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Updated: Jan 25, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Associated diseases and diuretic dosage to predict mortality in transthyretin amyloid cardiomyopathy
Vincent Algalarrondo1, Olivier Lairez2, Marion Narbeburu3
1Referral Center for Cardiac Amyloidosis CERAMIC-Cardio, AP-HP Bichat Hospital, Université Paris-Cité, 75018 Paris, France.
Insights
Diuretic dosage is a key predictor of mortality in transthyretin amyloid cardiomyopathy (ATTR-CM) patients, alongside other comorbidities. Comprehensive management of associated diseases is crucial for improving outcomes in ATTR-CM.
Area of Science:
- Cardiology
- Internal Medicine
- Pharmacology
Background:
- Transthyretin amyloidosis (ATTR) can progress to transthyretin amyloid cardiomyopathy (ATTR-CM), a serious form of heart failure.
- ATTR-CM is associated with a high burden of cardiac and extracardiac comorbidities.
Purpose of the Study:
- To determine the prevalence of co-existing conditions in ATTR-CM patients.
- To identify predictors of mortality in ATTR-CM, with a specific focus on diuretic dosage.
Main Methods:
- Utilized the French National Health Database to identify patients diagnosed with ATTR-CM between 2011 and 2019.
- Categorized diuretic (furosemide) doses into four distinct levels for analysis.
- Examined various medical characteristics and their association with mortality.
Main Results:
- Over 7800 ATTR-CM patients were analyzed, with significant proportions on different diuretic dose levels at diagnosis.
- Common extracardiac conditions included kidney disease (37.0%), diabetes (29.5%), and neurologic disorders (17.9%).
- Multivariable analysis identified age, male sex, diuretic dose, heart failure, arrhythmia, kidney disease, gastrointestinal disorders, and diabetes as significant mortality predictors.
Conclusions:
- ATTR-CM patients present with a substantial number of comorbidities necessitating integrated management strategies.
- Diuretic dosage is a critical prognostic factor in ATTR-CM, highlighting its importance in patient care and outcome prediction.
Background:
Transthyretin amyloidosis can lead to transthyretin amyloid cardiomyopathy (ATTR-CM) and heart failure.
Aims:
To describe the prevalence of cardiac and extracardiac diseases in patients with ATTR-CM and examine mortality predictors, including diuretic dosage, using the French National Health Database.
Methods:
Patients with ATTR-CM and their medical characteristics were identified from the French database from 2011 to 2019. Diuretic doses were categorized into four classes (furosemide dose: level 1<20mg; level 2 20 to <60mg; level 3 60 to<120mg; level 4 ≥120mg). Predictive factors for mortality were examined.
Results:
Of 7804 patients with ATTR-CM, 33.0% were on level 1 diuretics, 25.8% on level 2, 15.8% on level 3 and 25.3% on level 4 at diagnosis. Leading extracardiac conditions included kidney disease (37.0%), diabetes (29.5%), neurologic disorders (17.9%), gastrointestinal disorders (15.7%) and musculoskeletal conditions (11.8%). Median (95% confidence interval [CI]) survival was 3.1 (3.0-3.3) years. Multivariable analysis identified age at diagnosis (hazard ratio [HR] 1.482, 95% CI 1.400-1.558), male sex (HR 1.258, 95% CI 1.125-1.406), diuretic dose (HR 1.380, 95% CI 1.315-1.449), heart failure (HR 1.251, 95% CI 1.090-1.437), arrhythmia/conduction disorder (HR 1.143, 95% CI 1.001-1.306), kidney disease (HR 1.224, 95% CI 1.104-1.358), gastrointestinal disorder (HR 1.143, 95% CI 1.000-1.307) and diabetes (HR 1.192, 95% CI 1.071-1.326) as significantly associated with mortality.
Conclusion:
Patients with ATTR-CM face a significant burden of associated diseases requiring comprehensive management alongside their ATTR-CM treatment. Beyond addressing these comorbidities, diuretic dosage emerges as a pivotal prognostic indicator.
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