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. Objectives: We investigated the electrophysiological substrate subtending VA in MVP patients with Barlow disease phenotype. Methods: Twenty-three patients with VA were enrolled, including five with syncope and four with a history of SCD. Unipolar (Uni < 8.3 mV) and bipolar (Bi < 1.5 mV) low-voltage areas were analyzed with electro-anatomical mapping (EAM), and VA inducibility was evaluated with programmed ventricular stimulation (PES). Electrophysiological parameters were correlated with VA patterns, electrocardiogram (ECG) inferior negative T wave (nTW), and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance. Results: Premature ventricular complex (PVC) burden was 12 061.9 ± 12 994.6/24 h with a papillary-muscle type (PM-PVC) in 18 patients (68%). Twelve-lead ECG showed nTW in 12 patients (43.5%). A large Uni less than 8.3 mV 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 less than 1.5 mV area (5.0 ± 1.0 cm2) was identified in two patients. A history of SCD, and the presence of nTW, and LGE were associated with a greater Uni less than 8.3 mV extension: (32.8 ± 3.1 cm2 vs. 9.2 ± 8.7 cm2), nTW (20.1 ± 11.0 vs. 4.1 ± 3.8 cm2), and LGE (19.2 ± 11.7 cm2 vs. 1.0 ± 2.0 cm2, p =.013), respectively. All patients with PM-PVC had a Uni less than 8.3 mV area. Sustained VA (ventricular tachycardia 2 and VF 2) were induced by PES only in four patients (one with resuscitated SCD). Conclusions: Low unipolar low voltage areas can be identified with EAM in the basal inferolateral LV region and in the papillary muscles as a potential electrophysiological substrate for VA and SCD in patients with MVP and Barlow disease phenotype.
Characterization of the electrophysiological substrate in patients with Barlow's disease / Vergara, P.; Scarfo, I.; Esposito, A.; Colantoni, C.; Palmisano, A.; Altizio, S.; Falasconi, G.; Pannone, L.; Lapenna, E.; Gulletta, S.; Alfieri, O.; Castiglioni, A.; Maisano, F.; De Bonis, M.; Della Bella, P.; La Canna, G.. - In: JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY. - ISSN 1045-3873. - 32:12(2021), pp. 3179-3186. [10.1111/jce.15270]
Characterization of the electrophysiological substrate in patients with Barlow's disease
Esposito A.;Palmisano A.;Altizio S.;Falasconi G.;Pannone L.;Alfieri O.;Castiglioni A.;Maisano F.;De Bonis M.;
2021-01-01
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. Objectives: We investigated the electrophysiological substrate subtending VA in MVP patients with Barlow disease phenotype. Methods: Twenty-three patients with VA were enrolled, including five with syncope and four with a history of SCD. Unipolar (Uni < 8.3 mV) and bipolar (Bi < 1.5 mV) low-voltage areas were analyzed with electro-anatomical mapping (EAM), and VA inducibility was evaluated with programmed ventricular stimulation (PES). Electrophysiological parameters were correlated with VA patterns, electrocardiogram (ECG) inferior negative T wave (nTW), and late gadolinium enhancement (LGE) assessed by cardiac magnetic resonance. Results: Premature ventricular complex (PVC) burden was 12 061.9 ± 12 994.6/24 h with a papillary-muscle type (PM-PVC) in 18 patients (68%). Twelve-lead ECG showed nTW in 12 patients (43.5%). A large Uni less than 8.3 mV 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 less than 1.5 mV area (5.0 ± 1.0 cm2) was identified in two patients. A history of SCD, and the presence of nTW, and LGE were associated with a greater Uni less than 8.3 mV extension: (32.8 ± 3.1 cm2 vs. 9.2 ± 8.7 cm2), nTW (20.1 ± 11.0 vs. 4.1 ± 3.8 cm2), and LGE (19.2 ± 11.7 cm2 vs. 1.0 ± 2.0 cm2, p =.013), respectively. All patients with PM-PVC had a Uni less than 8.3 mV area. Sustained VA (ventricular tachycardia 2 and VF 2) were induced by PES only in four patients (one with resuscitated SCD). Conclusions: Low unipolar low voltage areas can be identified with EAM in the basal inferolateral LV region and in the papillary muscles as a potential electrophysiological substrate for VA and SCD in patients with MVP and Barlow disease phenotype.File | Dimensione | Formato | |
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Cardiovasc electrophysiol - 2021 - Vergara - Characterization of the electrophysiological substrate in patients with Barlow.pdf
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Mitrale_manuscript_2021-06-21_JCE_v2.pdf
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