A comprehensive cardiac assessment will unravel established CM in almost all patients with FA with electrocardiographic abnormalities as earliest signs. Advanced stages can be characterized by elevated hsTNT and replacement fibrosis leading to recession of hypertrophy, reduction of global myocardial function, and electrical instability.

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ObjectivesSince diastolic abnormalities are typical findings of cardiac amyloidosis (CA), we hypothesized that speckle-tracking-imaging (STI) derived longitudinal early diastolic strain rate (LSRdias) could predict outcome in CA patients with preserved left ventricular ejection fraction (LVEF >50%).BackgroundDiastolic abnormalities including altered early filling are typical findings and are related to outcome in CA patients. Reduced longitudinal systolic strain (LSsys) assessed by STI predicts increased mortality in CA patients. It remains unknown if LSRdias also related to outcome in these patients.MethodsConventional echocardiography and STI were performed in 41 CA patients with preserved LVEF (25 male; mean age 65±9 years). Global and segmental LSsys and LSRdias were obtained in six LV segments from apical 4-chamber views.ResultsNineteen (46%) out of 41 CA patients died during a median of 16 months (quartiles 5–35 months) follow-up. Baseline mitral annular plane systolic excursion (MAPSE, 6±2 vs. 8±3 mm), global LSRdias and basal-septal LSRdias were significantly lower in non-survivors than in survivors (all p<0.05). NYHA class, number of non-cardiac organs involved, MAPSE, mid-septal LSsys, global LSRdias, basal-septal LSRdias and E/LSRdias were the univariable predictors of all-cause death. Multivariable analysis showed that number of non-cardiac organs involved (hazard ratio [HR]  = 1.96, 95% confidence interval [CI] 1.17–3.26, P = 0.010), global LSRdias (HR = 7.30, 95% CI 2.08–25.65, P = 0.002), and E/LSRdias (HR = 2.98, 95% CI 1.54–5.79, P = 0.001) remained independently predictive of increased mortality risk. The prognostic performance of global LSRdias was optimal at a cutoff value of 0.85 S−1 (sensitivity 68%, specificity 67%). Global LSRdias <0.85 S−1 predicted a 4-fold increased mortality in CA patients with preserved LVEF.ConclusionsSTI-derived early diastolic strain rate is a powerful independent predictor of survival in CA patients with preserved LVEF.

Advanced coronary artery disease (CAD), impaired left ventricular function and prolongation of the QT-interval are considered risk factors for sudden cardiac death in CAD-patients. So far, however, there are no studies investigating in detail whether there is a correlation between the QT-interval and changes in coronary anatomy or changes in left ventricular function. Therefore, coronary angiographic data were related to QT-intervals in 304 patients, who were catheterized because of suspected coronary artery disease. QT-intervals were expressed as QTc = QT/square root RR (Bazett's correction for heart rate), left ventricular function was assessed by the ejection fraction of the ventricular angiogram, and coronary angiograms were classified according to the Gensini score as well as into 1-, 2- and 3-vessel disease (stenoses greater than or equal to 50%). A multidimensional linear regression model was employed to eliminate the effects of varying mean rates still present after application of Bazett's formula. In patients with 1-, 2- and 3-vessel disease, significant changes of QTc were observed only in patients with impaired left ventricular function (EF less than 60%). In these patients the QTc-interval increased significantly from 1- to 3-vessel disease. If the critical degree of coronary stenosis was changed from greater than or equal to 50% to greater than or equal to 90% further prolongations of QTc were noted. In patients with 1-, 2- and 3-vessel disease the QTc-duration difference was further enhanced if either the proximal part of the descending branch of the left coronary artery (LAD) or the left main stem were affected (stenoses greater than or equal to 50%). The data reveal that prolongation in the duration of electrical systole correlates with known cardiac risk factors for sudden death, i.e. 3-vessel-disease, proximal LAD or left main stem stenosis and impaired left ventricular function. In the individual patient, however, the prognostic value of a single QTc-determination is limited because of a large interindividual variation of the data.