Arrhythmogenic cardiomyopathy (AC) is a hereditary disease leading to sudden cardiac death or heart failure. AC pathology is characterized by cardiomyocyte loss and replacement fibrosis. Our goal was to determine whether cardiomyocytes respond to AC progression by pathological hypertrophy. To this end, we examined tissue samples from AC patients with end-stage heart failure and tissue samples that were collected at different disease stages from desmoglein 2-mutant mice, a well characterized AC model. We find that cardiomyocyte diameters are significantly increased in right ventricles of AC patients. Increased mRNA expression of the cardiac stress marker natriuretic peptide B is also observed in the right ventricle of AC patients. Elevated myosin heavy chain 7 mRNA expression is detected in left ventricles. In desmoglein 2-mutant mice, cardiomyocyte diameters are normal during the concealed disease phase but increase significantly after acute disease onset on cardiomyocyte death and fibrotic myocardial remodeling. Hypertrophy progresses further during the chronic disease stage. In parallel, mRNA expression of myosin heavy chain 7 and natriuretic peptide B is up-regulated in both ventricles with right ventricular preference. Calcineurin/nuclear factor of activated T cells (Nfat) signaling, which is linked to pathological hypertrophy, is observed during AC progression, as evidenced by Nfatc2 and Nfatc3 mRNA in cardiomyocytes and increased mRNA of the Nfat target regulator of calcineurin 1. Taken together, we demonstrate that pathological hypertrophy occurs in AC and is secondary to cardiomyocyte loss and cardiac remodeling.

Background: Older age and female sex are thought to be risk factors for adverse outcomes after repair of acute type A aortic dissection (AAAD). The aim of this study is to analyze age-and sex-related outcomes in patients undergoing AAAD repair.Methods: Retrospective analysis of patients undergoing emergency AAAD repair. Patients were divided in Group A, patients aged ≥75 years and Group B <75. Intraoperative and postoperative data were compared between groups before and after propensity score matching. Sex differences were analyzed by age group.

Background: Sleep-disordered breathing (SDB) is a highly common comorbidity in patients with heart failure (HF), and a known risk factor for ventricular tachycardia (VT) development. However, little is known about the impact of SDB treatment on VT burden in HF patients to date. Therefore, this study investigated VT burden, as well as implantable cardioverter-defibrillator (ICD) therapies in HF patients with SDB treatment, in comparison to untreated SDB HF patients. Methods: This retrospective study analyzed VT burden, rate of antitachycardia pacing (ATP), and the number of shocks delivered in a propensity score-matched patient cohort of patients with SDB treatment or control. Patients had moderate or severe SDB (n = 73 per each group; standardized mean difference of 0.08) and were followed for a minimum of one year. In addition, survival over 4 years was assessed. Results: Mean patient age was 67.67 ± 10.78 and 67.2 ± 10.10, respectively, with 15.06% and 10.95% of the patients, respectively, being female. Regarding SDB subtypes in the control and SDB treatment group, central sleep apnea was present in 42.46% and 41.09% of the patients, respectively, and obstructive sleep apnea was present in 26.02% and 31.50% of the patients, respectively. Mixed type sleep disorder was present in 31.50% and 27.40% of cases. Among the SDB treatment group, a significantly lower number of VTs (28.8% vs. 68.5%; p = 0.01), ATP (21.9% vs. 50.7%; p = 0.02), as well as a lower shock rate (5.5% vs. 31.5%; p < 0.01), was observed compared to the control group. Furthermore, the VT burden was significantly lower in the SDB treatment group when compared to the time prior to SDB treatment (p = 0.02). Event-free survival was significantly higher in the SDB treatment group (Log-rank p < 0.01). Conclusion: SDB treatment in HF patients with ICD leads to significant improvements in VT burden, ATP and shock therapy, and may even affect survival. Thus, HF patients should be generously screened for SDB and treated appropriately.

Background: Sleep disordered breathing (SDB) is a frequent comorbidity in cardiac disease patients. Nevertheless, the prevalence and relationship between SDB and severe primary mitral regurgitation (PMR) has not been well investigated to date. Methods: A cohort of 121 patients with significant PMR undergoing mitral valve surgery were prospectively enrolled and received a cardiorespiratory single night polygraphy screening using ApneaLink before surgery. Eighty-two of them underwent a follow-up examination including a follow-up single-night sleep study 3 months after surgery. Results: The mean age of patients was 65.3 ± 12.0 years. Sixty patients (49.6%) were female. The mean EuroSCORE II was 2.5 ± 2.4%. Initially, 91 (75.2%) patients presented with SDB, among whom 50.4% (46 patients, 38.0% of total cohort) were classified as moderate to severe. These patients tended to require significantly longer postoperative intensive care and mechanical ventilation. Among the 82 patients who completed follow-up exams, mitral valve surgery led to a significant reduction in relevant SDB (20.7%). The apnea-hypopnea index (from 11/h [4;18] to 4/h [3;14] (p = 0.04)), the oxygenation-desaturation index (from 8/h [3;18] to 5/h [3;12] (p = 0.008)) as well as the saturation time below 90% (from 32 min [13;86] to 18 min [5;36] (p = 0.005)), were all shown to be improved significantly. Conclusion: The prevalence of SDB is very high in patients with severe primary mitral regurgitation and may contribute to postoperative complications and prolonged intensive care. A significantly reduced but still high prevalence of SDB was observed 3 months after mitral valve surgery, highlighting the bidirectional relationship between SDB and heart failure.