Clinical Investigation
Independent Predictors of Survival in Primary Systemic (AL) Amyloidosis, Including Cardiac Biomarkers and Left Ventricular Strain Imaging: An Observational Cohort Study

https://doi.org/10.1016/j.echo.2010.03.027Get rights and content

Background

The prognostic value of Doppler myocardial imaging, including myocardial velocity imaging, strain, and strain rate imaging, in patients with primary (AL) amyloidosis is uncertain. The aim of this longitudinal study was to identify independent predictors of survival, comparing clinical data, hematologic and cardiac biomarkers, and standard echocardiographic and Doppler myocardial imaging measures in a cohort of patients with AL amyloidosis.

Methods

A total of 249 consecutive patients with AL amyloidosis were prospectively enrolled. The primary end point was all-cause mortality, and during a median follow-up period of 18 months, 75 patients (30%) died. Clinical and electrocardiographic data, biomarkers (brain natriuretic peptide and cardiac troponin T) and standard echocardiographic and longitudinal systolic and diastolic Doppler myocardial imaging measurements for 16 left ventricular segments were tested as potential independent predictors of survival.

Results

Age (hazard ratio [HR], 1.03; P = .03), New York Heart Association class III or IV (HR, 2.47; P = .01), the presence of pleural effusion (HR, 1.79; P = .08), brain natriuretic peptide level (HR, 1.29; P = .01), ejection time (HR, 0.99; P = .13), and peak longitudinal systolic strain of the basal anteroseptal segment (HR, 1.05; P = .02) were independent predictors in the final model.

Conclusions

Multivariate survival analysis identified independent predictors of clinical outcome in patients with AL amyloidosis: New York Heart Association class III or IV, presence of pleural effusion, brain natriuretic peptide level > 493 pg/mL, ejection time < 273 ms, and peak longitudinal systolic basal anteroseptal strain less negative than or equal to −7.5% defined a high-risk group of patients.

Section snippets

Study Population

This study was approved by the institutional review board of the Mayo Clinic. Subjects with AL amyloidosis were prospectively selected from patients undergoing evaluation in the Division of Hematology at the Mayo Clinic (Rochester, MN) from January 1, 2004, through October 31, 2005, and referred for echocardiography. A cohort of 279 consecutive patients with AL amyloidosis who underwent complete echocardiographic and Doppler exams constituted the initial study population. All patients were

Results

Baseline demographic and clinical variables as well as cardiac and hematologic biomarker levels are detailed in Table 1. Standard 2-dimensional and Doppler echocardiographic results are detailed in Table 2. Longitudinal systolic and diastolic DMI measurements for our study population are displayed in Table 3, Table 4, respectively.

The mean age was 63 years, and 155 patients (68%) were men. The heart was the major organ involved in 93 patients (37%), and myocardial biopsy was performed in 37

Discussion

The main findings of the current investigation are as follows: (1) in patients with AL amyloid, clinical variables, biomarkers, and DMI echocardiography were predictive of mortality; (2) ejection time was confirmed as an essential independent predictor of survival; and (3) longitudinal systolic DMI measurements, specifically sSR and sS, were among the most significant independent predictors of survival in multivariate analysis.

Different studies have demonstrated the prognostic usefulness of

Conclusion

We compared for the first time clinical, laboratory, and extensive echocardiographic variables to define the most significant independent predictors of survival for patients with AL amyloid, and the cut points for NYHA class III or IV, presence of pleural effusion, BNP level > 493 pg/mL, and LV ejection time < 273 ms identified patients with the worst prognoses. Similarly, longitudinal sS in the basal anteroseptal segment less negative than −7.5% was a powerful predictor of poor clinical

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