Marianna Fontana is Professor of Cardiology and Honorary Consultant Cardiologist at the National Amyloidosis Centre, Division of Medicine, University College London.

Marianna Fontana, MD, PhD

Marianna Fontana, MD, PhD: Declines in Kidney Function Frequent in ATTR-CM 

A decline in kidney function was common in patients with 

transthyretin amyloid cardiomyopathy (ATTR-CM)

 and linked to an elevated likelihood of mortality, even after adjustment for relevant biomarkers, according to new data presented at the 

American Heart Association (AHA) Scientific Sessions 2024

This retrospective, observational, single-center cohort study assesed individuals with ATTR-CM at the National Amyloidosis Centre (NAC) in the United Kingdom, who underwent an assessment of baseline estimated glomerular filtration rate (eGFR) and 1-year follow-up between January 2000 and April 2024.

“We took a large population of patients, more than 2000 patients, with a ATTR-CM, and we did a landmark analysis at 12 months, where we measured the eGFR. We found, at 12 months, a change of 20% decline, an independently predictive prognosis,” Marianna Fontana, MD, PhD, a professor of cardiology and honorary consultant cardiologist at the National Amyloidosis Centre, division of medicine, University College London, told 

The study’s primary outcome was the risk of all-cause mortality associated with a decline in kidney function, considered a decrease in eGFR of >20%.

Upon analysis, the decline in kidney function was associated with a 1.7-fold higher risk of mortality (hazard ratio [HR], 1.71; 95% CI, 1.43-2.04; 

 < .001), with a similar risk across all 3 genotypes (wild type: HR, 1.64; 95% CI, 1.31-2.04; p.(V142I): HR, 1.70; 95% CI, 1.21-2.39; non-p.(V142I): HR, 1.51; 95% CI, 0.87-2.61) (

 for interaction = .93) and the 3 NAC disease stages (stage 1: HR, 1.69; 95% CI, 1.22-2.32; stage 2: HR, 1.69; 95% CI, 1.30-2.18; stage 3: HR, 1.61; 95% CI, 1.11-2.35) (

Importantly, the decline in kidney function was consistently, independently associated with mortality, after adjustment for increases in NT-proBNP and outpatient diuretic intensification (HR, 1.48; 95% CI, 1.23-2.76; 

“This is just the first step, one of the things that will need to be assessed is, for example, using this treatment to guide treatment response and [determine if] it improves management of the patients,” Fontana told 

Disclosures: Relevant disclosures for Fontana include Alnylam Pharmaceuticals, Bayer, Intellia Therapuetics, Janssen Pharmaceuticals, Novo Nordisk, and others.

Ioannou A, Razvi Y, Porcari A, et al. Kidney Outcomes in Transthyretin Amyloid Cardiomyopathy. JAMA Cardiol. Published online November 17, 2024. doi:10.1001/jamacardio.2024.4578

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Decline in kidney function is prevalent in patients with transthyretin amyloid cardiomyopathy (ATTR-CM) and correlates with increased mortality risk, even after adjusting for biomarkers. A study at the National Amyloidosis Centre in the UK analyzed over 2000 patients, revealing a 20% decline in eGFR at 12 months as a significant prognostic indicator. The decline was linked to a 1.7-fold higher mortality risk across genotypes and disease stages. This association remained significant after adjusting for NT-proBNP and diuretic use, suggesting potential implications for patient management.

Marianna Fontana, MD, PhD describes the prognostic importance of a decline in eGFR in a retrospective cohort of patients with ATTR-CM.

Marianna Fontana, MD, PhD

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