Sites of Successful Ventricular Fibrillation Ablation in Bileaflet Mitral Valve Prolapse Syndrome.
BACKGROUND
Although the vast majority of mitral valve prolapse (MVP) is benign, a small subset of patients, predominantly women, with bileaflet prolapse, complex ventricular ectopy (VE), and abnormal T waves comprise the recently described bileaflet MVP syndrome. We compared findings on electrophysiological study in bileaflet MVP syndrome patients with and without cardiac arrest to identify factors that may predispose to malignant ventricular arrhythmia.


RESULTS
Fourteen consecutive bileaflet MVP syndrome patients (n=13 women; median [limits], age at index ablation, 33.8 [21.0-58.7] years; ejection fraction, 60% [45%-67%]; all ≤ moderate mitral regurgitation; n=6 with previous cardiac arrest and implantable cardioverter defibrillator shocks for ventricular fibrillation; and n=8 without implantable cardioverter defibrillator although with symptomatic complex VE) were included. The 2 groups had similar baseline echocardiographic and electrocardiographic characteristics. All patients had at least 1 left ventricular papillary or fascicular VE focus. Purkinje origin VE was identified as the ventricular fibrillation trigger in 6 of 6 cardiac arrest patients (4 from papillary muscle) and Purkinje origin of dominant VE was seen in 5 of 8 (3 from papillary muscle) nonarrest patients. Acute success was seen in 17 of 19 procedures, and a ventricular fibrillation storm occurred within 24 hours of ablation in a single patient. Repeat ablation for recurrent symptomatic arrhythmia was performed in 6 patients. At 478 (39-2099) days of follow-up, 2 cardiac arrest patients received appropriate shocks. Symptoms from VE were reduced in 12 of 14.


CONCLUSIONS
Bileaflet MVP syndrome is characterized by fascicular and papillary muscle VE that triggers ventricular fibrillation. Ablation of clinically dominant VE foci improves symptoms and reduces appropriate implantable cardioverter defibrillator shocks.
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Faisal F
FF
Syed
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN. ackerman.michael@mayo.edu.
Michael J
MJ
Ackerman
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Christopher J
CJ
McLeod
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Suraj
S
Kapa
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Siva K
SK
Mulpuru
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Chenni S
CS
Sriram
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Bryan C
BC
Cannon
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Samuel J
SJ
Asirvatham
From the Division of Cardiovascular Diseases/Department of Medicine (F.F.S., M.J.A., C.J.M., S.K., S.K.M., C.S.S., B.C.C., S.J.A., P.A.N.), Windland Smith Rice Sudden Death Genomics Laboratory/Department of Molecular Pharmacology & Experimental Therapeutics (M.J.A.), and Division of Pediatric Cardiology/Department of Pediatrics (B.C.C., S.J.A.), Mayo Clinic, Rochester, MN.
Peter A
PA
Noseworthy
D000328:Adult; D017115:Catheter Ablation / Q000379:methods*; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006329:Heart Conduction System / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D008945:Mitral Valve Prolapse / Q000150:complications* / Q000175:diagnosis; D012189:Retrospective Studies; D013577:Syndrome; D016896:Treatment Outcome; D014693:Ventricular Fibrillation / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D055815:Young Adult
catheter ablation; death, sudden; heart arrest; mitral valve; papillary muscles; ventricular fibrillation