Raslan, Mohamed

Background: Cardiac amyloidosis (CA) patients with reduced left ventricular ejection fraction (EF) are at increased risk for mortality. The benefit of cardiac resynchronization therapy (CRT) in terms of mortality reduction has not been established in this population. Aim: To evaluate the survival benefit of CRT, either CRT-D or CRT-P, in a large cohort of patients with cardiac amyloidosis and reduced EF. Methods: We retrospectively analyzed patients with confirmed CA and reduced EF (<40%) between 2009 and 2023 across Mayo Clinic sites. Patients were stratified into three groups: no device, CRT with pacemaker function (CRT-P), and CRT with defibrillator function (CRT-D). CRT devices were indicated for low ejection fraction less than 40% and patients who had chronic heart failure with a wide QRS. Kaplan-Meier survival estimates were generated, and log-rank tests were used for comparison. A secondary analysis was conducted combining CRT-P and CRT-D versus no device (EF <40%), and a tertiary analysis compared CRT-P versus CRT-D. Results: Among 184 patients [36 AL (20%), 148 ATTR (80%)], 38 (19%) had no cardiac device and EF <40%, 20 (11%) received CRT-P, and 116 (63%) received CRT-D. The median follow-up was 3.4 years (1.7, 5.0). The probability of survival was significantly higher in the CRT group compared to the no device-EF<40 group (Log Rank p=0.013; figure B). Among patients receiving CRT, there was no significant difference in all-cause mortality between those receiving CRT-P and those receiving CRT-D (Log Rank p=0.5; figure C). Conclusion: In patients with cardiac amyloidosis and reduced EF, CRT is associated with significantly improved survival compared to no device. No mortality difference was observed between CRT-P and CRT-D. Prospective studies are warranted.

Introduction: Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder caused by pathogenic variants in the COL3A1 gene, often leading to arterial, intestinal, and uterine rupture. While vascular complications are well-documented, the prevalence and characteristics of cardiac arrhythmias in this population remain underexplored. Aim: To assess the prevalence of arrhythmias and conduction abnormalities in patients with confirmed vEDS. Methods: A retrospective cohort study was conducted across all three Mayo Clinic sites. Patients with vEDS were initially identified using an electronic data extraction tool. Each case was subsequently confirmed through detailed chart review. Additional reviews were performed to assess the prevalence of heart blocks and both atrial and ventricular arrhythmias. Statistical analysis was conducted using SPSS to determine the prevalence of each arrhythmia type. Results: A total of 132 patients with confirmed vEDS were included in the study. The mean age was 45.7 ± 13 years. The cohort was predominantly white (93.7%), with 47 males (35.6%) and 85 females (64.4%). Atrial arrhythmias were identified in 15 patients (11.4%), including atrial fibrillation in 14 (10.6%) and atrial flutter in 8 (6.1%). Supraventricular tachycardia (SVT) was observed in 15 patients (11.4%). Conduction abnormalities included first-degree AV block in 8 (6.1%), second-degree AV block in 3 (2.3%), left bundle branch block in 8 (6.1%), and right bundle branch block in 15 (11.4%) patients. Ventricular arrhythmias were observed in 6 patients (4.5%) with sustained ventricular tachycardia, 6 (4.5%) with non-sustained ventricular tachycardia, and 7 (5.3%) with ventricular fibrillation. Postural orthostatic tachycardia syndrome (POTS) or orthostatic hypotension was documented in 18 patients (13.6%). Conclusion: Although arrhythmias are not the most common manifestation of vEDS, their prevalence in this cohort is notably higher than in the general population and tends to occur at a younger age. These findings support the consideration of routine screening for atrial and ventricular arrhythmias in patients with vEDS as part of their long-term cardiovascular management.

Background: Noncompaction cardiomyopathy (NCCM) is a distinct myocardial disorder associated with a high risk of ventricular arrhythmias and sudden cardiac death. Although implantable cardioverter-defibrillators (ICDs) are frequently used in this population, data on predictors of shock therapy remain limited. Aim: Evaluation of a large cohort of patients with NCCM and ICDs to identify incidence and clinical factors associated with ICD shock Methods: This retrospective cohort study included patients with confirmed NCCM between 2002 and 2023 at all Mayo Clinic sites. Baseline clinical data, genetic findings, cardiac imaging parameters, and device-related outcomes were collected through detailed chart review. Multivariable Logistic regression was used to identify independent predictors. Results: Among 145 patients with NCCM who received ICDs, the median age was 45 years (IQR: 34–56), with 75 (52%) female and 120 (83%) White. The median left ventricular ejection fraction (LVEF) was 35% (IQR: 22.5–47.5%), and the median follow-up from ICD implantation was 6.4 years (IQR: 2.9–11.0). Most ICDs (124, 85.5%) were implanted for primary prevention. Appropriate shocks were observed in 37 patients (25.5%), with 14 (9.8%) experiencing three or more appropriate shocks. Inappropriate shocks occurred in 15 patients (10.3%). Device-related complications were reported in 22 patients (15.2%), including infections in 4 (2.8%), lead fractures or dislodgement in 17 (11.9%), and pneumothorax in 1 (0.7%). Clinical predictors of shocks are illustrated in the figure. Conclusion: ICD therapy for NCCM patients prevented SCD in 25.5% of patients. Syncope, RBBB, troponin T >30 ng/L, and imaging-based apical trabeculations were independently associated with ICD shocks in patients with NCCM. These factors may aid in risk stratification and clinical decision-making.

Background: Spontaneous coronary artery dissection (SCAD) is increasingly recognized as a cause of acute coronary syndrome, particularly in young to middle-aged women. While most presentations are associated with preserved left ventricular ejection fraction (LVEF), a subset of patients present with acute systolic dysfunction. The characteristics of patients who recover their LVEF are not clearly defined. Methods: We retrospectively analyzed 495 patients with angiographically confirmed SCAD treated at three Mayo Clinic sites (Arizona, Rochester, and Florida) from 1994 to 2023. Patients with a prior history of myocardial infarction, coronary artery disease, and congenital heart disease were excluded. Baseline LVEF at SCAD presentation was categorized into <35%, 35–39%, 40–49%, and ≥50%. Follow-up LVEF was assessed by echocardiography performed between 30 days and 1 year post-SCAD. The primary outcome was improvement in LVEF to ≥50% at follow-up. Results: A total of 495 SCAD patients were included. At presentation, 26% had reduced LVEF: 6% had LVEF <35%, 5% had LVEF between 35–39%, and 15% had LVEF between 40–49%. Patients who did not normalize their EF at follow-up were slightly younger (45 vs. 48 years, p=0.04), were more likely to have had an in-hospital cardiac arrest (13% vs. 4%, p=0.01), required mechanical circulatory support during their care, and had SCAD involving the left main artery (11% vs. 2%, p=0.01). Predictors of EF normalization included SCAD which involved the obtuse marginals (15% vs. 4%, p<0.05). The group with the lowest EF at presentation had the highest probability of persistent EF <50% at follow-up (32% for those with LVEF <35%) while patients who had normal EF at presentation had a 6% chance of having EF <50% at follow-up (Figure 1). Conclusions: Our study shows that the majority of SCAD patients have normal EF at presentation. 80% of those with reduced EF at presentation will normalize at follow-up. Predictors of low EF at follow-up included dissection involving the left main artery, lower EF at SCAD presentation, in-hospital arrest and use of mechanical circulatory support.