The Burden of Cardiac Arrhythmias in Loeys-Dietz Syndrome: Prevalence, Etiology, and Therapeutic Interventions.

BACKGROUND

Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder with vascular, skeletal, and cutaneous abnormalities, with elevated aneurysm risk. Although vascular complications are well studied, cardiac arrhythmias remain underexplored.

OBJECTIVES

The objectives of the study were to evaluate the prevalence and clinical implications of cardiac arrhythmias, as well as complications of electrophysiological interventions, in LDS patients.

METHODS

A retrospective cohort study was conducted on LDS patients treated at Mayo Clinic sites. Chart reviews identified arrhythmia types, timing relative to cardiac surgery, and interventions. Descriptive statistics assessed prevalence and associations.

RESULTS

The cohort included 94 patients (mean age 44.7 ± 18.8 years; 50% female). Atrial arrhythmias were present in 31.9%. Atrial fibrillation occurred in 28.7% and atrial flutter in 12.7%, with some experiencing both. Supraventricular tachycardia occurred in 19.1%. Ventricular arrhythmias were identified in 20.2%, with most cases unrelated to surgery. Conduction abnormalities were found in 37.2%, and atrioventricular block in 31.9%, with all third-degree atrioventricular block occurring after cardiac surgery. Mitral valve prolapse (MVP) and mitral annular disjunction (MAD) were common (35.1% and 11.7%, respectively) and positively associated with a higher likelihood of ventricular arrhythmias (MVP: OR: 8.253; 95% CI: 2.623-25.961; P < 0.001; MAD: OR: 10.354; 95% CI: 2.625-40.844; P < 0.001). Interventions included ablation (10.6%), pacemakers (7.5%), and implantable cardioverter-defibrillators (2.2%), with a high complication rate.

CONCLUSIONS

Cardiac arrhythmias occur frequently in LDS. Structural abnormalities like MVP and MAD are linked to ventricular arrhythmias. Frequent electrophysiological interventions and complications underscores the importance of tailored arrhythmia surveillance and management in this population.