Barlow disease: effect of mitral valve repair on ventricular arrhythmias in 82 patients in a retrospective long-term study.

Background: Patients (pt) with mitral valve prolapse (MVP) due to Barlow disase (BD) have an increased incidence of ventricular arrhythmias (VA; including ventricular tachycardias VT) and sudden cardiac death (SCD). Data on the effect of MV repair on VA are scarce.

Methods: Pre- and postoperative VA in severe mitral regurgitation (MR) with MVP due to BD undergoing surgical mitral valve repair were analyzed. Patients with degenerative mitral valve disease not fulfilling BD criteria were excluded. Information was from charts, ECG/Holter ECG and/or pacemaker/ implantable cardioverter defibrillator (ICD) data. SCD, sustained VT>30 sec and/or ventricular fibrillation necessitating an ICD-shock were considered major events. Event probability was calculated using the Kaplan-Meier estimator throughout the follow-up period of 20.7 years.

Results: There were 82 pts (61% males), mean age at surgery 62±14 years. Bileaflet MVP was present in 54%, mitral annular dysjunction (MAD) in 37% and left ventricular ejection fraction (LVEF) <50% in 12%. MV repair included ring annuloplasty in all and artificial chords in 48%. Mean follow-up was 3.1 years (0.2 to 14.2 years). Postoperative rhythm surveillance by Holter ECG and/or pacemaker was available in 67%. A VA load of ≥10% and/or any VT was noted in 26% before and 32% after surgery (P=0.44). Postoperative VA load was not predicted by MAD, artificial chords, LVEF of <50%, age at surgery >50 years and/or residual ≥moderate MR (all P<0.05), it correlated only with bileaflet MVP (P=0.009). Major events occurred in 3 pts: SCD in 2 pts and ICD for sustained polymorphic VT in 1 pt (incidence 1.2%/year). The event probability of receiving a SCD or an ICD-shock was 4.9%.

Conclusions: VA burden does not seem to change after MV repair in MVP due to BD. The occurrence of major arrhythmic events can not be predicted reliably, thus, patients with MVP due to BD may need lifelong postoperative follow-up, especially in bileaflet MVP which was an independent risk factor for increased VA burden in this retrospective long-term study in a small but well selected patient group.