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ObjectivesTo evaluate sex differences in demographic and clinical characteristics, treatments and outcomes for patients with diagnosed obstructive hypertrophic cardiomyopathy (oHCM) in the USA.SettingRetrospective observational study of administrative claims data from MarketScan Commercial Claims and Encounters Database from IBM Watson Health.ParticipantsOf the 28 million covered employees and family members in MarketScan, 9306 patients with oHCM were included in this analysis.Main outcome measuresoHCM-related outcomes included heart failure, atrial fibrillation, ventricular tachycardia/ fibrillation, sudden cardiac death, septal myectomy, alcohol septal ablation (ASA) and heart transplant.ResultsAmong 9306 patients with oHCM, the majority were male (60.5%, p

Hypertrophic cardiomyopathy is the most common genetic heart disease in the US, with an estimated prevalence of 1 in 500. However, the extent to which obstructive hypertrophic cardiomyopathy is clinically recognized is not well-established. Therefore, the objective of this study was to estimate the annual prevalence of clinically diagnosed oHCM in the US from 2016 to 2018. Data from the MarketScan® database were queried from years 2016 to 2018 to identify patients with ≥1 claim of oHCM (International Statistical Classification of Disease and Related Health Problems diagnosis code: I42.1). Prevalence rates for oHCM were calculated and stratified by sex and age. In 2016, 4,612 unique patients had clinical diagnosis of oHCM, resulting in an estimated oHCM prevalence of 1.65 per 10,000. The prevalence of oHCM in males and females was 2.07 and 1.26, respectively. Prevalence of oHCM was highest in patients 55-64 years of age (4.82). Prevalence of oHCM generally increased with age, from 0.36 per 10,000 in those under 18 to 4.82 per 10,000 in those 55-65. Trends in prevalence of oHCM over time, including by sex and age group, remained similar and consistent in 2017 and 2018. The prevalence of oHCM was stable over the 3-year time period, including higher rates of oHCM in males and patients aged 55-64 years. These results suggest that the majority of privately insured patients with oHCM are undiagnosed in the US and reinforce the need for policies and research to improve the clinical identification of oHCM patients in the US.

Emerging technology allows patients to measure and record their heart rate (HR) remotely by photoplethysmography (PPG) using smart devices like smartphones. However, the validity and expected distribution of such measurements are unclear, making it difficult for physicians to help patients interpret real-world, remote and on-demand HR measurements. Our goal was to validate HR-PPG, measured using a smartphone app, against HR-electrocardiogram (ECG) measurements and describe out-of-clinic, real-world, HR-PPG values according to age, demographics, body mass index, physical activity level, and disease. To validate the measurements, we obtained simultaneous HR-PPG and HR-ECG in 50 consecutive patients at our cardiology clinic. We then used data from participants enrolled in the Health eHeart cohort between 1 April 2014 and 30 April 2018 to derive real-world norms of HR-PPG according to demographics and medical conditions. HR-PPG and HR-ECG were highly correlated (Intraclass correlation = 0.90). A total of 66,788 Health eHeart Study participants contributed 3,144,332 HR-PPG measurements. The mean real-world HR was 79.1 bpm ± 14.5. The 95th percentile of real-world HR was ≤110 in individuals aged 18-45, ≤100 in those aged 45-60 and ≤95 bpm in individuals older than 60 years old. In multivariable linear regression, the number of medical conditions, female gender, increasing body mass index, and being Hispanic was associated with an increased HR, whereas increasing age was associated with a reduced HR. Our study provides the largest real-world norms for remotely obtained, real-world HR according to various strata and they may help physicians interpret and engage with patients presenting such data.

