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Transthyretin Amyloid Cardiomyopathy in HFpEF
Quick Takes
- ATTR-CM is present in a substantial number of cases of HFpEF and ventricular wall thickening, especially in older men.
- Systematic evaluation for ATTR-CM may significantly increase its diagnosis and offer therapeutically relevant phenotyping of HFpEF.
- Systematic screening for ATTR-CM should be considered in selected older patients (particularly men) with HF, preserved EF, and ventricular wall thickening given that effective therapy is available for this condition.
Study Questions:
What is the prevalence of transthyretin amyloid cardiomyopathy (ATTR-CM) without and with systematic screening in patients with heart failure with preserved ejection fraction (HFpEF) and ventricular wall thickening?
Methods:
The investigators conducted a population-based cohort study to assess ATTR-CM prevalence in 1,235 consecutive patients in southeastern Minnesota with HFpEF both without (prospectively identified cohort study) and with (consenting subset of cohort study, n = 286) systematic screening. Key entry criteria included validated HF diagnosis, age ≥60 years, EF ≥40%, and ventricular wall thickness ≥12 mm. In this community cohort of 1,235 patients, 884 had no known ATTR-CM, contraindication to technetium Tc 99m pyrophosphate scanning, or other barriers to participation in the screening study. Of these 884 patients, 295 consented and 286 underwent scanning between October 5, 2017–March 9, 2020 (community screening cohort). Medical record review or technetium Tc 99m pyrophosphate scintigraphy and reflex testing for ATTR-CM diagnosis was performed. The main outcomes measure was the ATTR-CM prevalence by strategy (clinical diagnosis or systematic screening), age, and sex. Multivariable least-squares linear regression and multivariable logistic regression models were used to adjust associations with amyloidosis presence for potential confounders.
Results:
A total of 1,235 patients participated in the study, including a community cohort (median age, 80 years; interquartile range, 72-87 years; 630 [51%] male) and a community screening cohort (n = 286; median age, 78 years; interquartile range, 71-84 years; 150 [52%] male). In the 1,235 patients in the community cohort without screening group, 16 patients (1.3%; 95% confidence interval [CI], 0.7%-2.1%) had clinically recognized ATTR-CM. The prevalence was 2.5% (95% CI, 1.4%-4.0%) in men and 0% (95% CI, 0.0%-0.6%) in women. In the 286 patients in the community screening cohort, 18 patients (6.3%; 95% CI, 3.8%-9.8%) had ATTR-CM. Prevalence increased with age from 0% in patients 60-69 years of age to 21% in patients ≥90 years (p < 0.001). Adjusting for age, ATTR-CM prevalence differed by sex, with 15 of 150 men (10.0%; 95% CI, 5.7%-16.1%) and 3 of 136 women (2.2%; 95% CI, 0.4%-6.3%) having ATTR-CM (p = 0.002).
Conclusions:
The authors concluded that in this cohort study based in a community-based setting, ATTR-CM was present in a substantial number of cases of HFpEF with ventricular wall thickening, particularly in older men.
Perspective:
This community-based setting cohort study reports that ATTR-CM was present in a substantial number of cases of HFpEF and ventricular wall thickening, especially in older men. These data suggest that systematic evaluation for ATTR-CM may significantly increase its diagnosis and offer therapeutically relevant phenotyping of HFpEF. Overall, these data advocate for consideration of systematic screening for ATTR-CM in selected older patients (particularly men) with HF, preserved EF, and ventricular wall thickening given that effective therapy is available for this condition.
Clinical Topics: Cardiovascular Care Team, Geriatric Cardiology, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Prevention, Acute Heart Failure
Keywords: Amyloidosis, Cardiomyopathies, Diagnostic Imaging, Geriatrics, Heart Failure, Prealbumin, Secondary Prevention, Stroke Volume, Technetium Tc 99m Pyrophosphate, Ventricular Function
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