HCM patients with LV apical aneurysms are at high risk for arrhythmic sudden death and thromboembolic events. Identification of this phenotype expands risk stratification and can lead to effective treatment interventions for potentially life-threatening complications.

Aims Desmoplakin (DSP) cardiomyopathy is an increasingly recognized form of arrhythmogenic cardiomyopathy. With a genotype-specific approach, we characterized the diagnosis, natural history, and risk for ventricular arrhythmia and heart failure in DSP cardiomyopathy. Methods and results We followed 91 individuals [45 probands, 34% male, median age 27.5 years (interquartile interval 20.0–43.9)] with pathogenic or likely pathogenic DSP variants for a median of 4.3 years. Regarding the ventricular involvement, left predominance was most common (n = 22, 28%) followed by bi-ventricular in 12 (15%) and right predominance in 5 (6%). Myocardial injury (chest pain, elevated troponin, normal coronary angiogram) occurred in 20 (22%) individuals. Incidence rates of sustained ventricular arrhythmia and heart failure (ventricular dysfunction ± symptoms) were 5.9 [95% confidence interval (CI): 3.9–9.1] and 6.7 (95% CI: 4.5–9.8) per 100 person-years, respectively. In univariate regression, myocardial injury was associated with sustained ventricular arrhythmia [hazard ratio (HR) 2.53, 95% CI: 1.05–6.11] and heart failure (HR 7.53, 95% CI: 3.10–18.26). After adjustment, left ventricular ejection fraction <35% and right ventricular dysfunction were prognostic for sustained ventricular arrhythmia while proband status and myocardial injury were prognostic for heart failure (all P < 0.05). The sensitivity of the arrhythmogenic right ventricular cardiomyopathy Task Force Criteria in diagnosing left dominant disease was 0.73; 5/22 (23%) of patients with sustained ventricular arrhythmias did not meet these criteria. Conclusion DSP cardiomyopathy affects both ventricles and carries high risk for ventricular arrhythmia and heart failure. Myocardial injury is associated with worse disease outcomes. Both diagnosis and risk stratification of DSP cardiomyopathy need refinement.

BackgroundPrior studies have shown a close link between exercise and development of arrhythmogenic right ventricular cardiomyopathy. How much exercise restriction reduces ventricular arrhythmia (VA), how genotype modifies its benefit, and whether it reduces risk sufficiently to defer implantable cardioverter‐defibrillator (ICD) placement in arrhythmogenic right ventricular cardiomyopathy are unknown.Methods and ResultsWe interviewed 129 arrhythmogenic right ventricular cardiomyopathy patients (age: 34.0±14.8 years; male: 60%) with ICDs (36% primary prevention) about exercise participation. Exercise change was defined as annual exercise duration and dose in the 3 years before clinical presentation minus that after presentation. The primary outcome was appropriate ICD therapy for VA. During the 5.1 years (interquartile range: 2.7–10.8 years) after presentation, 74% (95/129) patients reduced exercise dose and 85 (66%) patients experienced the primary outcome. In multivariate analyses, top tertile reduction in exercise duration and dose were both associated with less VA (duration: hazard ratio: 0.23 [95% confidence interval, 0.07–0.81]; dose: hazard ratio: 0.14 [95% confidence interval, 0.04–0.44]). Greater reduction in exercise dose conferred greater reduction in VA (P=0.01 for trend). Patients without desmosomal mutations and those with primary‐prevention ICDs benefited more from exercise reduction (P=0.16 and P=0.06 for interaction); however, 58% (18/31) of athletes who reduced exercise dose by >80% still experienced VA.ConclusionsExercise restriction should be recommended to all arrhythmogenic right ventricular cardiomyopathy patients with ICDs. Patients who are “gene‐elusive” and those with primary‐prevention devices may particularly benefit. Exercise reduction is unlikely to reduce arrhythmia sufficiently in high‐risk patients to alter decision‐making regarding ICD implantation.

IntroductionProcalcitonin (PCT) is a biomarker for the clinical diagnosis of bacterial infection that is more specific and earlier than fever, changes in white blood cell count, and blood cultures. Congestive heart failure is an important cause of endotoxin resorption from the intestine, which significantly increases PCT expression in noninfected patients with heart failure. The diagnostic performance and cut-off value of PCT in patients with bacterial infection complicated by congestive heart failure needs to be confirmed.MethodsA total of 4,698 cases from different cities in China, including those with different classes of congestive heart failure, bacterial infection, bacterial infection complicated by heart failure and healthy individuals, were chosen for the diagnostic value analysis of PCT and screening candidate predictors of mortality in subjects with bacterial infection complicated by congestive heart failure.ResultsPatients with simple heart failure had significantly higher PCT levels than normal controls (P < 0.01), whereas patients with bacterial infection complicated by congestive heart failure had significantly higher PCT levels than those with simple infection (P < 0.01). Although it was useful for the diagnosis of infection (area under the receiver operating characteristic curve >80%), the positive predictive value of PCT decreased significantly with increasing severity of heart failure (P < 0.05), and the cut-off value of PCT concentrations for infection complicated by classes II, III and IV heart failure were 0.086, 0.192 and 0.657 μg/L, respectively. Heart failure degree, PCT level, and age were the candidate predictors of mortality in patients with bacterial infection complicated by congestive heart failure.ConclusionsThese data suggest that complicated heart failure elevates the PCT level in patients with bacterial infection. Thus, the results of the PCT test must be analyzed correctly in consideration of the severity of heart failure. Close attention should be paid to cardiac function and PCT expression in aged patients with infection complicated by congestive heart failure.