Background: Patients with obstructive hypertrophic cardiomyopathy (oHCM) have a substantial humanistic, clinical, and economic burden due to the array of symptoms and complications associated with the disease. The objective of this review was to identify key evidence gaps related to oHCM, specifically in Europe, North America, and Japan. Methods: A targeted literature review was conducted using PubMed to identify English-language studies published between 2012 and 2022 assessing patients with HCM/oHCM in France, Germany, Italy, Spain, the United Kingdom (UK), the United States (US), Canada, and Japan. Outcomes of interest were epidemiology, natural history, pathophysiology, management, and clinical, economic, and humanistic burden. Identified studies were assessed qualitatively to characterize evidence gaps. Results: Among 2,262 abstracts and 531 full-text articles screened, 178 articles were included from PubMed searches. An additional 16 unique studies were identified via a supplemental Google Scholar search initially conducted in January 2023 and updated in July 2024. Disease natural history, pathophysiology, and management were well documented globally. Significant evidence gaps were noted for the epidemiology, treatment, and burden of oHCM. Although multiple US studies were identified on the clinical, economic, and humanistic burden of oHCM, and one clinical burden study was found for Japan, there was a lack of evidence for France, Germany, Italy, Spain, the UK, and Canada. Conclusions: Major evidentiary gaps exist for the epidemiology, treatment, and burden of oHCM. Future research should address these gaps, with a specific focus on generating real-world evidence for Canada and European countries that will support the evaluation of emerging therapies in these regions. [ABSTRACT FROM AUTHOR]

Atrial fibrillation and atrial flutter (AF/AFL) are common arrhythmias in hypertrophic cardiomyopathy (HCM) and are associated with ongoing adverse remodeling. Septal myectomy (SM) is a commonly offered surgery in patients with symptomatic obstructive HCM. Beta blockers (BB) are frequently prescribed post-SM even in the absence of left ventricular outflow tract obstruction (LVOTO). BB have been shown to be detrimental in heart failure with preserved ejection fraction. The impact of BB on the incidence of new AF/AFL requiring treatment is unknown. This study investigated whether BB use post-SM were associated with decreased rates of new AF/AFL requiring treatment and/or intervention. Healthcare claims from the Symphony Health database between 2016-2021 were used to define a cohort of 3,532 patients with obstructive HCM with no known history of AF/AFL who underwent SM. The first chronological claim for SM was used as the index procedure and the first 30 days post SM was assigned as a blanking period. Primary endpoint was development of new AF/AFL beyond 30 days post-SM which required an intervention (cardioversion, ablation, anti-arrhythmics, anti-coagulants). Detailed BB use data were gathered using pharmacy claims and were categorized into continuous use, never use, and discontinuations. Multivariable extended Cox models adjusted for 19 variables selected a priori were constructed. During a median follow up period of 2.2 (0.6, 3.9) years, 606 (17.2%) patients developed AF/AFL and 2,637 (74.7%) patients used beta blockers at any point during the follow up period (Table 1). Patients who used BB during the follow-up period had higher rates of new AF/AFL compared to those who never used BB (unadjusted HR 2.05, 95% CI 1.67, 2.56; P<0.0001, and adjusted HR 2.03, 95% CI 1.61, 2.68; P<0.0001). Patients who discontinued BB during follow up had a trend towards lower risk of AF/AFL (unadjusted HR 1.41, 95% CI 0.99, 2.00; P=0.0528, and adjusted HR 1.43, 95% CI 1.00, 2.06; P=0.0524) (Figure 1A-B). In a sensitivity analysis of a 180-day blanking period post-SM, results were similar. In HCM patients who underwent SM, new AF/AFL requiring treatment and/or intervention occurred in 1 out of 5 patients. The use of BB post-SM was associated with a higher rate of developing AF/AFL requiring intervention. Further studies are needed to investigate this relationship given the wide use of BB in HCM. [ABSTRACT FROM AUTHOR]

