Abstract 4115542: Female patients with Andersen-Tawil Syndrome are more prone to develop cardiac arrest.

Background: Andersen-Tawil Syndrome (ATS) is an autosomal dominant disorder characterized by a triad of dysmorphic features, periodic muscular weakness, and ventricular arrhythmias. We presented a meta-analysis of cardiologic-affected ATS patients. Objective: This study sought to compare the characteristic features of ATS according to their gender with a special focus on the cardiologic anomalies. Methods: To enable a meta-analysis, we searched literature reviews of reported cases or cohort studies with ATS between 1994 and 2024. Cases were selected if they presented a cardiologic affection alone or combined with morphologic features, periodic paralysis, or both. A gender comparison was made using the available information. Each variable was calculated using the validated percentage. Numerical variables are expressed as mean or median and SD. Categorical variables are expressed as percentages. Student’s t-test was used for numerical variables. Categorical variables were analyzed using chi-square. The results were expressed as an odds ratio (OR) with a 95% confidence interval (CI). Values of p <0.05 were considered statistically significant. Results: The studied population consisted of 243 cardiologic-affected patients with ATS, 163 females, and 80 Males. Compared to males, females are less likely to present periodic paralysis (p = 0.03, OR = 0.54, 95% CI = 0.30-0.98). Analyzing cardiologic features, females had more syncope episodes than males (73% (n=52) vs. 22.4% (n=15). Only 45% of cases had a prolonged QTc. A longer QTc measure was reported in females (453 ± 52) compared to males (427 ± 55) (p = 0.004). Premature ventricular contractions (PVCs) were the most commonly reported arrhythmia, and bigeminy was the most common type of presentation. Polymorphic PVCs were more prevalent in females than males (p = 0.009, OR = 2.49, 95%CI = 1.24-4.99). Females reported more Ventricular tachycardias (VT) episodes than males, especially Bidirectional (p = 0.01, OR = 2.10, 95%CI = 1.17-3.78). Thirty-five patients had an episode of cardiac arrest, either non-fatal, fatal or in some cases non-fatal, followed by SCD after years. Females were more prone to develop cardiac arrest compared to males (n=29 vs. n=6, respectively; p = 0.03, OR = 2.66, 95%CI = 1.06-6.72). Conclusion: ATS females with cardiologic affection may require a closer follow-up due to the higher risk of developing life-threatening arrhythmias and cardiac arrest than males.