Orthostatic hypotension in hereditary ATTR Val30Met amyloidosis: predictors and associated clinical features. (14th October 2021)

• Record Type: Journal Article
• Authors: Dias De Frias, A F; Rodrigues, P; Costa, R; Pereira, A; Alexandre, A; Hipolito Reis, A; Torres, S
• Is Part Of: European heart journal. Volume 42(2021)Supplement 1
• Publication Date: 2021-10-14
• Subjects: Infiltrative Myocardial Disease; Cardiology -- Periodicals; Heart -- Diseases -- Periodicals; 616.12005
• DOI: 10.1093/eurheartj/ehab724.1796
• Languages: English
• ISSNs: 0195-668X

Abstract

Abstract: Introduction: The prevalence of orthostatic hypotension (OH) in patients with mutated transthyretin (TTR) amyloidosis (mATTR) is 40–60%. According to previous studies, OH is frequent and an early feature in patients with Val30Met mutation (the most prevalent form of mATTR). Aim: To characterize TTR Val30Met patients with OH and to identify clinical characteristics associated with OH development. Methods: Retrospective study of consecutive Val30Met TTR patients with suspected cardiac involvement observed at our cardiology clinic during 2019. Two groups were defined: group 1: patients without OH; group 2: patients with OH. Data was obtained by chart review. Statistically significant predictors of OH were found using logistic regression. Results: We included a total of 248 patients (group 1 – 173; group 2 – 75). Group 1 patients were 52% male, median age 45 [interquartile range (IQR) 39–55] and median age at onset 34 (IQR 29, 75–46, 25) years. Left ventricle hypertrophy [LVH, defined as maximal LV wall thickness (LVT) ≥12 mm] occurred in 26, 5%, with median maximal LVT 10 mm (IQR 9–12); 49, 7% had conduction disturbances, 30, 6% gastrointestinal (GI), 17, 3% genitourinary (GU) manifestations and 5% were in Coutinho staging ≥2/3. Group 2 had 56% male, median age of 49 years at evaluation (IQR 42–65) and 35 years at onset (IQR 30–59). LVH was present in 42, 9%, with median maximal LVT 11 mm (IQR 10–14); 74, 7% had conduction disturbances, 56% GI and 42, 7% GU manifestations and 21% were in Coutinho staging ≥2/3. In univariate analysis, higher age (p=0, 005), presence of LVH (p=0, 009), conduction disturbances (p<0, 001), GU manifestations (p<0, 001) and higher Coutinho staging (p<0, 001) were all associated with the presence of OH, while age at onset was not (p=0, 648). In multivariate analysis, only Coutinho staging [odds ratio (OR) 2.609; 95% confidence interval (95% CI) 1.344–5.065] and GU manifestations (OR 3, 151; 95% CI 1, 595–6, 225) were found to be significant predictors of OH. Conclusion: Our study suggests that OH is more associated with GU manifestations and Neurologic staging, than with amyloid cardiomyopathy or age, suggesting a predominant neurogenic component. The prevalence of OH in our sample of Val30Met patients was lower than previously described. Funding Acknowledgement: Type of funding sources: None.