Arrhythmogenic right ventricular cardiomyopathy/dysplasia on the basis of the revised diagnostic criteria in affected families with desmosomal mutations

Aims To evaluate arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) in affected families with desmosome mutations on the basis of the recently revised Task Force Criteria (TFC).Methods and results One hundred and three consecutive carriers of pathogenic desmosome mutations and 102 mutation-negative relatives belonging to 22 families with dominant and 14 families with recessive ARVC/D were evaluated according to the original and revised TFC. Serial cardiac assessment with 12-lead, signal-averaged, and 24 h ambulatory ECG and two-dimensional echocardiography was performed. Clinical events and outcome were prospectively analysed up to 24 years (median 4 years). With the revised criteria, 16 carriers were newly diagnosed on the basis of ECG abnormalities in 100%, ventricular arrhythmias in 79%, and functional/structural alterations in 31%, increasing diagnostic sensitivity from 57 to 71% (P=0.001). Task Force Criteria specificity improved from 92 to 99% (P=0.016). In dominant mutation carriers, penetrance changed significantly (61 vs. 42%, P=0.001); no changes were observed in recessive homozygous carriers (97 vs. 97%, P=1.00). Affected carriers according to the revised TFC (n=73) had 12-lead ECG abnormalities in 96%, ventricular arrhythmias in 91%, and functional/structural alterations fulfilling echocardiographic criteria in 76%. Cumulative and event-free survival did not differ significantly between dominant and recessive affected carriers, being at 78.6 vs. 76 and 51.7 vs. 55.4%, respectively, by the age of 40 years.Conclusion Revised TFC increased diagnostic sensitivity particularly in dominant ARVC/D. Serial family evaluation may rely on electrocardiography which seems to have the best diagnostic utility particularly in early disease that is not detectable by two-dimensional echocardiography

Anticoagulation with edoxaban in patients with long Atrial High-Rate Episodes ≥24 hours

BACKGROUND AND AIMS: Patients with long atrial high-rate episodes (AHRE) ≥ 24 hours and stroke risk factors are often treated with anticoagulation for stroke prevention. Anticoagulation has never been compared to no anticoagulation in these patients.METHODS: This secondary prespecified analysis of NOAH-AFNET 6 examined interactions between AHRE duration at baseline and anticoagulation with edoxaban compared to placebo in patients with AHRE and stroke risk factors. The primary efficacy outcome was a composite of stroke, systemic embolism, or cardiovascular death. The safety outcome was a composite of major bleeding and death. Key secondary outcomes were components of these outcomes and ECG-diagnosed atrial fibrillation.RESULTS: AHRE ≥24 hours were present at baseline in 259/2389 patients enrolled in NOAH-AFNET 6 (11%, 78 ± 7 years old, 28% women, CHA2DS2-VASc score 4). Clinical characteristics were not different from patients with shorter AHRE. During a median follow-up of 1.8 years, the primary outcome occurred in 9/132 patients with AHRE ≥24 hours (4.3%/patient-year, 2 strokes) treated with anticoagulation and in 14/127 patients treated with placebo (6.9%/patient-year, 2 strokes). AHRE duration did not interact with the efficacy (p-interaction = 0.65) or safety (p-interaction = 0.98) of anticoagulation. Analyses including AHRE as a continuous parameter confirmed this. Patients with AHRE ≥24 hours developed more ECG-diagnosed atrial fibrillation (17.0%/patient-year) than patients with shorter AHRE (8.2%/patient-year; p &lt; 0.001).CONCLUSIONS: This hypothesis-generating analysis does not find an interaction between AHRE duration and anticoagulation therapy in patients with device-detected AHRE and stroke risk factors. Further research is needed to identify patients with long AHRE at high stroke risk.</p

Atrial high-rate episodes and stroke prevention.

