Arrhythmic Mitral Valve Prolapse and Mitral Annular Disjunction: Clinical Features, Pathophysiology, Risk Stratification, and Management

Abstract

Mitral valve prolapse (MVP) is a common cause of valvular heart disease. Although many patients with MVP have a benign course, there is increasing recognition of an arrhythmic phenotype associated with ventricular arrhythmias and sudden cardiac death (SCD). Pathophysiologic mechanisms associated with arrhythmias include cardiac fibrosis, mechanical stress induced changes in ventricular refractory periods, as well as electrophysiologic changes in Purkinje fibers. Clinically, a variety of risk factors including demographic, electrocardiographic, and imaging characteristics help to identify patients with MVP at the highest at risk of SCD and arrhythmias. Once identified, recent advances in treatment including device therapy, catheter ablation, and surgical interventions show promising outcomes. In this review, we will summarize the incidence of ventricular arrhythmias and SCD in patients with MVP, the association with mitral annular disjunction, mechanisms of arrhythmogenesis, methods for arrhythmic and SCD risk stratification including findings with multimodality imaging, and treatments for the primary and secondary prevention of SCD.

Sudden Cardiac Arrest in a Youth with Multiple Arrhythmic Substrates

“…There is no specific treatment guideline for MAD, and treatment approaches are usually followed according to management guidelines for valvular disease, heart failure, and arrhythmias, as specific management for arrhythmias associated with MVP or MAD is unclear [ 8 ]. Treatments may include medication, devices (implantable cardioverter defibrillator (ICD)), procedures (ablation), or surgical intervention [ 9 ]. In patients at risk of ventricular arrhythmias, the insertion of an ICD should be considered for secondary prevention [ 9 ], as in this case.…”

“…Treatments may include medication, devices (implantable cardioverter defibrillator (ICD)), procedures (ablation), or surgical intervention [ 9 ]. In patients at risk of ventricular arrhythmias, the insertion of an ICD should be considered for secondary prevention [ 9 ], as in this case. Radiofrequency ablation may be done in some cases, such as those identified with frequent premature ventricular complexes on ECG or Holter monitor [ 6 ].…”

Arrhythmic Mitral Valve Prolapse and Sports Activity: Pathophysiology, Risk Stratification, and Sports Eligibility Assessment

“…A second fundamental step is the ascertainment of symptoms, including syncope, pre-syncope, and palpitations. The risk of VAs and SCD is not uniform among patients with AMVP but ranges from very high risk in patients recovered from sudden cardiac arrest, high risk in patients with unexplained syncope, and low risk in asymptomatic patients [ 5 , 64 ]. Syncope was reported in 35% of MVP patients with malignant arrhythmias or SCD [ 65 ]; thus, particularly if unexplained or occurring during exercise, in the sitting/supine position, without prodromal symptoms, or when preceded by palpitations should raise the suspicion for malignant VAs and dictate a more intense screening with 24 h Holter monitoring and exercise stress testing at a minimum [ 5 , 60 , 61 ], and mandate caution for the assessment of sports eligibility.…”

"Arrhythmic" Mitral Valve Prolapse

“…et al в 2022 г. предложили схему стратификации риска ВСС и жизнеугрожающих желудочковых аритмий у пациентов с ПМК (рис. 5) [24].…”