Wild-type Transthyretin Amyloid Deposition in an Ascending Aortic Aneurysm.

Amyloid deposition in aortic tissue is associated with increased stiffness. We report a patient with ascending aortic aneurysm and chronic abdominal aortic dissection who had significant wild-type transthyretin amyloid deposition on surgical pathology. The patient did not have cardiac involvement on further workup.

Contemporary outpatient management of patients with worsening heart failure with reduced ejection fraction: Clinical outcome results from the CHART-HF study.

Background: Based on available data from randomized clinical trials, patients with heart failure with reduced ejection fraction (HFrEF) and worsening HF events (WHFE) have substantial disease burden and poor outcomes. WHFE clinical outcome data in non-clinical trial patients, more representative of the US clinical practice, has not been demonstrated. Methods and results: CHART-HF collected data from two complementary, non-clinical trial cohort with HFrEF (LVEF <45 %): 1) 1,000 patients from an integrated delivery network and 2) 458 patients from a nationwide physician panel. CHART-HF included patients with WHFE between 2017 and 2019 followed by an index outpatient cardiology visit ≤6 months, and patients without WHFE in a given year between 2017 and 2019, with the last outpatient cardiology visit in the same year as the index visit. Compared to patients without WHFE (after covariate adjustment, all p < 0.05), patients with WHFE had a greater risk of HF-related hospitalization (hazard ratio [HR]: 1.53-2.40) and next WHFE event (HR: 1.67-2.41) following index visits in both cohorts. Conclusion: HFrEF patients with recent WHFE consistently had worse clinical outcomes in these non-clinical trial cohorts. Despite advances in therapies, unmet need to improve clinical outcomes in HFrEF patients with WHFE remains.

Treatment patterns of patients with worsening heart failure with reduced ejection fraction.

AIMS: Patients with HFrEF and worsening HF events (WHFE) are at particularly high risk and urgently need disease-modifying therapy. CHART-HF assessed treatment patterns and reasons for medication decisions among HFrEF patients with and without WHFE. METHODS AND RESULTS: CHART-HF collected retrospective electronic medical records of outpatients with HF and EF < 45% between 2017-2019 from a nationwide panel of 238 cardiologists (458 patients) and the Geisinger Health System (GHS) medical record (1000 patients). The index visit in the WHFE cohort was the first outpatient cardiologist visit ≤6 months following the WHFE, and in the reference cohort was the last visit in a calendar year without WHFE. Demographic characteristics were similar between patients with and without WHFE in both the nationwide panel and GHS. In the nationwide panel, the proportion of patients with versus without WHFE receiving ≥50% of guideline-recommended dose on index visit was 35% versus 40% for beta blocker, 74% versus 83% for ACEI/ARB/ARNI, and 48% versus 49% for MRA. The proportion of patients receiving ≥50% of guideline-recommended dose was lower in the GHS: 29% versus 34% for beta-blocker, 16% versus 31% for ACEI/ARB/ARNI, and 18% versus 22% for MRA. For patients with and without WHFE, triple therapy on index date was 42% and 44% of patients from the nationwide panel, and 14% and 17% in the GHS. Comparing end of index clinic visit with 12-month follow-up in the GHS, the proportion of patients on no GDMT increased from 14% to 28% in the WHFE cohort and from 14 to 21% in the non-WHFE group. CONCLUSIONS: Major gaps in use of GDMT, particularly combination therapy, remain among US HFrEF patients. These gaps persist during longitudinal follow-up and are particularly large among patients with recent WHFE.