Identification of wild-type transthyretin cardiac amyloidosis in patients with carpal tunnel syndrome surgery (CACTuS).

AIMS: Wild-type transthyretin cardiac amyloidosis (ATTRwt) is an infiltrative cardiomyopathy with a poor prognosis. The condition is associated with carpal tunnel syndrome (CTS), which often precedes the ATTRwt diagnosis by several years. The aim of the study was (i) to screen patients with a recent history of CTS for ATTRwt using red flags, (ii) to determine whether patients with screened ATTRwt had less advanced disease compared with patients with clinical ATTRwt, and (iii) to assess the sensitivity and specificity of known red flags in ATTRwt. METHODS AND RESULTS: Patients aged ≥60 years at the time of CTS surgery were invited for screening. Red flags were defined as elevated biomarker levels of N-terminal pro-B-type natriuretic peptide (NT-proBNP) or cardiac troponin, an electrocardiogram pattern associated with ATTRwt, left ventricular hypertrophy (LVH), and impaired longitudinal strain with apical sparring. All patients with a red flag were referred for a diagnostic scintigraphy. Patients with ATTRwt diagnosed by screening were compared with patients with clinical ATTRwt (n = 51) matched by age, gender, and CTS surgery. Among the 120 enrolled subjects (mean age 74.5 years, 90% male), the suspicion of ATTR was raised in 67 (55.8%), and 10 (8.3%) were diagnosed with ATTRwt. Patients identified with ATTRwt were predominantly asymptomatic and had mildly elevated NT-proBNP, mildly increased LVH, preserved left ventricular ejection fraction, and systolic longitudinal function, which differed significantly from clinical ATTRwt controls (P < 0.001). CONCLUSIONS: The study found an ATTRwt prevalence of 8.3% in a population of age and gender-selected patients with a recent history of CTS. The identified patients with ATTRwt had less structural and functional cardiac involvement than clinical ATTRwt controls.

Atrial function, atrial volume and cardiovascular clinical outcomes in patients with end-stage renal disease - A study of cardiac computed tomography.

BACKGROUND: Patients with chronic kidney disease (CKD) have an increased risk of cardiovascular events. Previous studies using 2-dimensional echocardiography show that left atrial end-diastolic volume (LAEDV) predicts cardiovascular outcomes and mortality in patients with CKD. However, contrast-enhanced cardiac CT may offers a more precise measure of atrial dimensions and function than 2-dimensional echocardiography and may provide improved predictionof patient outcome. AIM: The aim of the present study was to examine the association of LAEDV and left atrial ejection fraction (LAEF) assessed by CT with left ventricle end-diastolic volume (LVEDV), left ventricular mass, left ventricular ejection fraction and N-terminal plasma-pro-brain natriuretic peptide (NT-PRO-BNP). Furthermore, we examined LAEDV and LAEF as predictors of major adverse cardiac events (MACE) and mortality. METHODS: Kidney transplant candidates (n = 117) underwent contrast-enhanced CT screening for coronary artery disease as part of the work-up prior to kidney transplantation before being accepted on the transplantation waiting list. Left atrial (LA) and left ventricular (LV) volume and function were determined by cardiac CT. MACE and mortality data were extracted from the Western Denmark Heart Registry, a review of patient records and patient interviews. RESULTS: Baseline patient characteristics did not differ between LAEDV tertiles. LAEDV was positively associated with measures of LV function - both LVEDV (ß = 0.36, p < 0.05) and LV mass (ß = 0.30, p < 0.05). LAEF was not associated with measures of LV function. LAEDV was positively and LAEF negatively associated with NT-PRO-BNP (LAEDV: ß = 10.28, p < 0.05. LAEF: ß = -0.06, p < 0.05). During a median follow-up of 3.7-years, 19 (16.2%) patients died and 19 (16.2%) patients suffered MACE. MACE and survival analysis showed no relation to LAEDV or LAEF. CONCLUSIONS: Using contrast-enhanced CT, we demonstrated a correlation between atrial and ventricular functional parameters. However, we found no association with either LAEF or LAEDV or MACE and mortality in this cohort of kidney transplant candidates.