause or consequence. However, there is no information on vascular function in patients with wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM). The purpose of this study was to evaluate vascular function in patients with ATTRwt-CM. We measured flow-mediated vasodilation (FMD) as an index of endothelial function and nitroglycerine-induced vasodilation (NID) as an index of vascular smooth muscle function and brachial artery intima-media thickness (bIMT) and brachial-ankle pulse wave velocity (baPWV) as indices of arterial stiffness in 22 patients with ATTRwt-CM and in 22 one-by-one matched control patients using vascular function confounding factors. FMD was significantly greater in patients with ATTRwt-CM than in the controls (5.4 ± 3.4% versus 3.5 ± 2.4%, p = 0.038) and the N-terminal pro-brain natriuretic peptide (NT-proBNP) level was significantly greater in patients with ATTRwt-CM than in the controls (2202 ± 1478 versus 470 ± 677 pg/mL, p < 0.001). There were no significant differences in NID, bIMT or baPWV between the two groups. There was a significant relationship between NT-proBNP and FMD in patients with ATTRwt-CM (r = 0.485, p = 0.022). NT-proBNP showed no significant relationships with NID, bIMT or baPWV. Conclusions: Endothelial function was preserved in patients with ATTRwt-CM. Patients with ATTRwt-CM may have compensatory effects with respect to endothelial function through elevation of BNP.
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