Echocardiographic findings in systemic amyloidosis: Spectrum of cardiac involvement and relation to survival (original) (raw)
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Journal of Clinical Medicine, 2021
Aims: Multimodal imaging has allowed cardiac amyloidosis (CA) to be increasingly recognised as a treatable cause of heart failure with preserved ejection fraction, but its prognosis remains poor due to late diagnosis. To assess the left ventricular diastolic function (LVDF) patterns in a large contemporary CA cohort according to the current recommendations and to identify their determinants. Methods and Results: We conducted a monocentric, observational study on a cohort of CA patients from a tertiary CA referral centre. Diastolic function was analysed using standard echocardiography and clinical, laboratory and survival parameters were collected. Four hundred and sixty-four patients with one of the three main type of CA were included: 41% had grade III diastolic dysfunction (restrictive mitral pattern), 25% had grade II diastolic dysfunction, and 25% had grade I diastolic dysfunction; 9% were unclassified. No difference was found between the main CA types. After multivariate analys...
Journal of The American Society of Echocardiography, 2007
We examined the potential role of Doppler myocardial imaging including tissue velocity imaging, strain imaging, and strain rate imaging for detection of left ventricular systolic dysfunction in cardiac amyloidosis (CA) and determined the minimum dataset required to make the diagnosis. Methods and Results: Doppler myocardial imaging was performed in 103 patients with amyloidosis (AL). Peak longitudinal systolic tissue velocity, systolic strain rate (sSR), and systolic strain (sS) were determined for 16 left ventricular segments. Radial and circumferential sSR and sS were also measured. Patients with increased left ventricular wall thickness were classified with advanced-CA, and the remainder of the patients were classified with ALnormal-wall-thickness. The global means of peak systolic tissue velocity (3.6 ؎ 1.0 vs. 3.9 ؎ 0.9, P ؍ .007), sSR (؊0.8 ؎ 0.3 vs. ؊1.0 ؎ 0.2, P < .001), and sS (؊9.9 ؎ 3.7 vs. ؊15.6 ؎ 3.3, P < .001) were significantly lower in advanced-CA compared with AL-normal-wall-thickness. The mean of either sSR or sS from 6 middle or all 16 segments similarly differentiated patients with advanced-CA from AL-normalwall-thickness. Conclusions: Longitudinal sS most accurately detects longitudinal systolic dysfunction in AL and best differentiates patients with advanced-CA with increased ventricular thickness from patients with AL-normal-wall-thickness. Interrogation of six middle segments was sufficient in identifying patients with advanced-CA. Further studies are warranted to define the incremental prognostic value of these new parameters in predicting outcomes for patients with AL. (J Am Soc Echocardiogr
Circulation, 1984
The purpose of this study was to determine whether changes in myocardial wall echogenicity that suggest amyloid disease could be prospectively identified by a qualitative analysis of two-dimensional echocardiographic images. Two thousand seventy-eight consecutive echocardiograms obtained over a 14 month period were prospectively analyzed. The myocardial walls of 30 patients showed multiple, discrete, small highly refractive echoes; amyloid disease was not known or suspected in any of them. It was recommended that all 30 patients undergo gingival biopsy to confirm the diagnosis and biopsy was performed in 15 patients. The recommendation for biopsy was made only on the basis of two-dimensional echocardiographic images and was independent of findings regarding thickness of the walls or the dimensions of the cardiac chambers. Results of biopsy were positive in 11 patients and negative in four. We conclude that qualitative evaluation of two-dimensional echocardiographic images can identify changes in myocardial wall echogenicity that correlate with a result of gingival biopsy positive for amyloidosis. In patients who have a typical myocardial texture by twodimensional echocardiography and a positive gingival biopsy result, cardiac amyloidosis should be strongly suspected. Circulation 70, No. 3, 432-437. 1984. CLINICALLY SIGNIFICANT cardiac amyloidosis accounts for 5% to 10% of all forms of isolated noncoronary cardiomyopathy.' The two-dimensional echocardiographic features of the myocardium in amyloid heart disease have been described in patients with clinical and histologic evidence of diffuse amyloidosis. Cardiac walls are thickened and ventricular myocardium shows the presence of multiple, discrete, and small highly refractive echoes that are related to the amyloid deposits.1-l l The purpose of this study was to determine whether changes in myocardial wall echogenicity that suggest amyloid disease could be detected prospectively by qualitative evaluation of the myocardial texture. Methods Two-dimensional echocardiograms. Two thousand seventy-eight consecutive echocardiograms obtained over a period of 14 months in our laboratory for diagnostic purposes were prospectively analyzed. Qualitative evaluation of the echocardiographic images was performed at the time of the examination.
2010
The purpose of this study was to determine whether changes in myocardial wall echogenicity that suggest amyloid disease could be prospectively identified by a qualitative analysis of two-dimensional echocardiographic images. Two thousand seventy-eight consecutive echocardiograms obtained over a 14 month period were prospectively analyzed. The myocardial walls of 30 patients showed multiple, discrete, small highly refractive echoes; amyloid disease was not known or suspected in any of them. It was recommended that all 30 patients undergo gingival biopsy to confirm the diagnosis and biopsy was performed in 15 patients. The recommendation for biopsy was made only on the basis of two-dimensional echocardiographic images and was independent of findings regarding thickness of the walls or the dimensions of the cardiac chambers. Results of biopsy were positive in 11 patients and negative in four. We conclude that qualitative evaluation of two-dimensional echocardiographic images can identify changes in myocardial wall echogenicity that correlate with a result of gingival biopsy positive for amyloidosis. In patients who have a typical myocardial texture by twodimensional echocardiography and a positive gingival biopsy result, cardiac amyloidosis should be strongly suspected.
