Garvan, Kane C

Abstract Background Wild type transthyretin cardiac amyloidosis (wtATTR-CA) is increasingly recognized as a cause of heart failure with preserved ejection fraction (HFpEF) but prognosis is often limited due to late or misdiagnosis.Longitudinal left ventricular strain and biomarkers are established as markers of disease severity, but the role of RV free wall strain, reflecting RV contractility, is less well understood. Purpose We sought to determine whether RV free wall strain might add incremental prognostic value in wtATTR-CA. Methods Consecutive patients diagnosed with wtATTR-CA with tissue confirmation at Mayo clinic between 2013 and 2015 were included. Patients with TTR gene mutations were excluded. Baseline characteristics and transthoracic echocardiography measurements were obtained from the medical records. Speckle tracking RV free wall longitudinal 2D strain and peak LA longitudinal 2D strain were measured using Tom Tec Imaging System. Survival was determined using Kaplan Meier estimates and using the cox proportional hazard ratio, univariate and multivariable analysis were performed to identify predictors of mortality in patients with wtATTR. Results The study group comprised 139 patients (mean age 74.9±8.6, 92.8% male), of which 102 had adequate image quality for RV strain, and 99 for LA strain. Amongst these, 102 (73.3%) had AF and 118 patients (84.8%) had HF. During 3.23±2.0 years of follow up, 66 patients died. Both mean RV and LA strain were impaired at baseline: RV free wall strain was −14.7±4.9, and peak atrial longitudinal strain (PALS) was 13.2±8.8%. Using ROC analysis, RV strain of −16.8% was an independent predictor of all-cause mortality. In univariate modeling, higher levels of NT-proBNP (HR: 1.1 per 1000 pg.ml; 95%, CI 1.05–1.15, p<0.001) and Troponin T (HR: 2.0 per 0.1ng/ml; 95% CI 1.49–2.61, p<0.001) were associated with increased all-cause mortality. In addition, LV GLS (HR: 1.13 per 1%; 95% CI1.04–1.24, p=0.003), RV free wall LS (HR: 2.16 per 5%; 95%, CI 1.57–3.03, p<0.0001), and PALS (HR: 0.91 per1%; 95% CI 0.85–0.96, p<0.0001) were univariate predictors of all-cause mortality. In multivariate analysis using a stepwise regression model, RV free wall longitudinal strain (HR: 1.81; 95% CI 1.29–2.62, p<0.001) and Troponin T (HR: 1.7; 95% CI 1.25–2.26, p=0.001) remained independent predictors. Kaplan-Meier survival analysis demonstrated a higher mortality rate above −16.8 RV strain cut-off (Wilcoxon <0.0001). All stages were divided into two groups by −16.8% RV strain, and survival in individual stages analyzed. Stage 1 and 2 with <−16.8 RV free wall strain value had higher mortality than ≤-16.8% RV strain (Stage 1: Wilcoxon = 0.0041 and Stage 2: Wilcoxon = 0.023). However, there was not a survival difference between two RV strain groups in stage3 (Wilcoxon = 0.34) Conclusion RV free wall strain is an independent predictor of survival in wtATTR patients and may add incremental prognostic value to NT-proBNP and Troponin. Funding Acknowledgement Type of funding sources: None. Kaplan-Meier curve of all patientsKaplan-Meier curve of stages

Introduction: Pericardial decompression syndrome (PDS) has been reported as a spectrum of cardiac decompensation following pericardiocentesis including ventricular failure, cardiogenic shock and pulmonary edema. PDS has been reported in patients undergoing drainage ≥450 mL of pericardial fluid. Hence judicious drainage of large volume effusions to avoid complication has been recommended Hypothesis: PDS complicating undergoing transthoracic echocardiography (TTE) guided pericardiocentesis is uncommen Methods: Consecutive patients undergoing TTE guided pericardiocentesis at our center from 1/2007 to 12/2016 were reviewed. Procedures were performed in a standard fashion under moderate conscious sedation with TTE determining the location of aspiration and determining adequate resolution of pericardial fluid post procedure. All fluid was removed at time of centesis regardless of the size of effusion, except for patients with severe pulmonary hypertension (n=9), who had staged removal of 100cc every 10 minutes. Post procedure, a pigtail catheter was left in the pericardial space for ongoing intermittent drainage and removed after the total net output was <50 mL/24 hours. Patients were monitored in an intermediate level care unit. All patients underwent repeat TTE at the time of catheter removal. Large volume was defined as ≥450 mL of fluid. Patients in whom pericardiocentesis was technically unsuccessful were excluded Results: Over a 10-year period a total of 1067 patients (57% male, mean age 62±16 years) underwent 1164 technically successful pericardiocentesis procedures. Of these, 561 patients had large volume (≥450 mL) aspirated (mean 750±345 mL aspirated, range 450 - 4300 mL). Patients had a pericardial catheter in place for a mean 2.9±2 days. One patient with pre-existing severe biventricular dysfunction after aspiration of 400cc developed hypotension that improved with reversal of sedation, but proceeded to have PEA arrest and die. No other patient developed clinical events of acute left or right heart failure, ventricular dysfunction, unexplained hypotension, cardiogenic shock or other clinical events that could relate to PDS Conclusions: At most one episode of PDS was observed after technically successful percutaneous pericardiocentesis in 1164 cases (561 were large volume), although whether this case was PDS is unclear. PDS appears to be very rare, and staging pericardial decompression over time may be unnecessary, other than, perhaps, in patients with severe ventricular dysfunction or severe pulmonary hypertension