What is the clinical evidence from this study?

Study design: Observational. Population: Transthyretin amyloid cardiomyopathy (ATTR-CM) (n=8950). Intervention: Time period (2019 vs 2011) vs. 2011 baseline. Primary outcome: Annual incidence rate of ATTR-CM (per 100,000 person-years) (p=<0.001).

November 6, 2023Open Access

Incidence and survival of transthyretin amyloid cardiomyopathy from a French nationwide study of in- and out-patient databases

Q: What are the key findings of this study?

The incidence of transthyretin amyloid cardiomyopathy in France increased significantly from 0.6 per 100,000 person-years in 2011 to 3.6 per 100,000 person-years in 2019, with a median survival of 41.9 months.

TDThibaud DamyHeart Failure & Transplant GBGuillaume BourelUniversité Paris-Sud MSMichel SlamaCardiac Imaging

Key Result

The incidence of transthyretin amyloid cardiomyopathy in France increased significantly from 0.6 per 100,000 person-years in 2011 to 3.6 per 100,000 person-years in 2019, with a median survival of 41.9 months.

Study Design

Type

Observational (n=8,950)

Structured PICO

Population

8,950 patients with incident transthyretin amyloid cardiomyopathy in France, identified via nationwide claims data between 2011 and 2019, followed until death or the end of 2019.

Outcome

Annual incidence and survival of transthyretin amyloid cardiomyopathy (ATTR-CM)hard clinical

The diagnosed incidence of ATTR-CM in France increased six-fold from 2011 to 2019, likely reflecting improved recognition, with a median survival of approximately 3.5 years after diagnosis.

Main Result

Absolute Event Rate: 3.6% vs 0.6%

p-value: p=<0.001

Limitations

Lack of specific ICD-10 or CCAM codes for ATTR-CM in the database, requiring an algorithm based on amyloidosis and cardiac condition codes.
Exclusion of AL- and AA-amyloidosis relied on surrogate endpoints (treatments), which may have led to misclassification.
Inability to discriminate between hereditary (ATTRv-CM) and wild-type (ATTRwt-CM) forms of the disease.
Potential inclusion of rare amyloidosis types (e.g., Apo A2, Apo A4, Fibrinogen).
Patients without an E85 ICD-10 diagnosis code were missed, potentially underestimating incidence.

Abstract

BACKGROUND: Precise data about ATTR-CM incidence rates at national level are scarce. Consequently, this study aimed to estimate the annual incidence and survival of transthyretin amyloid cardiomyopathy (ATTR-CM) in France between 2011 and 2019 using real world data. We used the French nationwide exhaustive data (SNDS database) gathering in- and out-patient claims. As there is no specific ICD-10 marker code for ATTR-CM, diagnosis required both amyloidosis (identified by E85. ICD-10 code or a tafamidis meglumine delivery) and a cardiovascular condition (identified by ICD-10 or medical procedure codes related to either heart failure, arrhythmias, conduction disorders or cardiomyopathies), not necessarily reported at the same visit. Patients with probable AL-form of amyloidosis or probable AA-form of amyloidosis were excluded. RESULTS: Between 2011 and 2019, 8,950 patients with incident ATTR-CM were identified. Incidence rates increased from 0.6 / 100,000 person-years in 2011 to 3.6 / 100,000 person-years in 2019 (p < 0.001), reaching 2377 new cases in 2019. Sex ratios (M/F) increased from 1.52 in 2011 to 2.23 in 2019. In 2019, median age at diagnosis was 84.0 years (85.5 for women and 83.5 for men). Median survival after diagnosis was 41.9 months (95% CI 39.6, 44.1). CONCLUSIONS: This is the first estimate of nationwide ATTR-CM incidence in France using comprehensive real-world databases. We observed an increased incidence over the study period, consistent with an improvement in ATTR-CM diagnosis in recent years.

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