OBJECTIVES: To summarize published literature on the incidence of adverse drug effects (ADEs) associated with guideline-directed medical therapy (GDMT) for patients with heart failure with reduced ejection fraction (HFrEF). STUDY DESIGN: Systematic literature review. METHODS: A systematic literature review was conducted in PubMed, Ovid MEDLINE, and Clinical Key covering January 1990 to December 2018. Key search terms were ADEs for β-blockers (BBs), ACE inhibitors (ACEis), angiotensin receptor blockers (ARBs), mineralocorticoid receptor antagonists (MRAs), and/or angiotensin receptor-neprilysin inhibitors (ARNis) in adult patients (≥ 18 years) with HFrEF. RESULTS: A total of 279 eligible articles were identified, of which 29 reported drug-related adverse effects and were included in this review. Of the 29 studies, 11 examined BBs; 9, MRAs; 6, ARNis; 2, ACEis; and 1, ARBs. The most common reported ADEs across these therapeutic classes included bradycardia, dizziness, hypotension, hyperkalemia, cough, and renal impairment. The incidence of BB-induced bradycardia was 1% to 52% based on 9 studies, and 6 studies described dizziness as a result of BBs and ARNis (15%-43%). Fourteen studies reported induced hypotension (1.4%-63%); 13 studies, hyperkalemia (0.6%-30.2%); 3 studies, cough (37%-50%); and 4 studies, renal impairment (0.6%-7.6%). CONCLUSIONS: Findings show that drug-related adverse effects are commonly reported in clinical trials and highlight the sizable burden of ADEs with medical therapy across patients with HFrEF. Additional real-world evidence and studies aiming to improve the tolerability of GDMT for patients with HFrEF are warranted. [ABSTRACT FROM AUTHOR]

Background: An estimated two-thirds of heart failure (HF) patients with reduced ejection fraction (HFrEF) hospitalized in the United States have a severely reduced left ventricular ejection fraction (LVEF < 30%). Few studies have categorized patients according to their severity of left ventricular dysfunction beyond an LVEF of < 30%., Methods: Intermountain Health patients (≥18 years), with a primary HF diagnosis, ≥1 inpatient hospitalization with a primary discharge diagnosis of HF, a documented LVEF of < 30%, and a BNP > 100 pg/mL within one year of hospitalization were studied. Patients were stratified by LVEF levels (≤15%, 16-25%, and 26-29%) and evaluated for death, HF hospitalization, healthcare resource utilization, and medical costs., Results: Overall, 2 184 patients (mean age 64.2 ± 15.5 years, 72.5% male) were stratified by LVEF (≤15%, n = 468 [21.4%]; 16-25%, n = 1399 [64.1%]; and 26-29%, n = 317 [14.5%]). Lower LVEF was associated with younger age, male sex, and fewer comorbidities. Although one-year mortality differed significantly between LVEF stratifications, which remained after adjustment by risk factors (vs. LVEF 26-29% [referent]): ≤15%, hazard ratio (HR)=1.92, p < 0.0001; and 16-25%, HR = 1.42, p = 0.01), mortality was similar by 3-years. HF hospitalizations at 1- and 3-years were similar among LVEF groups. Total HF costs-driven by increased HF outpatient costs-were significantly higher among LVEF of ≤ 15%., Conclusions: Patients with an LVEF of ≤ 15% had a modestly increased risk of 1-year mortality, as well as significantly higher total HF costs. Patients with HFrEF and a severely reduced LVEF continue to face an increased clinicoeconomic burden, and novel therapies to treat this unmet medical need are warranted., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Society of Cardiology.)

