An evidence review and gap analysis for obstructive hypertrophic cardiomyopathy.

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.

Telehealth Interventions and Outcomes Across Rural Communities in the United States: Narrative Review.

BACKGROUND: In rural communities, there are gaps in describing the design and effectiveness of technology interventions for treating diseases and addressing determinants of health. OBJECTIVE: The aim of this study is to evaluate literature on current applications, therapeutic areas, and outcomes of telehealth interventions in rural communities in the United States. METHODS: A narrative review of studies published on PubMed from January 2017 to December 2020 was conducted. Key search terms included telehealth, telemedicine, rural, and outcomes. RESULTS: Among 15 included studies, 9 studies analyzed telehealth interventions in patients, 3 in health care professionals, and 3 in both patients and health care professionals. The included studies reported positive outcomes and experiences of telehealth use in rural populations including acceptability and increased satisfaction; they also noted that technology is convenient and efficient. Other notable benefits included decreased direct and indirect costs to the patient (travel cost and time) and health care service provider (staffing), lower onsite health care resource utilization, improved physician recruitment and retention, improved access to care, and increased education and training of patients and health care professionals. CONCLUSIONS: Telehealth models were associated with positive outcomes for patients and health care professionals, suggesting these models are feasible and can be effective. Future telehealth interventions and studies examining these programs are warranted, especially in rural communities, and future research should evaluate the impact of increased telehealth use as a result of the COVID-19 pandemic.

Differences in Healthcare Resource Use and Cost by Pharmacotherapy Among Patients with Symptomatic Obstructive Hypertrophic Cardiomyopathy: Real-World Analysis of Claims Data.

BACKGROUND: For symptomatic obstructive hypertrophic cardiomyopathy (oHCM), limited evidence exists on healthcare resource utilization (HRU) and cost for patients with symptomatic oHCM by treatment categories. We evaluated whether HRU and costs vary by initial treatment in symptomatic oHCM. METHODS: This is a retrospective study of medical and pharmacy claims from 2016 to 2021 to identify (per International Classification of Disease Tenth Revision diagnosis codes) adult patients in the USA with symptomatic oHCM. Patients included in the study cohort were required to be treatment naïve (≥ 12 months' activity before first treatment) and symptomatic (fatigue, chest pain, syncope, dyspnea, heart failure, or palpitations within 3 months of index date). Patients were grouped by first index treatment [beta blocker (BB), calcium channel blockers (CCB), disopyramide, combination therapy], and HRU and costs [per person per year (PPPY), in USD] by initial treatment were reported. RESULTS: Among 7334 patients with symptomatic oHCM, initial treatment included BB (65.8%), CCB (21.1%), disopyramide (1.2%), or BB + CCB (11.9%). Overall, 87.2% were prescribed monotherapy. Outpatient visits were the main driver of all-cause HRU (mean 11.5 PPPY), and varied by initial treatment (BB: 11.0, CCB: 10.5, disopyramide: 7.2, combination therapy: 12.1). All-cause urgent care visits were more frequent than inpatient visits (means: 5.4 and 0.83 PPPY, respectively). All-cause incurred costs were $46,628 PPPY overall and varied by treatment (BB: $47,029, CCB: $42,124, disopyramide: $27,007, combination therapy: $54,024). CONCLUSIONS: In this large, US-based cohort of patients with symptomatic oHCM, initial therapy was most commonly BB and CCB monotherapy. Costs and HRU were high for most patients, but greater for those treated initially with combination therapy.

Clinicoeconomic burden among heart failure patients with severely reduced ejection fraction after hospital admission: HF-RESTORE.

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 prognostic value of peak oxygen uptake in obstructive hypertrophic cardiomyopathy: a literature review to inform economic model development.

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.

Obstructive hypertrophy cardiomyopathy (oHCM) is a condition where the heart muscle thickens, obstructing blood flow and potentially impacting health. Peak oxygen uptake (pVO2) measures the highest amount of oxygen consumption during peak exercise and serves as an indicator of fitness. pVO2 can be used to assess heart health and predict severe conditions and death, acting as a surrogate endpoint. Surrogate endpoints are valuable in drug investigations since they allow earlier decisions on drug approval and funding before longer-term patient follow-up is available.This study reviewed evidence on the relationship between pVO2 values in patients with heart disease and the risk of becoming sicker or dying. Our goal was to assess if these relationships had been established and whether it is feasible to use them to predict future treatment benefits and support economic evaluations of new treatments. Our review found that most studies reported on patients with heart failure, with only nine focusing on HCM. Evidence indicates that low pVO2 values in patients with heart disease are linked to an increased risk of developing other heart conditions, needing a heart transplant, or dying.