Aims In arrhythmogenic right ventricular cardiomyopathy (ARVC) patients, exercise worsens disease course, so exercise restriction is recommended. However, recommendations for genotype-positive ARVC family members is incompletely resolved. We aimed to provide evidence for exercise recommendations for genotype-positive ARVC family members. Methods and results Arrhythmogenic right ventricular cardiomyopathy family members inheriting a pathogenic desmosomal variant were interviewed about exercise history from age 10. Exercise was characterized by duration, intensity, and dose (duration*intensity). Associations between exercise and (i) diagnosis by 2010 Task Force Criteria and (ii) development of sustained ventricular arrhythmias were examined. The study included 101 family members (age: 40.5 ± 19.3 years, male: 41%, Plakophilin-2 variant: 81%). Forty-four individuals (44%) met diagnostic criteria and 16 (16%) experienced sustained ventricular arrhythmia. Individuals who met diagnostic criteria had significantly higher average exercise duration and dose, but not peak intensity than those who did not. Only one individual who exercised below the American Heart Association recommended minimum (650 metabolic equivalent of task-hours/year) met diagnostic criteria or experienced sustained ventricular arrhythmia as opposed to 50% of individuals who exceeded it (adjusted odds ratio = 0.03, 95% confidence interval 0.003–0.26). The difference in exercise exposure between affected and unaffected individuals was more striking in females than in males. Females who had done high-dose exercise in adolescence had the worst survival free from diagnosis (P &lt; 0.01). Conclusions In phenotype-negative ARVC family members with a pathogenic desmosomal variant, athletic activities should be limited, particularly exercise dose. Exercise may play a greater role in promoting disease in female family members.

Background Successful anticoagulation is critical for stroke prevention in adults with atrial fibrillation (AF). Anticoagulation satisfaction is a key indicator of treatment success. While physical, cognitive, and psychosocial limitations are common in elderly AF patients, their associations with anticoagulation satisfaction are unknown. Objective Examine whether anticoagulation satisfaction differs among AF patients with and without physical, cognitive, and psychosocial conditions. Methods The study comprised AF patients greater than or equal to 65 years old who were prescribed an oral anticoagulant (warfarin 57%; direct oral anticoagulant [DOAC] 43%). Frailty, cognitive function, social support, depressive symptoms, vision, hearing, and anxiety were assessed using validated measures. Anticoagulation satisfaction was measured using the anticlot treatment scale. Results Participants (n = 1037, 50% female) were on average 76 years old. The following conditions were prevalent: frailty (14%), cognitive impairment (42%), social isolation (13%), vision impairment (35%), hearing impairment (36%), depression (29%), and anxiety (24%). Average anticlot treatment burden scale was 55 out of 60 (lower burden scales indicating higher perceived burden). Patients with high perceived burden were older, more likely to be female, and receive warfarin. After adjusting for confounders, visual impairment (adjusted odds ratio [95% confidence interval]: 1.7 [1.2‐2.4]), depressive symptoms (2.4 [1.6‐3.7]), and anxiety (1.8 [1.2‐2.7]) were significantly associated with high perceived burden. Different conditions were associated with high perceived burden in warfarin vs DOAC users. Conclusion Physical, cognitive, and psychosocial limitations are prevalent and associated with high perceived anticoagulation burden among elderly AF adults. These conditions merit consideration in anticoagulation prescribing.

Betel nut use at long duration or high cumulative exposure levels is associated with subclinical atherosclerosis as manifested through carotid IMT. This effect is especially pronounced among men and cigarette smokers.

Background In older patients with atrial fibrillation (AF), cognitive impairment and frailty are prevalent. It is unknown whether the risk and benefit of anticoagulation differ by cognitive function and frailty. Methods A total of 1244 individuals with AF with age ≥65 years and a CHADSVASC score ≥2 were recruited from clinics in Massachusetts and Georgia between 2016 and 18 and followed until 2020. At baseline, frailty status and cognitive function were assessed. Hazard ratios of anticoagulation on physician adjudicated outcomes were adjusted by the propensity for receiving anticoagulation and stratified by cognitive function and frailty status. Results The average age was 75.5 (± 7.1) years, 49% were women, and 86% were prescribed oral anticoagulants. At baseline, 528 (42.4%) participants were cognitively impaired and 172 (13.8%) were frail. The adjusted hazard ratios of anticoagulation for the composite of major bleeding or death were 2.23 (95% confidence interval: 1.08–4.61) among cognitively impaired individuals and 0.94 (95% confidence interval: 0.49–1.79) among cognitively intact individuals (P for interaction = 0.08). Adjusted hazard ratios for anticoagulation were 1.84 (95% confidence interval: 0.66–5.13) among frail individuals and 1.39 (95% confidence interval: 0.84–2.40) among not frail individuals (P for interaction = 0.67). Conclusion Compared with no anticoagulation, anticoagulation is associated with more major bleeding episodes and death in older patients with AF who are cognitively impaired.