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Background The emergence of specific therapies for transthyretin cardiac amyloidosis (CA) warrants the need for a systematic review of the literature. Methods and Results A systematic review of the literature was conducted according to Preferred Reporting Items for Systematic Reviews and Meta‐Analyses (PRISMA) guidelines. A systematic search was performed on MEDLINE, PubMed, and Embase databases on November 29, 2019. Studies were selected based on the following predefined eligibility criteria: English‐language randomized controlled trials (RCTs), non‐RCTs, or observational studies, which included adult patients with variant/wild‐type transthyretin‐CA, assessed specific therapies for transthyretin‐CA, and reported cardiovascular outcomes. Relevant data were extracted to a predefined template. Quality assessment was based on National Institute for Health and Care Excellence recommendations (RCTs) or a checklist by Downs and Black (non‐RCTs). From 1203 records, 24 publications were selected, describing 4 RCTs (6 publications) and 16 non‐RCTs (18 publications). Tafamidis was shown to significantly improve all‐cause mortality and cardiovascular hospitalizations and reduce worsening in 6‐minute walk test, Kansas City Cardiomyopathy Questionnaire—Overall Summary score, and NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) in variant/wild‐type transthyretin‐CA. Patisiran showed promising results in a subgroup analysis of patients with variant transthyretin‐CA, which have to be confirmed in RCTs. Inotersen showed conflicting results on cardiac imaging parameters. The one study on AG10 had only a 1‐month duration and cardiovascular end points were exploratory and limited to cardiac biomarkers. Limited evidence from noncomparative single‐arm small non‐RCTs existed for diflunisal, epigallocatechin‐3‐gallate (green tea extract), and doxycycline+tauroursodeoxycholic acid/ursodeoxycholic acid. Conclusions This systematic review of the literature supports the use of tafamidis in wild‐type and variant transthyretin‐CA. Novel therapeutic targets including transthyretin gene silencers are currently under investigation.

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Introduction Coronavirus disease (COVID-19) has led to significant changes in healthcare systems and its impact on the treatment of cardiovascular conditions, such as ST-elevation myocardial infarction (STEMI), is unknown in countries where the healthcare systems were not saturated, as was the case in Portugal. As such, we aimed to assess the effect on STEMI admissions and outcomes in Portuguese centers. Methods We conducted a single-center, observational, retrospective study including all patients admitted to our hospital due to STEMI between the date of the first SARS-CoV-2 case diagnosed in Portugal and the end of the state of emergency (March and April 2020). Patient characteristics and outcomes were assessed and compared with the same period of 2019. Results A total of 104 STEMI patients were assessed, 55 in 2019 and 49 in 2020 (-11%). There were no significant differences between groups regarding age (62±12 vs. 65±14 years, p=0.308), gender (84.8% vs. 77.6% males, p=0.295) or comorbidities. In the 2020 group, there was a significant decrease in the proportion of patients transported to the hospital in pre-hospital emergency medical transportation (38.2% vs. 20.4%, p=0.038), an increase in system delay (49 [30-110.25] vs. 140 [90-180] minutes, p=0.019), a higher Killip-Kimball class, with a decrease in class I (74.5% vs. 51%) and an increase in class III (1.8% vs. 8.2%) and IV (5.5% vs. 18.4%) (p=0.038), a greater incidence of vasoactive support (3.7% vs. 26.5%, p=0.001), invasive mechanic ventilation usage (3.6% vs. 14.3%, p=0.056), and an increase in severe left ventricular dysfunction at hospital discharge (3.6% vs. 16.3%, p=0.03). In-hospital mortality was 14.3% in the 2020 group and 7.3% in the 2019 group p=0.200). Conclusion Despite a lack of significant variation in the absolute number of STEMI admissions, there was an increase in STEMI clinical severity and significantly worse outcomes during the SARS-CoV-2 pandemic. An increase in system delay, impaired pre-hospital care and patient fear of in-hospital infection can partially justify these results and should be the target of future actions in further waves of the pandemic.