While the benefit of oral anticoagulants (OACs) for stroke prevention in patients with atrial fibrillation (AF) is well established, it is not known whether oral anticoagulation is indicated in patients with atrial high-rate episodes (AHRE) recorded on a cardiac implantable electronic device, sometimes also called subclinical AF, and lasting for at least 6 min in the absence of clinically diagnosed AF. Clinical evidence has shown that short episodes of rapid atrial tachycarrhythmias are often detected in patients presenting with stroke and transient ischaemic attack. Patients with AHRE have a higher likelihood of suffering from subsequent strokes, but their stroke rate seems lower than in patients with diagnosed AF, and not all AHRE episodes correspond to AF. The prognostic and pathological significance of AHRE is not yet fully understood. Clinical trials of OAC therapy are being conducted to determine whether therapeutic intervention would be beneficial to patients experiencing AHRE in terms of reducing the risk of stroke

Complications Related to Cardiac Rhythm Management Device Therapy and Their Financial Implication: A Prospective Single-Center Two-Year Survey

Cardiac rhythm management devices (CRMDs) have proven their clinical effectiveness for patients with heart rhythm disorders. Little is known about safety and complication rates during the implantation of these devices. This study demonstrated the complication rates related to CRMD implantation, and estimated the additional hospital stay and cost associated with the management of complications. Methods: During a period of one year, a total of 464 consecutive recipients underwent CRMD implantation and were followed for 2 years. Finally, data were analyzed for 398 patients who completed the two-year follow up, resulting in a total of 796 patient-years. Results: Of the 201 patients with initial pacemaker (PM) implantations, 6 (2.99%) had seven complications (5 patients had lead dislodgement, 1 of them twice), and 1 patient developed pocket infection. Of the 117 PM replacements, 1 (0.85%) patient developed a complication (pocket erosion). Two patients with complications (1 with an initial PM and 1 with a replacement) died before completing the follow up for reasons unrelated to cardiac causes. There were no complications in either initial implantations (69 patients) or replacements (11 patients) of implantable cardioverter-defibrillators. The average prolongation of the hospital stay was 7 days, ranging from 1 to 35 days, resulting in €17,411 of total additional direct hospital costs. Conclusion: This study found relatively low rates of complications in patients undergoing CRMD implantation, initial or replacement, in our center, compared with other studies. The additional hospitalization days and costs attributable to these complications depend on the nature of the complication

Primary Biliary Cirrhosis in a genetically homogeneous population: Disease associations and familial occurrence rates

Abstract Background Primary biliary cirrhosis (PBC) is a disease with genetic and environmental pathogenetic background. Chemicals, infectious agents, hormone therapy, reproductive history and surgical interventions have been implicated in the induction of PBC. Familial PBC has been documented in first degree relatives (FDR). Most cohort studies are genetically heterogeneous. Our study aimed to determine eventual lifestyle or disease associations and familial occurrence rates in a genetically homogeneous and geographically defined population of PBC patients. Methods 111 consenting PBC patients, were compared with 115 FDR and 149 controls matched for age, sex, Cretan origin and residence. All participants completed a questionnaire regarding demographics, lifestyle, medical, surgical and reproductive history. Significant variables on the univariate analysis were analyzed by multivariate analysis using a forward step-wise logistic regression model. Results Dyslipidaemia was found in 69.4% of patients, 60% of FDR and 40.9% of controls (p  Conclusions Dyslipidaemia and autoimmune diseases were significantly increased not only in patients as expected but also in their FDR. An increased prevalence of malignancies was found in patients. Primary educational level, cholecystectomy and the presence of at least another autoimmune disease were found as putative risk factors for PBC. No association was found with smoking, urinary tract infection or reproductive history. The reported high familial occurrence of PBC could imply screening with AMA of FDR with at least another autoimmune disease.</p

Progression of Macular Atrophy in Patients Receiving Long-Term Anti-VEGF Therapy for Age-Related Macular Degeneration: Real-Life Data

Purpose: This study aimed to evaluate the progression of macular atrophy (MA) based on near-infrared reflectance (NIR) and optical coherence tomography (OCT) images, in patients with age-related macular degeneration (AMD), receiving anti-vascular endothelial growth factor (anti-VEGF) treatment for at least a 6-year period. Materials and Methods: This retrospective study included 53 naïve patients (53 eyes) with neovascular AMD from 2 centers, who were treated with anti-VEGF intravitreal injections and had no MA at baseline. MA was evaluated in an annual basis using NIR images, while all available OCT images were used to confirm that the atrophic area fulfilled the criteria proposed by the Classification of Atrophy Meetings (CAM) group for complete retinal pigment epithelium and outer retinal atrophy. Incidence and progression of MA were evaluated. Associations with best-corrected visual acuity (BCVA) and total number of injections were also studied. Results: Treatment duration of our patients was 7.34 ± 1.54 years. The mean number of anti-VEGF injections was 24.4 ± 13.6. BCVA at baseline was 0.38 ± 0.27 logMAR, while at the final visit, it was 0.60 ± 0.35 logMAR (p = 0.731). The cumulative incidence of new MA at years 1, 2, 3, 4, 5, and 6 was 1.89%, 18.87%, 32.08%, 39.62%, 49.06%, and 50.94%, respectively. In patients who developed MA, mean MA area increased from zero at baseline to 5.66 ± 7.18 mm2 at the final visit. The estimated annual enlargement of MA was 0.45 mm/year based on square root transformation (1.12 mm2/year, untransformed data). MA progression does not appear to be significantly associated with age (R = 0.055; p = 0.784), gender (R = 0.113; p = 0.576), BCVA (R = 0.168; p = 0.404), and total number of injections (R = 0.133; p = 0.255). Conclusion: In this real-life setting, half of the neovascular AMD patients under anti-VEGF treatment, without MA at therapy initiation, developed MA over a period of at least 6 years. In this work, the number of injections did not seem to have a significant association with MA progression.</p

