Genotype and Phenotype of Transthyretin Cardiac Amyloidosis: THAOS (Transthyretin Amyloid Outcome Survey)

Background Transthyretin amyloidosis (ATTR) is a heterogeneous disorder with multiorgan involvement and a genetic or nongenetic basis. Objectives The goal of this study was to describe ATTR in the United States by using data from 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 with data from patients from other regions of the world (ROW) (n = 2,140). The focus was on the phenotypic expression and survival in the majority of U.S. subjects with valine-to-isoleucine substitution at position 122 (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 the 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 (85%) of 107 patients with Val122Ile were from the United States, where Val122Ile subjects were younger and more often female and black than patients with wild-type disease, 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 transthyretin 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 male subjects with a cardiac-predominant phenotype. Val122Ile is the most common transthyretin mutation, and neurologic phenotypic expression differs between wild-type disease and Val122Ile, but survival from enrollment in THAOS does not. (Transthyretin-Associated Amyloidoses Outcome Survey [THAOS]; NCT00628745

SGLT-2 inhibitors for patients with heart failure: What have we learned recently?

Purpose of review: In this review, we discuss the mechanisms of action of sodium-glucose cotransporter-2 inhibitors (SGLT-2i) and the purported protective effects for mitigating heart failure (HF)-related outcomes.Recent findings: Major randomized clinical trials have demonstrated the cardiovascular safety and efficacy of SGLT-2i among patients without known HF and those with established HF with reduced ejection fraction or preserved ejection fraction (HFrEF and HFpEF respectively). Recent HF guidelines have incorporated SGLT-2i in HF treatment algorithms. SGLT-2i have emerged as a novel treatment for both prevention of HF and reduction of cardiovascular morbidity and mortality among patients with existing HFrEF or HFpEF

Stakeholder Insights from Zika Virus Infections in Houston, Texas, USA, 2016–2017

Responding to Zika virus infections in Houston, Texas, USA, presented numerous challenges across the health system. As the nation’s fourth-largest city, in a subtropical region with high travel volume to Latin America and the Caribbean, Houston was an ideal location for studying experiences encountered by clinicians and public health officials as they responded to the Zika virus crisis. To identify the challenges encountered in the response and to explore strategies to improve future responses to emerging infectious diseases, we interviewed 38 key stakeholders who were clinical, scientific, operational, and public health leaders. From the responses, we identified 4 key challenges: testing, travel screening, patient demographics and immigration status, and insufficient collaboration (between public health officials and clinicians and among clinical providers). We also identified 5 strategic areas as potential solutions: improved electronic health record support, specialty centers and referral systems, standardized forms, centralized testing databases, and joint academic/public health task forces

Abstract 9573: Advance Care Planning and Prognosis Education in Patients With Heart Failure: Insights from the NCDR PINNACLE Registry

Introduction: Advance care planning (ACP) and prognosis education are key components of high-quality, patient-centered care in heart failure (HF) and associated with better patient-reported outcomes. While research suggests that these occur infrequently in HF, studies have been limited to small and regional samples. We examined the prevalence of documented ACP and prognosis/end-of-life education as well as their associations with healthcare utilization in a large national sample of outpatients with HF. Method: Among patients with HF in the NCDR PINNACLE Registry enrolled between January 2013 and June 2018, we determined the proportion receiving ACP and prognosis education. T-tests and chi-squares were used to examine characteristics of ACP and prognosis education. In those over 65 who could be linked with Medicare claims data, adjusted multivariable linear and two-level hierarchical logistic regression modelss were conducted to examine the associations of ACP and prognosis education with inpatient and outpatient utilization. The first heart failure encounter for each patient was analyzed for presence of ACP and prognosis education. Results: Among 1.6 million patients with HF (Mage = 68.6+14.3), approximately 34% received ACP, while only 2% received prognosis education. Rates of ACP and prognosis education were lower in women, patients in the northeast and rural areas, and those in NYHA classes III-IV. Both ACP and prognosis education were low overall among patients with cardiac devices. Among 276,916 patients linked to Medicare claims data (Mage = 77.2+7.8), ACP was associated with fewer outpatient visits (p \u3c 0.001), and both a lower likelihood of 30-day all-cause (OR = 0.86; 95% CI = 0.82-0.90) and HF related admissions (OR = 0.78, 95% CI = 0.73-0.84). A similar pattern was observed for prognosis education. Conclusions: Less than half of patients with HF had documented ACP and almost no patients had documented prognosis education, despite being quality metrics in PINNACLE. Further, these quality metrics were associated with lower healthcare utilization rates. Research is needed to better understand the factors that promote ACP and prognosis education, and the effects of these quality metrics on clinical and patient-reported outcomes