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Echocardiographic phenotype for refined risk stratification and treatment selection in light chain amyloidosis with
1Division of Cardiology and Department of Internal Medicine, Tongji Hospital, Tongji Medical College of Huazhong University of Science and Technology, #1095 Jiefang Ave., Wuhan, 430030, People's Republic of China.
Insights
Echocardiography can refine risk assessment for light chain amyloidosis (AL) patients with heart failure (HF). Specific echocardiographic findings like right ventricular wall thickness (RVT) and relative wall thickness (RWT) predict survival beyond current staging.
Area of Science:
- Cardiology
- Cardiovascular Imaging
- Amyloidosis Research
Background:
- Light chain amyloidosis (AL) with heart failure (HF) often presents at advanced revised Mayo (rMayo) stages III/IV, carrying a poor prognosis.
- Current staging systems may not fully capture the prognostic nuances within this high-risk AL-HF subgroup.
- There is a need for refined risk stratification to guide personalized treatment strategies.
Purpose of the Study:
- To investigate the value of echocardiographic phenotypes in further risk stratifying AL patients with HF and rMayo stage III/IV.
- To determine if specific echocardiographic parameters can guide risk-adapted treatment decisions in this population.
Main Methods:
- Retrospective analysis of 95 AL patients with HF and rMayo stage III/IV.
- Echocardiographic and laboratory measurements were collected prior to chemotherapy initiation.
- Multivariate Cox regression and Kaplan-Meier survival analyses were used to assess the association of variables with survival.
Main Results:
- Right ventricular wall thickness (RVT), relative wall thickness (RWT), and left ventricular ejection fraction (LVEF) < 50% were independently associated with survival in the overall cohort.
- RVT and RWT showed prognostic value in specific subgroups (chemotherapy vs. non-chemotherapy).
- High RVT (≥ 6.5 mm) or low LVEF (< 50%) significantly reduced survival, irrespective of rMayo stage.
Conclusions:
- Echocardiographic phenotypes provide incremental prognostic information beyond rMayo staging in advanced AL with HF.
- Higher RVT, RWT, and lower LVEF are associated with worse survival outcomes.
- Echocardiography can aid in tailoring treatment strategies for AL-HF patients.
Aims:
Light chain amyloidosis (AL) patients with heart failure (HF) are usually with revised Mayo (rMayo) stage III/IV disease and have a poor prognosis. We sought to investigate whether and what echocardiographic phenotype provides value for further risk stratification and guiding optimal risk-adapted treatment in this subgroup of AL patients.
Methods And Results:
95 AL patients who presented with HF and were on rMayo stage III/IV were retrospectively included. Of them, 51 patients (53.7%) were with stage III, 44 (46.3%) were with stage IV, and 44 (46.3%) underwent chemotherapy. Laboratory and echocardiographic measurements were acquired before the initiation of chemotherapy. The relevance of different variables with survival was assessed in the entire cohort, chemotherapy, and non-chemotherapy group. By Multivariate Cox regression analysis, right ventricular wall thickness (RVT) [HR 1.145, 95% confidence interval (CI) 1.026-1.279, P = 0.016], relative wall thickness (RWT) (HR 6.709, 95% CI 1.101-40.877, P = 0.039), and left ventricular ejection fraction (LVEF) < 50% (HR 1.939, 95% CI 1.048-3.590, P = 0.035) were found to be independently associated with survival in the entire cohort, RWT (HR 15.488, 95% CI 2.045-117.292, P = 0.008) in the non-chemotherapy group, and RVT (HR 1.331, 95% CI 1.054-1.681, P = 0.016) in the chemotherapy group, respectively. Kaplan-Meier survival analysis revealed that survival was significantly reduced in the presence of RVT ≥ 6.5 mm or LVEF < 50% in the entire cohort, and the significance of RVT ≥ 6.5 mm was irrespective of rMayo stages. In the chemotherapy group, survival was decreased if RVT ≥ 6.5 mm alone or together with RWT ≥ 0.67 were present, particularly in patients on rMayo stage IV.
Conclusions:
Echocardiographic phenotype provides incremental value beyond rMayo staging for predicting survival and could further guide treatment in advanced AL with HF. Those with high-risk echocardiographic phenotypes as higher RVT and RWT and lower LVEF had a worse prognosis.
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