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Characterization of the Electrophysiological Substrate in Patients with Barlow's Disease
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  • Pasquale Vergara,
  • Iside Scarfò,
  • Antonio Esposito,
  • Caterina Colantoni,
  • Anna Palmisano,
  • Savino Altizio,
  • Giulio Falasconi,
  • Luigi Pannone,
  • Elisabetta Lapenna,
  • Simone Gulletta,
  • Ottavio Alfieri,
  • Alessandro Castiglioni,
  • Francesco Maisano,
  • Michele De Bonis,
  • Paolo Della Bella,
  • Giovanni La Canna
Pasquale Vergara
Ospedale San Raffaele
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Iside Scarfò
Humanitas Group
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Antonio Esposito
San Raffaele Hospital
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Caterina Colantoni
San Raffaele Hospital
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Anna Palmisano
San Raffaele Hospital
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Savino Altizio
Ospedale San Raffaele
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Giulio Falasconi
San Raffaele Hospital
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Luigi Pannone
Ospedale San Raffaele
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Elisabetta Lapenna
San Raffaele Hospital
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Simone Gulletta
Ospedale San Raffaele
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Ottavio Alfieri
San Raffaele Hospital
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Alessandro Castiglioni
San Raffaele Hospital
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Francesco Maisano
San Raffaele Hospital
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Michele De Bonis
San Raffaele Hospital
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Paolo Della Bella
San Raffaele Hospital
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Giovanni La Canna
San Raffaele Hospital
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Abstract

BACKGROUND. Myxomatous mitral valve prolapse (MVP) and mitral-annular disjunction (Barlow disease) are at-risk for ventricular arrhythmias (VA). Fibrosis involving the papillary muscles and/or the infero-basal left ventricular (LV) wall was reported at autopsy in sudden cardiac death (SCD) patients with MVP. METHODS AND RESULTS: Twenty-three patients with VA were enrolled, including five with syncope and four with a history of SCD. Electrophysiological parameters were correlated with VA patterns, ECG inferior negative T wave (nTW), and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance. Premature ventricular complex (PVC) burden was 12061.9±12994.6 /24 hours with a papillary-muscle type (PM-PVC) in 18 patients (68%). Twelve-lead ECG showed nTW in 12 patients (43.5%). A large Uni<8.3mV area (62.4+/- 45.5 cm2) was detected in the basal infero-lateral LV region in 12 (73%) patients, and in the papillary muscles (2.2+/-2.9 cm2) in 5 (30%) of 15 patients undergoing EAM. A concomitant Bi<1.5 mV area (5.0±1.0 cm2) was identified in 2 patients. A history of SCD, and the presence of nTW, and LGE were associated with a greater Uni<8.3mV extension: (32.8+/-3.1 cm2 vs. 9.2+/-8.7 cm2), nTW (20.1+/-11.0vs.4.1+/-3.8cm2), and LGE (19.2 ± 11.7 cm2 vs. 1.0 ± 2.0 cm2, p=0.013), respectively. All patients with PM-PVC had a Uni<8.3mV area. CONCLUSIONS: Low unipolar low voltage areas can be identified with EAM in the basal infero-lateral LV region and in the papillary muscles as a potential electrophysiological substrate for VA and SCD in patients with MVP and Barlow disease phenotype

Peer review status:IN REVISION

24 Jun 2021Submitted to Journal of Cardiovascular Electrophysiology
02 Jul 2021Assigned to Editor
02 Jul 2021Submission Checks Completed
06 Jul 2021Reviewer(s) Assigned
25 Jul 2021Review(s) Completed, Editorial Evaluation Pending
30 Jul 2021Editorial Decision: Revise Minor