ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 2 of 2-Diagnostic criteria and appropriate utilization.

Cardiac amyloidosis is emerging as an underdiagnosed cause of heart failure and mortality. Growing literature suggests that a noninvasive diagnosis of cardiac amyloidosis is now feasible. However, the diagnostic criteria and utilization of imaging in cardiac amyloidosis are not standardized. In this paper, Part 2 of a series, a panel of international experts from multiple societies define the diagnostic criteria for cardiac amyloidosis and appropriate utilization of echocardiography, cardiovascular magnetic resonance imaging, and radionuclide imaging in the evaluation of patients with known or suspected cardiac amyloidosis.

ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI Expert Consensus Recommendations for Multimodality Imaging in Cardiac Amyloidosis: Part 2 of 2-Diagnostic Criteria and Appropriate Utilization.

Cardiac amyloidosis is emerging as an underdiagnosed cause of heart failure and mortality. Growing literature suggests that a noninvasive diagnosis of cardiac amyloidosis is now feasible. However, the diagnostic criteria and utilization of imaging in cardiac amyloidosis are not standardized. In this paper, Part 2 of a series, a panel of international experts from multiple societies define the diagnostic criteria for cardiac amyloidosis and appropriate utilization of echocardiography, cardiovascular magnetic resonance imaging, and radionuclide imaging in the evaluation of patients with known or suspected cardiac amyloidosis.

Outcomes of Octogenarian Patients Treated with Tafamidis for Transthyretin Amyloid Cardiomyopathy.

Patients with transthyretin amyloid cardiomyopathy (ATTR-CM) benefit from disease-modifying agents such as tafamidis. However, the survival benefit of tafamidis in elderly patients (age ≥80 years) is not reported. This study aimed to assess the survival of patients with ATTR-CM aged 80 years and older who were treated with tafamidis compared with patients aged <80 years. We conducted a retrospective analysis of patients with ATTR-CM who underwent tafamidis treatment, aged 45 to 97 years at the time of diagnosis between January 1, 2008, and May 31, 2021. A total of 484 patients were included, with 208 in the ≥80 years group and 276 in the <80 years group. The cohort was followed up for mortality outcomes, and hazard ratios with 95% confidence intervals were calculated. After a median follow-up of 18.5 months, 72 deaths were recorded in the entire cohort. Kaplan-Meier curves showed no differences in survival probability between the 2 groups at 30 months (p for log-rank test = 0.76). The survival rates for patients aged ≥80 years who underwent treatment at 1, 2, 3, 4, and 5 years were 94.7%, 86.0%, 77.0%, 77.0%, and 38.5%, respectively. The corresponding rates for patients aged <80 years who underwent treatment were 93.2, 84.8, 74.4, 68.2, and 64.6%, respectively. In the multivariable analysis, the hazard ratio (95% confidence interval) of the mortality comparing treatment patients aged ≥80 years with those aged <80 years was 0.81 (0.41 to 1.61). In conclusion, tafamidis treatment is associated with similar reductions in mortality in older and younger patients with ATTR-CM.

Impact of patisiran on health status and quality of life in patients with transthyretin cardiac amyloidosis