Objective: Hypertrophic cardiomyopathy (HCM), including obstructive HCM (oHCM), is the most common inherited cardiomyopathy causing lifestyle-limiting symptoms. Data are lacking about patients' perspectives on the daily impact of their symptoms. This qualitative interview study was conducted to better understand patients' experiences with oHCM., Methods: In October 2019, telephone interviews were conducted with 20 US adults with oHCM identified by the Hypertrophic Cardiomyopathy Association. Using a semi-structured interview guide, key symptoms, impacts of oHCM and oHCM treatment goals were discussed., Results: Median age was 54 years (range 29-78), 55% were women, 85% were white and 15% were Hispanic or Latino. Median time since diagnosis was 3 years. Symptoms included shortness of breath, dizziness/light-headedness, heart palpitations/fluttering (all 95%), fatigue (90%) and chest pain/pressure (80%). All participants reported limitations in physical functioning/activities; most reported additional impacts (emotional stress (80%), fear of dying (55%)). Shortness of breath and fatigue were among their most bothersome symptoms; an effective oHCM treatment would need to improve ≥1 of these symptoms (allowing increased physical/social activity)., Conclusions: Patients with oHCM experience a high symptom burden and psychosocial impacts, affecting health status. Improved shortness of breath, fatigue and physical functioning are highly valued by patients and represent important treatment goals., Competing Interests: Competing interests: SSh has received a grant from the American Heart Association (grant ID 855105). CE, KK and SF are employees of RTI Health Solutions. LS has no conflicts of interest to declare. MB, SBH and DJ are employees of Cytokinetics, Incorporated, and hold stock in Cytokinetics, Incorporated. SSa has received consultant/adviser fees from Bristol Myers Squibb and research grants from Bristol Myers Squibb, Cytokinetics, Novartis and Actelion Pharmaceuticals., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Aims: Patients with obstructive hypertrophic cardiomyopathy (oHCM) experience significant clinical burden which is associated with a high economic burden. Peak oxygen uptake (pVO2), measured by cardiopulmonary exercise testing, is used to quantify functional capacity, and has been studied as a primary endpoint in recent clinical trials. This study aimed to gather evidence to consolidate the prognostic value of pVO2 in oHCM and to assess whether it is feasible to predict health outcomes in an economic model based on changes in pVO2., Methods: A targeted literature review was conducted in MEDLINE ( via PubMed) and Embase databases to identify evidence on the prognostic value of pVO2 as a surrogate health outcome to support future oHCM economic model development. Following screening, study characteristics, population characteristics, and pVO2 prognostic association data were extracted., Results: A total of 4,687 studies were identified. In total, 3,531 and 538 studies underwent title/abstract and full-text screening, respectively, of which 151 were included and nine of these were in hypertrophic cardiomyopathy (HCM); only three studies focused on oHCM. The nine HCM studies consisted of one systematic literature review and eight primary studies reporting on 27 potentially predictive relationships from a pVO2-based metric with clinical outcomes including all-cause mortality, cardiovascular mortality, sudden cardiac death, transplant, paroxysmal, and permanent atrial fibrillation. pVO2 was described as a predictor of single and composite endpoints, in three and six studies, respectively, with one study reporting on both., Limitations: This study primarily uses systemic literature review methods but does not qualify as one due to not entailing parallel reviewers during title-abstract and full-text stages of review., Conclusion: The findings of this study suggest pVO2 is predictive of multiple health outcomes, providing a rationale to use pVO2 in the development of an economic model.

Background: Patients with obstructive hypertrophic cardiomyopathy (oHCM) and severe refractory symptoms may require invasive septal reduction therapies (SRTs), either surgical septal myectomy (SM) or transcatheter alcohol septal ablation (ASA). The main objective of this study was to quantify all-cause and oHCM-related healthcare resource utilization (HCRU) and costs for patients receiving SM or ASA., Methods: This retrospective study utilized medical and pharmacy claims submitted during 2012-2020. HCRU and costs for 119 adults with oHCM who had at least 1 SM (n = 95) or ASA (n = 24) were compared for baseline and follow-up periods., Results: The mean inpatient hospitalization stay was longer for SM (8.3 days) than ASA (6.0 days). Postprocedure HCM-related medication usage was greater following SM (98%) than ASA (88%). The mean number of HCM-related outpatient visits increased from pre- to post procedure (12.2 vs 15.9 in the SM group; 7.2 vs 9.5 in the ASA group), with most patients having at least 1 cardiology visit post procedure (86% of the SM group; 83% of the ASA group). Total mean HCM-related costs (reported in United States currency) increased with both procedures ($27,045 vs $119,772 in the SM group; $11,278 vs $54,351 in the ASA group), driven by increased inpatient hospitalization ($10,325 vs $112,923 in the SM group; $5509 vs $47,450 in the ASA group) and surgical costs ($6665 vs $92,031 in the SM group; $52 vs $44,815 in the ASA group)., Conclusions: Our results indicate increasing costs for patients undergoing SRT, driven by inpatient hospitalizations and surgical costs. Commercially insured and Medicare Advantage patients with oHCM experience high healthcare costs and economic burden attributable to SRT.