JHVS: Prevalence, Genetics, and Imaging Characteristics of Patients with Mitral Valve Prolapse and Arrhythmogenic Right Ventricular Cardiomyopathy

And in this article, the authors describe the the prevalence, genetics, and imaging characteristic of mitral valve prolapse in arythmogenic right ventricular cardiomyopathy.

So, in a cohort of 111 patients with arrhythmogenic right ventricular cardiomyopathy, the authors reported that 14% had mitral valve prolapse.

And among 69 patients who had concomitant mitral valve prolapse and arrhythmogenic right ventricular cardiomyopathy, actually, they found that the PKP2 mutations were highly prevalent, actually, 83% of this patient with concomitant mitral valve prolapse and arrhythmogenic right ventricular cardiomyopathy.

So, the authors conclude that mitral valve prolapse is a prevalent finding in arrhythmogenic right ventricular cardiomyopathy and is characterized by PKP2 genetic variation in most cases.

Typical features of arrhythmic mitral valve prolapse, such as biliflate involvement and analysis junction, are actually rare in patients with mitral valve prolapse and concomitant arrhythmogenic right ventricular cardiomyopathy.

whereas features of arrhythmogenic left-sided cardiomyopathy are more common.

So further studies are now needed to understand the role of the PKP2 genetic variation and mitral valve prolapse interaction in arrhythmic wrist stratification and left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy.

So this article is accompanied by an elegant editorial by Avi Sabag from Sheba Hospital in Israel, who stated that this study represents an important step forward in understanding the intricate arrythmiogenic mechanisms of both mitral valve prolapse and arrhythmogenic right ventricular cardiomyopathy.

Therefore, paving the way for further research focused on the interaction of mitral valve collapse with other established independent arrhythmogenic substrates.

So mitral valve prolapse in arrhythmogenic right ventricular cardiobiopathy may in fact define an overlapping phenotype with more left ventricular involvement, different arrhythmic mechanism than isolated mitral valve prolapse, and possible enrichment for PKP2 variants.