Qualitative interview study of patient-reported symptoms, impacts and treatment goals of patients with obstructive hypertrophic cardiomyopathy.

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.

Effect of Aficamten on Health Status Outcomes in Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA-HCM.

BACKGROUND: A primary goal in treating obstructive hypertrophic cardiomyopathy (oHCM) is to improve patients' health status: their symptoms, function, and quality of life. The health status benefits of aficamten, a novel cardiac myosin inhibitor, have not been comprehensively described. OBJECTIVES: This study sought to determine the effect of aficamten on patient-reported health status, including symptoms of fatigue, shortness of breath, chest pain, physical and social limitations, and quality of life. METHODS: SEQUOIA-HCM (Phase 3 Trial to Evaluate the Efficacy and Safety of Aficamten Compared to Placebo in Adults With Symptomatic oHCM) randomized symptomatic adults with oHCM to 24 weeks of aficamten (n = 142) or placebo (n = 140), followed by a 4-week washout. The Kansas City Cardiomyopathy Questionnaire (KCCQ) and Seattle Angina Questionnaire 7-item (SAQ7) were serially administered. Changes in mean KCCQ-Overall Summary Score (KCCQ-OSS) and SAQ7-Summary Score (SAQ7-SS) from baseline to 24 weeks and following treatment withdrawal were compared using linear regression adjusted for baseline scores and randomization strata. Proportions of patients with clinically important changes were compared. RESULTS: Among 282 participants, the mean age was 59 ± 13 years, 115 (41%) were female, and 223 (79%) were White. Baseline KCCQ-OSS (69.3 ± 20.1 vs 67.3 ± 18.8) and SAQ7-SS (72.0 ± 21.0 vs 72.4 ± 18.3) were similar between aficamten and placebo groups. Treatment with aficamten, compared with placebo, improved both the mean KCCQ-OSS (13.3 ± 16.3 vs 6.1 ± 12.6; mean difference: 7.9; 95% CI: 4.8-11.0; P < 0.001) and SAQ7-SS (11.6 ± 17.4 vs 3.8 ± 14.4; mean difference: 7.8; 95% CI: 4.7-11.0; P < 0.001) at 24 weeks, with benefits emerging within 4 weeks. No heterogeneity in treatment effect was found across subgroups. A much larger proportion of participants experienced a very large health status improvement (≥20 points) with aficamten vs placebo (KCCQ-OSS: 29.7% vs 12.4%, number needed to treat: 5.8; SAQ7-SS: 31.2% vs 13.9%, number needed to treat: 5.8). Participants' health status worsened significantly more after withdrawal from aficamten than placebo (KCCQ-OSS: -16.2 ± 19.0 vs -3.0 ± 9.6; P < 0.001; SAQ7-SS: -17.4 ± 21.4 vs -2.5 ± 13.3), further confirming a causal effect of aficamten. CONCLUSIONS: In patients with symptomatic oHCM, treatment with aficamten resulted in markedly improved health status, including significant improvement in chest pain-related health status, than placebo. (Phase 3 Trial to Evaluate the Efficacy and Safety of Aficamten Compared to Placebo in Adults With Symptomatic oHCM [SEQUOIA-HCM]; NCT05186818).

Impact of Health Literacy on Medication Adherence Among Black Medicaid Beneficiaries with Hypertension in Delaware: A Cross-Sectional Study.

Racial and ethnic minorities are disproportionately affected by limited health literacy. Therefore, this study assessed census block health literacy level and medication adherence in Delaware among Black individuals with hypertension (HTN) receiving health care through Medicaid. This was a cross-sectional study of Black Delaware Medicaid beneficiaries (18-64 years old) from the 3 counties in Delaware (Kent, New Castle, and Sussex) from 2016 to 2019. The primary outcome was medication adherence (full adherence = 80%-100%, partial adherence = 50%-79%, and nonadherence = 0-49%) as a function of health literacy. Health literacy scores were categorized as below basic (0-184), basic (184-225), intermediate (226-309), and proficient (310-500). The results of the study showed that 18,958 participants (29%) had ≥1 HTN diagnosis during the study period. Mean area health literacy score for participants without HTN was significantly higher than participants with HTN (234.9 vs. 233.7, P < 0.0001). Men had lower odds of adherence compared with women (odds ratio [OR]: 0.83, 95% confidence interval [CI]: 0.75-0.92, P < 0.001). Increased time enrolled in Medicaid decreased full adherence. Participants 21-30 and 31-50 years of age are significantly less likely to have full adherence in comparison with participants 51-64 years of age (P < 0.0001). Participants living in an area with basic level of health literacy reported lower medication adherence than those living in an area with an intermediate level of health literacy (OR: 0.72, 95% CI: 0.64-0.81, P < 0.001). In conclusion, men, younger adults, increased time enrolled in Medicaid for the study period, and basic health literacy were significantly associated with low adherence to medication among 3 census blocks in Delaware.

