Background: The p.Val142Ile variant, predominantly found among people of African descent, is the most common cause of variant transthyretin amyloidosis and carriers predominantly develop a cardiomyopathy (variant transthyretin amyloidosis cardiomyopathy) phenotype. Yet, there are conflicting data on the prevalence and outcomes of p.Val142Ile variant carriers. Methods: We performed a systematic review of the prevalence and outcomes of p.Val142Ile variant transthyretin amyloidosis cardiomyopathy among subjects of African descent. We found 62 relevant articles after searching the MEDLINE databases from 1980 to 2020 that reported data for ≈150 000 subjects. Results: The reported worldwide prevalence of the p.Val142Ile variant is 0.3% to 1.6% in the general population. Among people of African descent, the reported prevalence from all studies ranges from 1.1% to 9.8%, but for studies with >1000 subjects, it is 3% to 3.5%. The prevalence of the p.Val142Ile variant in a region is dependent on the reported percentage of subjects who are of African descent in that region. p.Val142Ile variant transthyretin amyloidosis cardiomyopathy typically presents in the seventh to eighth decade of life and the majority of cases reported were male, with 25% to 38% diagnosed with atrial fibrillation. It was associated with a longitudinally worse quality of life and a lower adjusted survival compared with other types of transthyretin amyloidosis cardiomyopathy. Conclusion: The p.Val142Ile variant is the most common variant of the transthyretin gene with most carriers being of African descent. The true penetrance is unknown but the p.Val142Ile variant is associated with increased rates of incident heart failure and portends a lower overall survival. Increased awareness could lead to earlier diagnosis and improved heart failure outcomes among those of African descent, which is of increasing importance given the advent of novel therapeutics for this disease.

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Aims Amiodarone reduces the incidence of atrial fibrillation (AF) following coronary artery bypass surgery; however, the benefit of perioperative amiodarone in patients undergoing septal myectomy (SM) for obstructive hypertrophic cardiomyopathy (oHCM) has not been studied. We hypothesized that prophylactic amiodarone would reduce the incidence of postoperative AF (POAF) following SM for oHCM. Methods and Results A single‐centre, pre‐post intervention open‐label study of oral amiodarone (200 mg twice daily starting 7 days preoperatively and 200 mg once daily continuing for 30 days postoperatively) in patients without prior AF undergoing SM for oHCM from 2014 to 2018. The primary outcome was incident AF within 30 days. Secondary outcomes were unplanned readmission, AF treatment, total and intensive care unit (ICU) length of stay (LOS), and pacemaker implantation for high‐grade atrioventricular (AV) block. 61 patients met inclusion criteria with 34 (55.8%) in the pre‐intervention (control) group and 27 (44.2%) in the post‐intervention (amiodarone) group. The incidence of POAF was 11.0% in the amiodarone group compared with 38.2% in the control group (P = 0.017). After adjusting for age, amiodarone was associated with less POAF [adjusted odds ratio (aOR) 0.21; 95% confidence interval (CI) 0.05, 0.76; P = 0.016]. ICU (2 days [IQR 1, 4] vs. 3 days [IQR 2, 4]; P = 0.165) and total (6 days [IQR 5, 6] vs. 6 days [IQR 5, 7]; P = 0.165) LOS were similar, as was the rate of pacemaker implantation (7.4% vs. 8.3%, P > 0.999). There were no adverse events associated with amiodarone. Conclusions Perioperative oral amiodarone is safe and was associated with lower incidence of POAF following SM for oHCM.