No abstract

No abstract

No abstract

No abstract

No abstract

Background-The landmark Randomized Evaluation of Mechanical Assistance for the Treatment of Congestive HeartFailure (REMATCH) trial first demonstrated that implantation of left ventricular assist devices (LVADs) as destination therapy (DT) can provide survival superior to any known medical treatment in patients with end-stage heart failure who are ineligible for transplantation. In the present study, we describe outcomes of DT in the post-REMATCH era in the United States. Methods and Results-The present study included 280 patients who underwent HeartMate XVE LVAD implantation between November 2001 and December 2005. A preoperative risk score for in-hospital mortality after LVAD implantation was established in 222 patients with complete data. All patients were followed up until death or December 2006. The 1-year survival after LVAD implantation was 56%. The in-hospital mortality after LVAD surgery was 27%.The main causes of death included sepsis, right heart failure, and multiorgan failure. The most important determinants of in-hospital mortality were poor nutrition, hematological abnormalities, markers of end-organ or right ventricular dysfunction, and lack of inotropic support. Stratification of DT candidates into low (nϭ65), medium (nϭ111), high (nϭ28), and very high (nϭ18) risk on the basis of the risk score calculated from these predictors corresponded with 1-year survival rates of 81%, 62%, 28%, and 11%, respectively. Conclusions-Appropriate

No abstract

BACKGROUND Transthyretin amyloidosis (ATTR) is a heterogeneous disorder with multiorgan involvement and a genetic or nongenetic basis. OBJECTIVES We described ATTR in the United States in the THAOS (Transthyretin Amyloidosis Outcomes Survey) registry. METHODS Demographic, clinical, and genetic features of patients enrolled in the THAOS registry in the United States (n = 390) were compared to other regions of the world (ROW) (n = 2,140) with a focus on the phenotypic expression and survival in the majority of U.S. subjects with Val122Ile (n = 91) and wild-type ATTR (n = 189). RESULTS U.S. subjects are older (70 vs. 46 years), more often male (85.4% vs. 50.6%) and more often of African descent (25.4% vs. 0.5%) than ROW. A significantly higher percentage of U.S. patients with ATTR amyloid seen at cardiology sites had wild-type disease than the ROW (50.5% vs. 26.2%). In the United States, 34 different mutations (n = 201) have been reported, with the most common being Val122Ile (n = 91; 45.3%) and Thr60Ala (n = 41; 20.4%). Overall, 91 of 107 patients with Val122Ile (85%) were from the United States, where Val122Ile subjects were younger and more often women and black than wild-type patients, and had similar cardiac phenotype but a greater burden of neurologic symptoms (pain, numbness, tingling, and walking disability) and worse quality of life. Advancing age and lower mean arterial pressure, but not the presence of a TTR mutation, were independently associated with higher mortality from a multivariate analysis of survival. CONCLUSIONS In the THAOS registry, ATTR in the United States is overwhelmingly a disorder of older adult males with a cardiac-predominant phenotype. Val122Ile is the most common TTR mutation, and neurologic phenotypic expression differs between wild-type disease and Val122Ile, but survival from enrollment in THAOS does not. CLINICAL TRIAL NCT00628745