Health utilities and quality-adjusted life years for patients with amyotrophic lateral sclerosis receiving reldesemtiv or placebo in FORTITUDE-ALS.

AIMS: To estimate the health utilities and quality-adjusted life years (QALYs) in patients with amyotrophic lateral sclerosis (ALS) receiving reldesemtiv versus placebo in FORTITUDE-ALS. MATERIALS AND METHODS: We performed a post hoc analysis of clinical trial data from FORTITUDE-ALS (NCT03160898). This Phase IIb, double-blind, randomized, dose-ranging, placebo-controlled, parallel-group, 12-week trial evaluated reldesemtiv in patients with ALS. Health utilities from the five-level version of the EuroQol five-dimensional questionnaire (EQ-5D-5L) were estimated using ALS Functional Rating Scale-Revised (ALSFRS-R) scores collected during the trial. QALYs were estimated using the area under the curve method. RESULTS: The full analysis set consisted of 456 patients (reldesemtiv n = 342, placebo n = 114), who received at least one dose of the double-blind study drug, and had ALSFRS-R assessed at baseline and at least one post-baseline assessment. The difference in EQ-5D-5L utility least-squares (LS) mean change from baseline to week 12 for reldesemtiv versus placebo, adjusted for baseline values, was statistically significant (0.03, 95% confidence interval [CI]: 0.01, 0.05; p = .0008). The incremental QALY of reldesemtiv versus placebo adjusted for baseline utility values showed a modest, but statistically significant, difference (0.004, 95% CI: 0.001, 0.007; p = .0058). CONCLUSIONS: This post hoc analysis of FORTITUDE-ALS suggests that reldesemtiv showed a modest but significant benefit in health utilities and QALYs compared with placebo. Future long-term studies that include direct collection of EQ-5D-5L data will be needed to confirm our findings. CLINICALTRIALS.GOV IDENTIFIER: NCT03160898.

Adverse drug effects across patients with heart failure: a systematic review.

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 ß-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.

Clinical Characteristics and Healthcare Resource Utilization among Patients with Obstructive Hypertrophic Cardiomyopathy Treated in a Range of Settings in the United States.

Obstructive hypertrophic cardiomyopathy (oHCM) has been studied primarily in comprehensive centers of excellence. Broadening the understanding of patients with oHCM in the general population may improve identification and treatment in other settings. This retrospective cohort study identified adults with oHCM from a large electronic medical record database comprising data from 39 integrated delivery networks (IBM Explorys; observational period: January 2009-July 2019). Clinical characteristics, healthcare resource utilization (HCRU), and outcomes were reported. Of 8791 patients, 53.0% were female and the mean index age was 61.8 years. Cardiovascular drugs prescribed included beta-blockers (80.5%), calcium channel blockers (46.0%), and disopyramide (2.4%). Over time, heart failure, atrial fibrillation, and ventricular arrhythmias increased. Surgical procedures included septal myectomy (22.0%), alcohol septal ablation (0.6%), and heart transplantation (0.3%). Implantable cardioverter defibrillators were present in 11.2% of patients. After initial septal reduction therapy (SRT), HCRU increased and 550 patients (27.7%) required a reintervention. Of the overall group, 2.7% experienced sudden cardiac arrest by end of study. In conclusion, this cohort of patients with oHCM had guideline-recommended drug therapy and procedures. Despite this, heart failure, atrial fibrillation, and ventricular arrhythmias increased, and more than a quarter of patients undergoing SRT required reintervention. These unresolved issues emphasize the unmet need for new, effective therapies for patients with oHCM.

Sex differences in clinical outcomes for obstructive hypertrophic cardiomyopathy in the USA: a retrospective observational study of administrative claims data.

