Background—
A plethora of echo parameters has been suggested for distinguishing cardiac amyloidosis (CA) from other causes of myocardial thickening with, however, scarce data on their head-to-head comparison. This study aimed at comparing the diagnostic accuracy of various deformation and conventional echo parameters in differentiating CA from other hypertrophic substrates, especially in the gray zone of mild hypertrophy (maximum wall thickness ≤16 mm) or normal ejection fraction (EF).
Methods and Results—
We included 100 subjects, of which 40 were patients with newly diagnosed, biopsy-proven CA (65.5±10.8 years, 65% male, 62.5% amyloidosis light chain [AL] type), 40 patients with hypertrophic cardiomyopathy matched for demographics and maximum wall thickness (60.1±14.8 years, 85% male), and 20 hypertensives with prominent myocardial remodeling. Quantifiable conventional morphological and functional parameters along with multidimensional strain and strain-derived ratios indices, previously suggested to diagnose CA, were analyzed. EF global longitudinal strain ratio showed the best performance to discriminate CA (area under the curve, 0.95; 95% confidence intervals, 0.89–0.98;
P
<0.00005). Traditional echo indices showed overall low sensitivities and high specificities (among them myocardial contraction fraction ratio had the highest area under the curve, 0.80; 95% confidence intervals, 0.7–0.87;
P
<0.0001). In the challenging subgroups (maximum wall thickness ≤16 mm and EF>55%), EF global longitudinal strain ratio remained the best predicting parameter of CA diagnosis (multiple logistic regression models
P
<0.00005 and
P
=0.0002, respectively) independent of the CA type.
Conclusions—
Our study demonstrated that in patients with thickened hearts, EF global longitudinal strain ratio has the best accuracy in detecting CA, even among the most “challenging” patient subgroups as those with mild hypertrophy and normal EF.
The aim of this prospective, randomised, open-label, blinded-end point study was to compare the efficacy and safety of eplerenone versus spironolactone in patients with bilateral idiopathic hyperaldosteronism (IHA). After a 2-week washout period, 34 patients with IHA were assigned to receive either spironolactone 25 mg b.i.d. (n = 17) or eplerenone 25 mg b.i.d. (n = 17) for 24 weeks. If the patients' blood pressure (BP) was not < 140/90 mmHg, the doses were gradually increased up to 400 mg for spironolactone and 200 mg for eplerenone. If the patients' BP remained uncontrolled, a daily dose of hydrochlorothiazide 12.5 mg was added at week 16. The primary outcome was the percentage of patients with BP < 140/90 mmHg at 16 weeks (i.e., with aldosterone antagonist monotherapy). The patients' BP was normalised in 13 out of 17 (76.5%) and 14 out of 17 (82.4%) patients in the spironolactone and eplerenone groups, respectively (p = 1.00). Systolic BP decreased more rapidly with eplerenone. Serum potassium levels were normalised (> 3.5 mmol/l) in all patients at 4 weeks. Mild hyperkalaemia was observed in two patients receiving 400 mg of spironolactone and in three patients receiving 150 mg of eplerenone. Two patients presented with bilateral painful gynaecomastia at the end of week 16 while receiving 400 mg of spironolactone. Switching spironolactone to 150 mg of eplerenone daily resulted in resolution of gynaecomastia and also maintained BP control. At the end of the study, 19 patients were on eplerenone and 15 were on spironolactone. However, this did not affect the primary end point, because the switch from spironolactone to eplerenone (in two patients) occurred at the end of week 16. It was concluded that eplerenone was as effective as spironolactone in reducing BP in patients with IHA. The risk of mild hyperkalaemia was similar with both drugs.
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