LAUSR

INTRODUCTION Cardiac involvement is a leading cause of morbidity and mortality in primary light chain (AL) amyloidosis. The electrocardiographic spatial QRS-T angle reflects changes in the direction of the repolarization sequence and is a powerful predictor of outcome in patients with heart failure. We examined the significance of the frontal QRS-T angle in predicting the clinical outcome in patients with AL cardiac amyloidosis. METHODS Forty-three consecutive patients with cardiac involvement of AL amyloidosis were studied. Patients were followed for survival. RESULTS Patient median age was 62 years, 56% were males. After a median follow up of 56 months, 16 out of 43 patients had died (37%). The median QRS-T angle was 102° (interquartile range 35-148). QRS-T angle>102° was associated with increased prevalence of lambda free light chain disease and the presence of a pleural effusion. It was also associated with increased interventricular septum thickness, smaller left ventricle end-diastolic diameter, echocardiographic myocardial sparkling texture, pericardial effusion, elevated NT-Pro-BNP and increased restrictive physiology evident by increased E/A and E/e`. A QRS-T angle>102° was a significant predictor of increased mortality by Kaplan-Meier survival analysis (71.6 ± 11.1% vs. 45.7 ± 11.1%, P = .02). A QRS-T angle>102° was an independent predictor of mortality by Cox regression analysis (HR 3.00, 95% CI 1.01-8.89, P < .05). CONCLUSIONS The QRS-T angle is associated with indices of advanced amyloid disease and is an independent predictor of survival.