OBJECTIVES: To evaluate sex differences in demographic and clinical characteristics, treatments and outcomes for patients with diagnosed obstructive hypertrophic cardiomyopathy (oHCM) in the USA. SETTING: Retrospective observational study of administrative claims data from MarketScan Commercial Claims and Encounters Database from IBM Watson Health. PARTICIPANTS: Of the 28 million covered employees and family members in MarketScan, 9306 patients with oHCM were included in this analysis. MAIN OUTCOME MEASURES: oHCM-related outcomes included heart failure, atrial fibrillation, ventricular tachycardia/ fibrillation, sudden cardiac death, septal myectomy, alcohol septal ablation (ASA) and heart transplant. RESULTS: Among 9306 patients with oHCM, the majority were male (60.5%, p<0.001) and women were of comparable age to men (50±15 vs 49±15 years, p<0.001). Women were less likely to be prescribed beta blockers (42.7% vs 45.2%, p=0.017) and undergo an implantable cardioverter-defibrillator (1.7% vs 2.6%, p=0.005). Septal reduction therapy was performed slightly more frequently in women (ASA: 0.08% vs 0.05%, p=0.600; SM: 0.35% vs 0.18%, p=0.096), although not statistically significant. Women were less likely to have atrial fibrillation (6.7% vs 9.9%, p<0.001). CONCLUSION: Women were less likely to be prescribed beta blockers, ACE inhibitors, anticoagulants, undergo implantable cardioverter-defibrillator and have ventricular tachycardia/fibrillation. Men were more likely to have atrial fibrillation. Future research using large, clinical real-world data are warranted to understand the root cause of these potential treatment disparities in women with oHCM.

Healthcare resource utilization and cost of obstructive hypertrophic cardiomyopathy in a US population.

Background: There are limited data evaluating all-cause and disease-related healthcare resource utilization (HCRU) and cost of care for patients with obstructive hypertrophic cardiomyopathy (oHCM). Methods: This was a retrospective study using US longitudinal medical and pharmacy claims data during 2012-2020. Adults with ≥2 oHCM diagnoses were identified, with the first diagnosis date used as the index date. HCRU and costs of care were reported for the year preindex (baseline) and at 1- and 2-year follow-ups. Results: We identified 1841 patients with oHCM (63 ± 15 years; 52% male). The mean number of hypertrophic cardiomyopathy (HCM)-related outpatient and cardiology visits increased from baseline to 1-year follow-up (2.3 vs. 7.8 and 0.6 vs. 2.2, respectively). At baseline, 8% of patients had ≥1 HCM-related inpatient hospitalization (mean 0.11 visits, 5.4 days length of stay), increasing to 27% postdiagnosis (mean 0.42 visits, 5.9 days length of stay). Total HCM-related costs increased from $5968 to $20,290 at 1-year follow-up, largely driven by inpatient hospitalization costs ($3889 vs. $14,369) and surgical costs ($2259 vs. $7217). The proportion with ≥1 HCM-related prescription increased from baseline (69%; mean fills 5.3) to 1-year follow-up (82%; mean fills 7.8). Pharmacy costs were generally low but also increased ($449 vs. $752). Conclusions: This benchmark economic dataset for management and evaluation of patients with oHCM shows increased HCM-related costs over a 2-year period after oHCM diagnosis, driven by inpatient hospitalizations and surgical costs. Medication use was high, but costs were low, possibly reflecting use of generic multi-indication drugs for oHCM treatment.

Characteristics of Patients With Obstructive Hypertrophic Cardiomyopathy in Real-World Community-Based Cardiovascular Practices.

The clinical profile of patients with obstructive hypertrophic cardiomyopathy (oHC) is not well characterized, with little evidence outside selected referral populations. Using longitudinal medical claims data from a United States nationwide database, we retrospectively identified adults who were newly diagnosed with oHC. Clinical characteristics were compared from 1 year before diagnosis and at the 2-year follow-up. Patients (N = 1,841) with oHC (age 63 ± 15 years; 52% were male) with geographic representation across the United States were identified. Most patients received care within community-based cardiovascular practices and 7% at referral hypertrophic cardiomyopathy (HC) centers. Baseline diagnostic procedures included electrocardiogram (66%), echocardiogram (51%), magnetic resonance imaging (4%), and HC genetic testing (0.7%). Baseline co-morbidities were hypertension (59%), coronary artery disease (30%), diabetes (19%), and atrial fibrillation (19%). For all HC-related medications, use significantly increased after diagnosis. During follow-up, 144 patients (8%) received an implantable cardioverter-defibrillator for sudden death prevention, 99 underwent septal myectomy (5%), and 24 underwent alcohol septal ablation (1%). By the 1-year follow-up, 2% of patients had sudden cardiac arrest and 26% had atrial fibrillation, and heart failure increased from 16% to 27%. In conclusion, in a community-based population of patients with oHC, patients' age at diagnosis of oHC was older than reported for referral populations and patients had a significant co-morbidity burden. Cardiovascular medication use was appropriate, but the rate of guideline-supported surgical procedures was low.

