All-cause mortality rate in cardiac amyloidosis patients was 171/1000 PY, highest in Black patients (232/1000 PY); key mortality predictors had HR >1.5 including age 85+, low SBP, and heart failure.
In a UK cohort of patients with cardiac amyloidosis, all-cause mortality was high (50% over follow-up), with advanced age, hypotension, and heart failure being strong predictors of death.
Absolute Event Rate: 0% vs 0%
Abstract Background Cardiac amyloidosis (CA) is associated with a high mortality rate due to sudden or progressive heart failure. The two most common amyloid subtypes are light-chain (AL) and transthyretin (ATTR) amyloidosis both of which are often misdiagnosed. Early detection and risk stratification remain crucial for optimising patient outcomes, yet comprehensive data on clinical predictors of disease progression and adverse outcomes remain limited. Purpose To describe incidence rates and risk factors for all-cause mortality in patients coded to have CA in the UK clinical setting using electronic health records. Methods In this retrospective observational study, anonymised, linked primary and secondary care data (Clinical Practice Research Datalink, Hospital Episode Statistics, and death registrations) were used to describe individuals newly diagnosed with CA. Patients aged 18 years and older with primary care diagnosis records (SNOMED-CT/Read codes) combined with secondary care diagnosis (ICD-10: E85.x coinciding with cardiology codes) in the period between January 2004 to March 2021 were included. The primary outcome was all-cause mortality (ACM), described using crude event rates (per 1,000 person-years, PY) and analysed using Cox proportional hazards models. Demographic and clinical data were summarised using descriptive statistics. Hazard ratios (HR) with 95% confidence intervals (CI) were estimated to account for risks of ACM. Results A total of 3,215 patients with CA were identified, with an average age of 71 years; 62% were male. AL amyloidosis was the predominant subtype (66%), while 34% had ATTR amyloidosis. Over a median follow-up of 9,475 PY, 1,622 (50%) patients died of any cause (crude event rate: 171 per 1000 PY). ACM incidence rates varied by ethnicity, with highest rates observed in the Black (232 per 1000 PY), followed by White (181 per 1000 PY) ethnic group. (Figure) Among the independent risk factors identified, the strongest predictors of ACM (HR 1.5) included age 85+ years (1.7), SBP 90 mmHg (1.9), eGFR 15-30 mL/min/1.73m2 (1.5), haematocrit 10-20% (25), serum monoclonal protein 10 g/L (from 2.1), severe thrombocytopenia (5), alkaline phosphatase 600 IU/L (2.4) and the presence of heart failure (2.1) and nephrotic syndrome (1.6). (Table) Conclusions Mortality among patients coded to have CA is high. A key limitation of existing coding practices is that AL and ATTR amyloidosis, which are distinct conditions, cannot be distinguished. The incidence rate of all-cause mortality was highest in Black patients with CA. This study confirmed known risk factors for all-cause mortality in CA, aligning with previous reports and underscores the need for better coding practice to inform risk stratification and clinical decisions.Table Figure
Anderson et al. (Sat,) reported a other. All-cause mortality rate in cardiac amyloidosis patients was 171/1000 PY, highest in Black patients (232/1000 PY); key mortality predictors had HR >1.5 including age 85+, low SBP, and heart failure.