Journal of the American College of Cardiology最新文献

Aim: The "2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease" provides recommendations to guide clinicians on the evaluation and treatment of adult patients with congenital heart disease. It incorporates new evidence to replace the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease."

Methods: A comprehensive literature search was conducted with a focus on literature published from 2017 to 2024; in some instances, older literature was also collected and reviewed. Clinical studies, systematic reviews and meta-analyses, and other evidence conducted on human participants and published in English were identified from MEDLINE (via PubMed), EMBASE, the Cochrane Library, Agency for Healthcare Research and Quality, and CINAHL for selected searches.

Structure: Recommendations from the "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease" have been updated with new evidence to guide clinicians.

Peripheral artery disease (PAD) is prevalent among people with diabetes, and the combination is associated with high risk of cardiovascular events and adverse limb outcomes. Greater attention to this population is needed to mitigate the adverse consequences of this severe manifestation of atherosclerotic cardiovascular disease. Accordingly, the diagnosis and management of PAD in people with diabetes is an important public health concern, especially among vulnerable populations. Many people with diabetes do not experience typical symptoms of PAD, and PAD may go undetected at earlier stages in those with diabetes. The diagnosis is therefore often delayed until PAD is advanced to chronic limb-threatening ischemia. As a result, the rates of amputation are disproportionately high in this population. In addition, guideline-directed medical therapies for treatment of PAD in people with diabetes are underutilized. To address these concerns, this scientific statement provides an overview of current recommendations for screening, diagnosis, and management, including consensus recommendations that underscore evidence-based treatments. This scientific statement also outlines promising areas for future research, including use of electronic health records to support more timely diagnosis, optimal timing and methods for screening, and standardization of clinical trial endpoints.

Background: Timely diagnosis and treatment are critical to reduce morbidity and mortality for patients with transthyretin amyloidosis with cardiomyopathy (ATTR-CM). Limited existing data suggest underdiagnosis of ATTR-CM and inequity in predictors of diagnosis patterns.

Objective: This study sought to describe the time from heart failure (HF) diagnosis to ATTR-CM diagnosis and identify predictors for delayed diagnosis of ATTR-CM.

Methods: This retrospective cohort study used Veterans Health Administration (VHA) data. We identified patients with HF and ATTR-CM diagnosed between 2016 and 2022 using an algorithm based on diagnoses and medications. The primary outcome was the time to diagnosis of ATTR-CM and was defined as the number of days between each patient's first HF diagnosis and their first ATTR-CM diagnosis. We also evaluated the number of days between first HF hospitalization or first loop diuretic prescription and ATTR-CM diagnosis. We used multivariable logistic regression to assess demographic, clinical, and socioeconomic predictors of time to ATTR-CM diagnosis using >6 months as a meaningful delay.

Results: A total of 2,557 patients with HF and ATTR-CM were identified. The mean age at the time of ATTR-CM diagnosis was 81 years. Most veterans were male (2,544; 99.5%) and White (1,440; 56%). The median time to diagnosis to ATTR-CM was 490 days. For the 1,882 veterans with a loop diuretic prescription before ATTR-CM diagnosis, the median time between initial loop prescription and ATTR-CM diagnosis was 835 days (Q1-Q3: 250-1850). Across Department of Veterans Affairs sites, the median number of days to diagnosis ranged from 169 to 1,070 days. After adjustment, Black race (OR: 0.71; 95% CI: 0.57-0.88) and older age (OR: 0.66; 95% CI: 0.59-0.73) were associated with a shorter time to diagnosis, whereas a history of atrial fibrillation (OR: 1.21; 95% CI: 1.00-1.45), coronary artery disease (OR: 1.38; 95% CI: 1.15-1.64), or chronic kidney disease (OR: 1.79; 95% CI: 1.50-2.15) was associated with longer time to diagnosis.

Conclusions: There are clinically important delays between incident HF and diagnosis of ATTR-CM. Carrying a diagnosis of atrial fibrillation, coronary artery disease, or chronic kidney disease was associated with a longer delay to diagnosis. These findings uncover an opportunity for clinicians to consider concomitant ATTR-CM in patients with alternate etiologies for their cardiomyopathy, as expediting evaluation for ATTR-CM following HF diagnosis is critical to reduce the morbidity of this progressive condition.

Background: The most common causes of death may change over time in heart failure with reduced ejection fraction (HFrEF). These shifts can influence the risk-benefit balance of interventions such as implantable cardioverter-defibrillators (ICDs), which are designed to prevent sudden cardiac death. Long-term follow-up is therefore essential to determine whether early benefits are sustained, attenuated, or lost over time.

