Arrhythmogenic Mitral Valve Prolapse (Arrhythmogenic Barlow disease)
Presentation
Polymorphic ventricular extrasystoles. Known mitral valve disease.
Patient Data
prolapse + annular disjunction
Mitral regurgitation fraction = 41% in MR
Left Atrium dilated (37cm²)
Prolapsus of both mitral leaflets between 8 to 11 mm compared to annulus.
Disjunction of mitral annulus between 6.0 to 6.5mm relative to the myocardium.
Curling (systolic "rolling" movement of LV inferolateral myocardium next to the annular disjunction)
LGE of lateral papillar muscle
Late gadolinium enhancement of the lateral papillary muscle indicating fibrosis: potential origin of polymorphic ventricular extrasystoles.
Case Discussion
Arrhythmogenic Barlow disease is a newly described entity combining 1,2:
mitral valve prolapse (MVP), a common condition
presence of complex or frequent ventricular arrhythmias in the absence of any other well-known arrhythmic substrate or cause (including myocardial ischemia/infarction/scar, cardiomyopathy, channelopathy etc.)
The arrhythmogenic events might be related to fibrotic changes in the papillary muscles caused by the prolapsing valve. Our case displays all those features.
Patients with a 5 mm or longer MAD distance and late gadolinium enhancement at MRI may have an elevated risk for cardiac arrhythmia and sudden cardiac death compared with patients with a shorter MAD distance and/or no fibrosis.
An ICD and/or mitral valve repair should be considered in truly high-risk patients.
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