INTRODUCTION APOLLO-B is a Phase 3 study of patisiran in patients with transthyretin (ATTR) cardiac amyloidosis (NCT03997383), which demonstrated a significant benefit in functional capacity (6-MWT), and health status and quality of life (QoL) (KCCQ-OS) with patisiran vs placebo at Month (M) 12. HYPOTHESIS Patisiran improves health status and QoL in the daily lives of patients with ATTR cardiac amyloidosis vs placebo. METHODS Patients were 18-85 years old with ATTR amyloidosis and a medical history of heart failure (HF) due to ATTR cardiomyopathy, with ≥1 prior hospitalization for HF or current clinical evidence of HF. Patients were randomized (1:1) to intravenous patisiran 0.3 mg/kg or placebo every 3 weeks. These post-hoc analyses evaluated percentage of responders reporting ≥5-point improvement in KCCQ-OS, and change from baseline in 4 KCCQ domains and questions within the domains. RESULTS 359 patients received study drug (patisiran, N=181; placebo, N=178): median age (range), 76 (41, 85) years; male, 89%; wild-type ATTR, 80%; 25% were on tafamidis at baseline. At M12, patisiran showed significant benefit vs placebo in KCCQ-OS (LS mean [SEM] change from baseline: patisiran, 0.30 [1.26]; placebo, -3.41 [1.28]; LS mean [SEM] difference: 3.71 [1.80]; p=0.0397). A ≥ 5-point improvement in KCCQ at M12 was more frequent with patisiran vs placebo (34.1 vs 24.0%: difference [95% CI] 10.1% [0.7, 19.5]). Improvement vs placebo was consistent across domains, with LS mean differences [95% CI] in change from baseline (patisiran - placebo) in Physical Limitations (2.75 [-1.24, 6.74]), Total Symptoms (4.55 [0.75, 8.34]), QoL (4.27 [-0.12, 8.65]), and Social Limitations (2.76 [-2.21, 7.73]). Categorical changes from baseline to M12 demonstrated greater percentages of placebo-treated patients reporting worsening for questions in each domain, including activities requiring greater cardiometabolic demand. In patients with values at baseline and M12, notably greater percentages (>5%) of placebo- vs patisiran-treated patients reported worsening (percent difference; n=placebo/patisiran) for questions related to Walking 1 Block on Level Ground (10%; n=159/162), Frequency and Burden of Dyspnea (9.5% and 7.6%; n=164/170), Frequency of Orthopnea (9.6%; n=163/170), Feeling about Spending the Rest of Their Life with HF the Way It Is Right Now (6.4%; n=164/170), and Intimate Relationships (6.3%; n=88/86). Improvement from baseline was reported by greater percentages (>5%) of patisiran-treated patients (percent difference; n=patisiran/placebo) in Enjoyment of Life Limited Due to HF (12.8%; n=170/164) and Hobbies/Recreational Activities (6.0%; n=141/143). CONCLUSIONS In APOLLO-B, improvements in health status and QoL with patisiran vs placebo were apparent across all 4 KCCQ domains. Greater percentages of patisiran-treated patients had KCCQ-OS improved by ≥ 5 points at M12 and they more often reported improvements in QoL, and ability to enjoy life and perform hobbies/recreational activities. More placebo-treated patients reported worsening in walking on level ground, HF symptoms and QoL.

Digoxin therapy for cor pulmonale: A systematic review.

OBJECTIVE: Right heart failure is associated with increased mortality and morbidity. The optimal treatment for patients with RV failure is not established. The aim of this study is to conduct a systematic review of the literature to assess the relative benefits and harms of digoxin therapy in patients with RV failure. METHODS: We performed a literature search in MEDLINE, EMBASE, and Cochrane Central Register of Controlled Trials (CENTRAL) on Nov. 4, 2014. We did not use publication type, period or language restrictions to the search strategy. Exclusions included: trials that excluded patients with RV failure, included patients requiring mechanical or intravenous inotropic support, review papers and case reports. The primary outcome was long-term efficacy outcomes of digoxin in right heart failure. Two reviewers screened titles and abstracts of identified citations independently and in duplication using calibration exercises and standardized screening forms. RESULTS: The search strategy identified 4097 citations, and 4 studies were included in this analysis (n=76 patients). Of the four studies, two assessed improvements in RVEF, two studies compared exercise capacity indexes, and one assessed symptoms with digoxin compared with placebo. No study assessed mortality outcomes. Overall, there was no statistically significant improvement in RVEF, exercise capacity, NYHA class, heart failure score, or body weight. CONCLUSIONS: There are few studies evaluating Digitalis use for RV failure, which are limited to patients with cor pulmonale. In these patients, Digitalis use provides no improvement in RVEF, exercise capacity, or NYHA class. Randomized clinical trials are needed to address this question.

Extracorporeal ultrafiltration vs. conventional diuretic therapy in advanced decompensated heart failure.

Compared with conventional diuretic (CD) therapy, ultrafiltration (UF) is associated with greater weight loss and fewer re-hospitalizations in patients admitted with decompensated heart failure (HF). Concerns have been raised regarding its safety and efficacy in patients with more advanced heart failure. The authors conducted a single-center, prospective, randomized controlled trial in patients with advanced HF admitted to an intensive care unit for hemodynamically guided therapy, comparing UF (n=17) with CD (n=19) at admission. The primary end point was the time required for pulmonary capillary wedge pressure (PCWP) to be maintained at a value of ≤18 mm Hg for at least 4 consecutive hours. Secondary end points included levels of cytokines and neurohormones, as well as several clinical outcomes. In our study cohort, the time to achieve the primary end point was lower in the UF group but did not reach statistical significance (P = .08). UF resulted in greater weight reduction, higher total volume removed, and shorter hospital length of stay. There were no differences in kidney function, biomarkers, or adverse events. In patients with advanced HF under hemodynamically tailored therapy, UF can be safely performed to achieve higher average volume removed than CD therapy without leading to adverse outcomes.