Objectives: This study sought to examine the long-term effect of primary prevention ICD implantation, compared with usual clinical care, in patients with nonischemic HFrEF enrolled in the DANISH (Danish Study To Assess the Efficacy of ICDs in Patients With Nonischemic Systolic Heart Failure on Mortality) trial.

Methods: The DANISH trial enrolled 1,116 patients with nonischemic HFrEF, left ventricular ejection fraction ≤35%, NYHA functional class II-III (class IV if cardiac resynchronization therapy was planned), and elevated natriuretic peptide levels. The primary outcome was all-cause death, and secondary outcomes were cardiovascular death and sudden cardiovascular death. In this study with extended follow-up, patients were followed until death or January 31, 2024, whichever came first.

Results: During a median follow-up of 13.2 years (Q1-Q3: 11.6-14.6 years), 294 patients (52.9%) in the ICD group and 299 (53.4%) in the control group died. Compared with usual clinical care, ICD implantation did not significantly reduce the long-term rate of all-cause death (HR: 0.96; 95% CI: 0.82-1.13), but it did reduce the long-term rate of sudden cardiovascular death (HR: 0.54; 95% CI: 0.36-0.80). The effect of ICD implantation on all-cause death was consistent regardless of age (P_interaction = 0.89). However, age significantly modified the effect of ICD implantation on sudden cardiovascular death, such that ICD implantation reduced the rate of this outcome in patients ≤70 years (HR: 0.38; 95% CI: 0.23-0.62), but not in those >70 years (HR: 1.27; 95% CI: 0.56-2.89; P_interaction = 0.01). Similar trends were observed when age was analyzed as a continuous variable. The effect of ICD implantation was generally consistent across other key subgroups, including cardiac resynchronization therapy use at baseline.

Conclusions: In patients with nonischemic HFrEF, during a median follow-up of 13.2 years, primary prevention ICD implantation did not reduce all-cause death, but it did reduce sudden cardiovascular death, and younger individuals appeared to derive a greater benefit. (Danish ICD Study in Patients With Dilated Cardiomyopathy [DANISH]; NCT00542945).

Background: No therapy is approved for patients with symptomatic nonobstructive hypertrophic cardiomyopathy (nHCM). The ODYSSEY-HCM (A Study of Mavacamten in Non-Obstructive Hypertrophic Cardiomyopathy [ODYSSEY-HCM]; NCT05582395) trial, the largest to date in patients with hypertrophic cardiomyopathy (HCM), evaluating the efficacy of mavacamten in symptomatic adults with nHCM, did not demonstrate improvements in its primary endpoints (functional capacity and patient-reported health status).

Objectives: The current exploratory analysis from the ODYSSEY-HCM trial reports the associations between: 1) baseline biomarkers (N-terminal pro-B-type natriuretic peptide [NT-proBNP] and high-sensitivity cardiac troponin I [cTnI]) with clinical, exercise, and echocardiographic characteristics; and 2) comparing changes in these biomarkers from baseline to week 48 between mavacamten and placebo groups.

Methods: Symptomatic nHCM patients were randomized to placebo or mavacamten (starting at 5 mg/d, with titration ranging from 1 to 15 mg, based on left ventricular [LV] ejection fraction). Along with clinical and echocardiographic parameters, NT-proBNP and hs-cTnI were measured for changes from baseline to week 48.

Results: Among 580 randomized patients (mean age 56 ± 15 years, 266 [45.9%] women), baseline biomarkers were elevated with median NT-proBNP 917.5 ng/L (IQR: 463-1,725 ng/L) and hs-cTnI of 29.1 ng/L (IQR: 14.4-91.7 ng/L). On multivariable analysis, female sex, body mass index, NYHA functional class, maximal LV wall thickness, left atrial volume index, and E/e' were associated with higher baseline NT-proBNP. Only younger age and LV mass index were associated with higher baseline hs-cTnI level. At week 48, the mavacamten group had a 58% reduction in NT-proBNP (geometric mean ratio: 0.42; 95% CI: 0.37-0.47; P < 0.001) and a 51% reduction in hs-cTnI (geometric mean ratio: 0.49; 95% CI: 0.45-0.53; P < 0.001). No significant change in either biomarker was seen in the placebo group. These reductions occurred early and were maintained through week 48.

Conclusions: Treatment with mavacamten for 48 weeks in nHCM patients was associated with marked biomarker improvements compared with placebo. Whether these changes translate into longer-term adaptive remodeling and improvements in patient-reported health status, exercise capacity, and outcomes remain to be ascertained. (A Study of Mavacamten in Non-Obstructive Hypertrophic Cardiomyopathy [ODYSSEY-HCM]; NCT05582395).