Costs and Healthcare Resource Utilization for Obstructive Hypertrophic Cardiomyopathy With Septal Reduction Therapy.

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.

Stable Rates of Obstructive Hypertrophic Cardiomyopathy in a Contemporary Era.

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.

Examining medication adherence and preferences for a lifestyle intervention among Black and Latinx adults with hypertension: a feasibility study.

BACKGROUND: Approximately 116.4 million adults in the USA have hypertension, and the rates of uncontrolled hypertension remain higher among racial and ethnic minorities. There is a need for effective interventions that promote healthy behaviors and long-term behavioral change in the management of hypertension. The primary objective of this study was to determine the feasibility of developing a lifestyle intervention that would assess hypertension management and the use of technology among Blacks and Latinx with hypertension. The secondary objective is to explore perceptions of community-based resources for hypertension and preferences for a lifestyle intervention for hypertension among Blacks and Latinx with hypertension. METHODS: In this explanatory mixed-methods study, quantitative data were collected using surveys, participants reported their use of technology and adherence to antihypertensive medication. Participants were Black and Latinx adults with hypertension living in Central Pennsylvania, USA. Qualitative data were obtained from semi-structured interviews and focus groups, and participants were asked about managing hypertension, local resources, and preferences for a behavioral intervention. Data were examined using summary statistics for quantitative data and thematic analysis for qualitative data. RESULTS: Black and Latinx participants (n=30) completed surveys for the quantitative study. The majority (75%) of participants self-reported being confident in managing their medication without help and remembering to take their medication as prescribed. Fewer participants (54.2%) reported using technology to help manage medication. There were 12 participants in the qualitative phase of the study. The qualitative findings indicated that participants felt confident in their ability to manage hypertension and were interested in participating in a lifestyle intervention or program based online. Some participants reported a lack of resources in their community, while others highlighted local and national resources that were helpful in managing high blood pressure. CONCLUSION: This study provides important insights on barriers and facilitators for managing hypertension, current use of technology and interest in using technology to manage hypertension, and preferences for future lifestyle interventions among racial and ethnic minorities. This study also provides insights to the health needs and resources available in this community and how future behavioral interventions could be tailored to meet the needs of this community. The findings of this study will be used to inform the tailoring of future lifestyle interventions; specifically, we will include text messaging reminders for medication and to disseminate educational materials related to hypertension and provide resources to connect study participants with local and national resources.

Clinical Diagnosis of Hypertrophic Cardiomyopathy Over Time in the United States (A Population-Based Claims Analysis).

Hypertrophic cardiomyopathy (HC) is a common genetic heart disease. However, the number of gene mutation carriers who develop HC and manifest clinical symptoms is not well established. Our objective was to estimate annual prevalence and incidence rates of clinically diagnosed HC in the United States. Data from the HealthCore Integrated Research Database (HIRD) were interrogated for years 2013-2019 to identify patients with ≥1 claim of HC International Classification of Diseases, Clinical Modification Ninth and Tenth Revision diagnosis codes. In 2013, among 16,243,109 patients, 8,526 were identified with HC, yielding an estimated prevalence of clinically diagnosed HC of 0.052% (0.035% for obstructive [oHC], 0.017% for nonobstructive [nHC]). This prevalence yielded an estimated 164,403 patients with clinical diagnosis of HC. For the same year, the incidence of new HC diagnoses was 0.030% (0.020% for oHC, 0.010% for nHC). Over the following 6 years, prevalence and incidence of HC increased by 0.005%/year (p <0.01) and 0.001%/year (p <0.01), respectively, with an estimated 262,591 patients with a clinical diagnosis of HC in 2019. Over this period, incidence of nHC increased (0.012% vs 0.026%, p <0.01), whereas incidence of oHC decreased (0.020% versus 0.015%, p <0.01). In conclusion, over 6 years, the number of patients with clinically diagnosed HC in the United States increased 1.5-fold to ∼262,591, primarily because of a rise in nHC diagnoses. These prevalence data support further investigation to better understand factors accounting for increasing clinical recognition of HC.

Real-world heart rate norms in the Health eHeart study.

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.