Malignant MVP

https://johnsonfrancis.org/professional/malignant-mvp/

Malignant MVP (Mitral Valve Prolapse)

Various terms like “Malignant bileaflet mitral valve prolapse syndrome” [1] and “Arrhythmic mitral valve prolapse”[2] and “malignant arrhythmic MVP”[3] have been used to describe mitral valve prolapse associated with ventricular arrhythmias and sudden cardiac death.

The subset of patients with malignant MVP are those who have life threatening ventricular arrhythmias. They have bileaflet MVP, female gender and frequent complex ventricular ectopy. Ventricular ectopy originating from the outflow tract alternate with those from papillary muscle or fascicles [1].

Pathophysiological mechanisms of electrical instability include [3]:

  1. Fibrosis in the papillary muscles and inferobasal left ventricular wall
  2. Mitral annulus disjunction
  3. Systolic curling

 The typical case is described as a female with bileaflet myxomatous disease, mid systolic click, repolarization abnormalities in inferior leads and complex ventricular arrhythmias of polymorphic or right bundle branch block morphology. Significant regurgitation is uncommon [3].

References

  1. Sriram CS, Syed FF, Ferguson ME, Johnson JN, Enriquez-Sarano M, Cetta F, Cannon BC, Asirvatham SJ, Ackerman MJ. Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest.J Am Coll Cardiol. 2013; 62:222–230.
  2. Basso C, Perazzolo Marra M, Rizzo S, De Lazzari M, Giorgi B, Cipriani A, Frigo AC, Rigato I, Migliore F, Pilichou K, Bertaglia E, Cacciavillani L, Bauce B, Corrado D, Thiene G, Iliceto S. Arrhythmic mitral valve prolapse and sudden cardiac death.Circulation. 2015; 132:556–566.
  3. Basso C, Iliceto S, Thiene G, Perazzolo Marra M. Mitral Valve Prolapse, Ventricular Arrhythmias, and Sudden Death. Circulation. 2019 Sep 10;140(11):952-964.

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