PulseExploreJournal ClubDebatesTrendingResearchersJournals
Instagram
HomeExploreJournal ClubTrending
Synapse
⌘+K
Synapse
March 6, 2026The International Journal of Cardiovascular Imaging0 citationsOpen Access

Elevated filling pressures but rare restrictive physiology in transthyretin amyloid cardiomyopathy: genotype-specific patterns and the role of left atrial strain

AEAnna EkmanAVAshwin VenkateshvaranSASandra Arvidsson

Key Result

In patients with ATTR-CM, incorporating left atrial reservoir strain into echocardiographic assessment increased detection of elevated left atrial pressure from 53% to 68%, while restrictive filling was uncommon (22%) and varied by genotype (59% in ATTRwt vs 14% in ATTRv).

Key Points

  • To characterize diastolic dysfunction severity and genotype-specific differences in ATTR-CM patients, and evaluate left atrial strain as a diagnostic tool.
  • Analyzed 100 patients with ATTR-CM via transthoracic echocardiography
  • Compared restrictive filling prevalence between hereditary (ATTRv) and wild-type (ATTRwt) genotypes
  • Integrated left atrial reservoir strain (LASr) into assessments of diastolic dysfunction
  • Restrictive filling was present in 22% of the cohort
  • Restrictive filling was more common in ATTRwt (59%) than ATTRv (14%)
  • Using LASr, detection of elevated filling pressure rose from 53% to 68%
  • Indeterminate cases decreased from 13% to 3%

Study Design

Type

Observational (n=100)

Multicenter

No

Structured PICO

Does incorporating left atrial reservoir strain (LASr) improve the detection of elevated filling pressures in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) compared to standard echocardiographic algorithms?

P
Population
100 patients with transthyretin amyloid cardiomyopathy (ATTR-CM), including 76 with hereditary (ATTRv) and 24 with wild-type (ATTRwt) genotypes. Median age 72.5 years, 27% female. Excluded patients with permanent atrial fibrillation, poor image quality, significant valvular disease, and LV remodeling post MI.
I
Intervention
Echocardiographic assessment of left ventricular diastolic dysfunction (LVDD) incorporating left atrial reservoir strain (LASr) into the 2016 ASE/EACVI algorithm.
C
Comparator
Conventional 2016 ASE/EACVI diastolic function algorithm (without LASr) and comparison between ATTRwt and ATTRv genotypes.
O
Outcome
Prevalence and severity of left ventricular diastolic dysfunction (LVDD), specifically restrictive filling and elevated left atrial pressure (LAP), and the reclassification rate when incorporating LASr.surrogate

Restrictive filling is uncommon in early ATTR-CM and varies by genotype, but incorporating left atrial reservoir strain into standard echocardiographic algorithms significantly improves the detection of elevated filling pressures.

Main Result

Effect estimate: Increase from 53% to 68% prevalence of elevated LAP with addition of LASr

Absolute Event Rate: 68% vs 53%

Limitations

  • Retrospective observational design
  • Small sample size, especially ATTRwt subgroup (n=24)
  • Exclusion of patients with persistent or permanent atrial fibrillation limits generalizability
  • Incomplete availability of some echocardiographic parameters
  • Lack of patient-level reclassification and agreement analyses for LASr incorporation
  • Small study population of ATTRwt
  • Exploratory nature of analyses
  • Partial violation of model assumptions
  • Retrospective design

Abstract

Transthyretin amyloid cardiomyopathy (ATTR-CM) is associated with cardiac stiffness and diastolic dysfunction. While recommendations highlight restrictive filling as a common echocardiographic marker in ATTR-CM, a detailed characterization of diastolic dysfunction (LVDD) severity and differences in prevalence between ATTR-CM genotypes have not been explored. In a well-characterized ATTR-CM cohort, we investigated the prevalence, severity and genotype-specific distribution of LVDD and assessed the added value of left atrial reservoir strain (LASr) for its detection. Diastolic dysfunction was graded by transthoracic echocardiography in 100 patients with ATTR-CM (76 hereditary ATTRv and 24 wild-type (ATTRwt) according to current ASE/EACVI recommendations, with LASr incorporated for comparison. Restrictive filling was observed in only 22% of the cohort and was more common in in ATTRwt compared with ATTRv (59% vs. 14% respectively). Incorporating LASr into LVDD assessment did not change the proportion of patients with restrictive filling pressure but increased the proportion of patients identified with elevated filling pressure from 53% to 68% and reduced indeterminate cases from 13% to 3%. Restrictive filling in uncommon in ATTR-CM and varies by genotype, while incorporation of LASr markedly improves detection of elevated filling pressure. These findings underscore the need for a personalized approach and precision therapy tailored to genotypical differences and comorbidities.

Ask AI
Helpful
Bookmark
Share
View Full Paper

Cite This Study

Ekman et al. (2026) conducted an observational in Patients with transthyretin amyloid cardiomyopathy (ATTR-CM), including 76 hereditary ATTRv and 24 wild-type ATTRwt, median age 72 years, 27% female (n=100). Incorporation of left atrial reservoir strain (LASr) into standard echocardiographic diastolic dysfunction assessment vs. Standard 2016 ASE/EACVI diastolic function algorithm using tricuspid regurgitation velocity was evaluated on Prevalence of elevated left atrial pressure (LAP) and restrictive filling pattern in ATTR-CM assessed by echocardiography (Increase from 53% to 68% prevalence of elevated LAP with addition of LASr). In patients with ATTR-CM, incorporating left atrial reservoir strain into echocardiographic assessment increased detection of elevated left atrial pressure from 53% to 68%, while restrictive filling was uncommon (22%) and varied by genotype (59% in ATTRwt vs 14% in ATTRv).

synapsesocial.com/papers/69aa70d6531e4c4a9ff5af6ahttps://doi.org/10.1007/s10554-026-03667-z
Ask AI
Helpful
Bookmark
Share
View Full Paper

Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Left atrial myopathy in cardiac amyloidosis: implications of novel echocardiographic techniques2004 · 105 citations
  2. 2Assessing left atrial dysfunction in cardiac amyloidosis using LA–LV strain slope2024 · 5 citations
  3. 3Left atrial reservoir strain improves diagnostic accuracy of the 2016 ASE/EACVI diastolic algorithm in patients with preserved left ventricular ejection fraction: insights from the KARUM haemodynamic database2022 · 63 citations
  4. 4Invasive Haemodynamics at Rest and Exercise in Cardiac Amyloidosis2023 · 19 citations
  5. 5Restrictive cardiomyopathy: definition and diagnosis2022 · 172 citations