Peripheral Blood MicroRNAs as Potential Biomarkers of Myocardial Damage in Acute Viral Myocarditis

Background: microRNAs (miRs) have emerged as important modulators of cardiovascular development and disease. Our aim was to determine whether cardiac-related miRs such as miR-21-5p and miR-1-3p were differentially expressed in acute viral myocarditis and whether any of them was related with the extent of myocardial damage and left ventricular dysfunction. Methods: We enrolled 40 patients with acute viral myocarditis. Blood samples were taken on admission and miRs expression levels in peripheral blood mononuclear cells were quantified by real-time reverse transcription polymerase chain reaction. Results: miR-21-5p, miR-1-3p were significantly elevated in acute myocarditis. miR-21-5p levels showed a strong correlation with global longitudinal strain (r = 0.71, p &lt; 0.01), while miR-1-3p had significant correlations with troponin I (r = 0.79, p &lt; 0.01). Conclusions: The expression of miR-21-5p and miR-1-3p in peripheral blood is increased in acute viral myocarditis, and this increase is correlated with myocardial damage and indicative of left ventricular systolic dysfunction in these patients

Long-term serial changes in platelet activation indices following sirolimus elution and bare metal stent implantation in patients with stable coronary artery disease

Background: Platelet activation is crucial in the development of stent thrombosis following percutaneous coronary intervention (PCI). We carried out a long-term assessment of multiple factors implicated in the thrombotic process and monitored markers of platelet activation after the implantation of sirolimus-eluting stents (SES) in patients with stable coronary artery disease (CAD). Additionally, we compared these findings with those after bare-metal stent (BMS) implantation. Methods: A cohort of 47 consecutive patients, aged 70% narrowing of the lumen) of one major epicardial coronary artery and stable CAD underwent successful elective PCI. Patients were randomly allocated to SES (n = 25) or BMS (n = 22). Venous blood was obtained 24 hours before and 24 hours, 48 hours, 1 month, and 6 months after PCI for measurements of plasma levels of sP-selectin, von Willebrand Factor (vWF), fibrinogen, d-dimer, sCD40, factor VIII, b-thromboglobulin (b-TG) and platelet factor 4 (PF-4). Results: There were no significant differences between the two groups in levels of fibrinogen or d-dimers in peripheral blood. However, we observed a significant kinetic effect (p<0.001) and stent-effect (p<0.015) on vWF levels and a significant kinetic effect (p = 0.012) on factor VIII, sP-selectin (p = 0.04), b-TG (p<0.001), and PF4 (p = 0.016). A trend towards a significant stent effect on sCD40 was also detected (p = 0.06). Conclusions: SES and BMS did not show significant differences in relationship to markers of platelet activation and coagulation in patients with stable CAD. Although some markers showed an increase after stent implantation, they returned to the initial levels 6 months later. Keywords: Stents, Platelet activation, Coronary artery diseas

sj-docx-3-jet-10.1177_15266028231210232 – Supplemental material for Outcomes After Open Surgical, Hybrid, and Endovascular Revascularization for Acute Limb Ischemia

Supplemental material, sj-docx-3-jet-10.1177_15266028231210232 for Outcomes After Open Surgical, Hybrid, and Endovascular Revascularization for Acute Limb Ischemia by Nikolaos Konstantinou, Angeliki Argyriou, Felicitas Dammer, Theodosios Bisdas, Gregory Chlouverakis, Giovanni Torsello, Nikolaos Tsilimparis and Konstantinos Stavroulakis in Journal of Endovascular Therapy</p