Hirak Shah

Importance Mavacamten is highly effective in treating symptomatic obstructive hypertrophic cardiomyopathy (oHCM) and was approved for commercial use with a risk mitigation program (REMS) to monitor the impact on left ventricular systolic function (LVSD). The impact of mavacamten on atrial fibrillation (AF) occurrence is not well characterized. Objective Determine the real-world incidence of new-onset and recurrent AF among patients with HCM treated with commercial mavacamten. Secondary objectives included assessing the incidences of LVSD, heart failure (HF), and cardiogenic shock. Design A multicenter cohort study. Center-level data were aggregated for patients who received mavacamten from May 2022 through December 2024. Setting Twenty-one outpatient HCM centers in the United States. Participants Consecutive patients ≥18 years of age with oHCM were included; those with permanent AF were excluded. Exposures At least one dose of commercial mavacamten. Main Outcomes and Measures Incidence of new-onset and recurrent AF, LVSD, HF, and cardiogenic shock. Results Among 1,538 patients (median age, 66 years [95% CI, 65.9–66.1]; 57% female [95% CI, 54–59%]), 25% [95% CI, 22-27%] had prior AF. Median mavacamten exposure was 13.4 months [95% CI, 11.8–15.0]. Overall AF incidence was 13% [95% CI, 10–17%], including 5% [95% CI, 4–7%] new-onset and 39% [95% CI, 27–51%] recurrent AF in those with history of AF prior to mavacamten initiation. LVEF Conclusions and Relevance In this multicenter cohort receiving commercial mavacamten, we identified an annual incidence of 5% new-onset AF and 39% recurrent AF, while 70% of patients who developed LVEFQuestion What are the real-world burden and consequences of atrial fibrillation (AF) in patients with hypertrophic cardiomyopathy treated with commercial mavacamten? Findings In a multicenter cohort of more than 1,500 patients, 5% experienced new-onset AF, while 39% of those with pre-existing AF experienced recurrence after mavacamten initiation. Left ventricular ejection fraction (LVEF) fell below 50% in 8% of patients, 70% of whom had concurrent AF. Meaning Real-world experience with mavacamten emphasizes that regular surveillance for AF is an important aspect of managing patients with HCM. Additionally, the identification of AF should prompt LVEF assessment and aggressive rhythm management, given the potential association between AF and decrease in LVEF. The observed incidence of AF on mavacamten highlights the need for further studies on potential mechanisms and for proactive surveillance and management strategies.

We performed a retrospective review comparing outcomes between traditional ice storage (ICE) with short ischemic times (4 hours) using data from the GUARDIAN registry, a retrospective observational trial. To minimize baseline differences, propensity-matched (PSM) cohorts for site and era were performed. SherpaPak Cardiac Transport System travel distance was almost 10-fold greater than ICE (82 miles ICE vs. 765 miles SCTS). There was no significant difference in primary graft dysfunction (PGD) (20.8% vs. 18.2%, p = 0.58), length of stay (LOS) (24.7 vs. 24.8, p = 0.98), posttransplant mechanical circulatory support (MCS) (25.1% vs. 20.3%, p = 0.34), and 30 day survival (100% vs. 98.6%, p = 0.20). SherpaPak Cardiac Transport System showed statistically significant reduction in 24 hour inotrope scores (17.6 vs. 13.6, p = 0.007) and right ventricular (RV) dysfunction (31.1% vs. 15.7%, p = 0.002). Propensity-matched cohorts showed statistically similar rates of MCS utilization and PGD, but SCTS trended toward less RV dysfunction (26.0% vs. 16.2%, p = 0.11) and lower inotrope scores (16.5 vs. 12.9, p = 0.06) despite almost double the ischemic time. In conclusion, donor heart preservation with SCTS continues to be effective in prolonged ischemic times without sacrificing postheart transplantation clinical outcomes. This may aid in expanding donor organ geography.

Background: There is a need for simple, noninvasive solutions to remotely monitor and predict worsening heart failure (HF) events. SCALE-HF 1 (Surveillance and Alert-Based Multiparameter Monitoring to Reduce Worsening Heart Failure Events) is a prospective, multicenter study that will develop and assess the accuracy of the heart function index—a composite algorithm of noninvasive hemodynamic biomarkers from a cardiac scale—in predicting worsening HF events. Methods: Approximately 300 patients with chronic HF and recent decompensation will be enrolled in this observational study for model development. Patients will be encouraged to take daily cardiac scale measurements. Results: Approximately 50 HF events, defined as an urgent, unscheduled clinic, emergency department, or hospitalization for worsening HF will be used for model development. The composite index will be developed from hemodynamic biomarkers derived from ECG, ballistocardiogram, and impedance plethysmogram signals measured from the cardiac scale. Biomarkers of interest include weight, peripheral impedance, pulse rate and variability, and estimates of stroke volume, cardiac output, and blood pressure captured through the cardiac scale. The sensitivity, unexplained alert rate, and alerting time of the index in predicting worsening HF events will be evaluated and compared with the performance of simple weight-based rule-of-thumb algorithms (eg, weight increase of 3 lbs in 1 day or 5 lbs in 7 days) that are often used in practice. Conclusions: SCALE-HF 1 is the first study to develop and evaluate the performance of a composite index derived from noninvasive hemodynamic biomarkers measured from a cardiac scale in predicting worsening HF events. Subsequent studies will validate the heart function index and assess its ability to improve patient outcomes. Registration: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT04882449.

Cardiac transplantation requires the careful allocation of a limited number of precious organs. Therefore, it is critical to select candidates that will receive the greatest anticipated medical benefit but will also serve as the best stewards of the organ. Individual transplant teams have established prerequisites pertaining to recreational drug, tobacco, alcohol, and controlled substance use in potential organ recipients and post-transplantation. Legalization of cannabis and implementation of its prescription-based use for the management of patients with chronic conditions have been increasing over the past years. Center requirements regarding abstinence from recreational and medical cannabis use vary due to rapidly changing state regulations, as well as the lack of clinical safety data in this population. This is evident by the results of the multicenter survey presented in this paper. Developing uniform guidelines around cannabis use will be imperative not only for providers but also for patients.