American Journal of Cardiology, 2008
We examined the potential role of Doppler myocardial imaging for early detection of systolic dysfunction in patients with systemic amyloidosis (AL) but without evidence of cardiac involvement by standard echocardiography. We identified 42 patients without 2-dimensional echocardiographic or Doppler evidence of cardiac involvement. These patients had normal ventricular wall thickness and normal velocity of the medial mitral annulus. Myocardial images were obtained in these patients and in 32 age-and gendermatched healthy controls. Peak longitudinal systolic tissue velocity (sTVI), systolic strain rate (sSR), and systolic strain (sS) were determined for 16 left ventricular segments. Radial and circumferential sSR and sS were also measured. Compared with controls in this group of patients with AL, peak longitudinal sSR (؊1.0 ؎ 0.2 vs ؊1.4 ؎ 0.2, p <0.001) and peak longitudinal sS (؊15.6 ؎ 3.3 vs ؊22.5 ؎ 2.0 p <0.001) were significantly decreased. In conclusion, the mean sS from all 6 basal segments, or from all 16 left ventricular segments differentiated patients with AL with normal echocardiography from controls, with similar accuracy for the mean sSR from the 6 basal segments. This distinction was not apparent from peak longitudinal sTVI or from radial or circumferential sSI or sSR.
Echocardiographic Evaluation of Systolic and Diastolic Function in Patients With Cardiac Amyloidosis
The American Journal of Cardiology, 2011
The typical appearance of cardiac amyloidosis using standard echocardiographic techniques is usually a late finding only in patients with relatively advanced stages of the disease. Early noninvasive identification of cardiac amyloidosis is of growing clinical importance. Newer echocardiographic techniques, including tissue Doppler imaging and deformation imaging (strain rate imaging and 2-dimensional speckle tracking), are powerful tools for quantifying regional myocardial motion and deformation. Using these advanced techniques, early functional impairment in cardiac amyloidosis may be detectable when the results of standard echocardiography are still normal or inconclusive. This review provides a comprehensive overview of the different echocardiographic approaches for the assessment of systolic and diastolic function in patients with cardiac amyloidosis. Special attention is paid to regional myocardial function assessed by tissue Doppler imaging, strain rate imaging, and 2-dimensional speckle-tracking imaging.
European Journal of Heart Failure, 2007
Background: Patients with AL amyloidosis often present with signs of congestive heart failure. Aim: This study was prospectively designed to assess the significance of RV dysfunction in AL amyloidosis. Methods and results: Seventy-four patients with biopsy proven AL amyloidosis underwent a thorough echocardiographic evaluation. A tricuspid annular plane systolic excursion (TAPSE) b 17 mm was taken as marker of RV dysfunction. Plasma NT-proBNP determinations were performed in all cases. RV function was normal in 60 patients and reduced in 14 patients. Patients with RV dysfunction had thicker left ventricular (LV) walls (p b 0.01), lower LV end-diastolic volumes (p b 0.01), lower LV ejection fraction (p b 0.01) and more frequently a restrictive LV filling pattern (p b 0.01). RV dimensions and RV free wall thickness were not significantly different in the two groups. A thick interventricular septum and a reduced TAPSE were associated with high NT-proBNP levels (both p b 0.01). Seven patients died during a median follow-up period of 19 months; TAPSE b 17 mm was the only echocardiographic parameter associated with poor survival. Conclusion: In patients with AL amyloidosis, RV dysfunction is associated with more severe involvement of the left ventricle, higher plasma levels of NT-proBNP and with poor prognosis.
Left atrial myopathy in cardiac amyloidosis: implications of novel echocardiographic techniques
European Heart Journal, 2004
Aims To assess left atrial (LA) function and determine the prevalence of LA dysfunction in AL amyloidosis (AL) using conventional and strain echocardiography. Methods and results LA ejection fraction, LA filling fraction, LA ejection force, peak LA systolic strain rate (LAsSR), and LA systolic strain (LA 1) were determined in 95 AL patients (70 with and 25 without echocardiographic evidence of cardiac involvement, abbreviated CAL and NCAL, respectively), 30 age-matched controls (CON), and 20 patients with diastolic dysfunction and LA dilatation (DD). Peak LAsSR .2 standard deviations below mean CON value was used as the cut-off for normal LA function. LA ejection fraction was lower in CAL when compared with CON (40.4 + 13.6 vs. 67.0 + 6%, P ¼ 0.01). Left atrial septal strain rate and strain were lower in CAL (0.8 + 0.5 s -1 and 5.5 + 4%, respectively) compared with CON (1.8 + 0.8 s -1 and 14 + 4%, respectively, P ¼ ,0.0001), NCAL (1.6 + 0.8 s -1 and 13 + 7%, respectively, P , 0.0001) and DD (1.3 + 0.4 s -1 and 10 + 2%, respectively, P , 0.0001). Based on peak LA systolic strain rate criteria, the cut-off values for normal LA function were -1.1 s -1 and -1.05 s -1 for lateral and septal walls. Using these criteria, LA dysfunction was identified in 32% (lateral LA criteria) and 60% (septal LA criteria) of CAL patients. Lateral and septal LAsSR were lower in CAL patients with vs. those without symptoms of heart failure. Inter-and intra-observer agreement was high for LA strain echocardiography. Conclusion LA function assessment using strain echocardiography is feasible with low intra-and inter-observer variability. LA dysfunction is observed in AL patients without other echocardiographic features of cardiac involvement and may contribute to cardiac symptoms in CAL.