A 35-year-old man was referred for cardiac check-up because of
suspected reflex syncope. He underwent a tilt-test challenge without
syncope provocation. The electrocardiography (ECG) showed sinus
rhythm with polymorphic premature ventricular contractions (PVC),
with right bundle branch block morpholog

Larrea-Iñarra, A. (Ander)

Martinez-de-Miguel, S. (Sara)

Riesgo-Garcia, A. (Alvaro)

English

Dadun, University of Navarra

European Heart Journal - Case Reports (2023) 7, 1–2 https://doi.org/10.1093/ehjcr/ytad033ECG CHALLENGE ElectrophysiologyECG, an essential tool to guide arrhythmic mitral valve prolapse diagnosisAlvaro Riesgo Garcia *, Ander Larrea Iñarra, and Sara Martinez de MiguelDepartment of Cardiology, Electrophysiology Unit, Clínica Universidad de Navarra, Avenida Pío XII 36, 31008 Pamplona, SpainReceived 9 December 2022; first decision 19 December 2022; accepted 12 January 2023; online publish-ahead-of-print 1 February 2023* Corresponding author. Tel: +34633423991, +34948255400, Fax: +34948296500, Email: ariesgo@unav.es.Handling Editor: Stefano Bordignon© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.comA 35-year-old man was referred for cardiac check-up because of suspected reflex syncope. He underwent a tilt-test challenge without syncope provocation. The electrocardiography (ECG) showed sinus rhythm with polymorphic premature ventricular contractions (PVC), with right bundle branch block morphology (Figure 1). There were T-wave inversions with terminal notch of the QRS in inferior leads. An echocardiogram was performed and it showed bileaflet mitral valve prolapse with moderate regurgitation. Left atrium was mildly dilated and left ventricular ejection fraction was preserved. Afterwards, the pa-tient underwent prolonged Holter monitoring with frequent pleo-morphic PVCs (7%), and three non-sustained fast (>180 beats per minute) runs of monomorphic ventricular tachycardia. Cardiac magnet-ic resonance was performed showing mitral annular disjunction, and late gadolinium enhancement in posteromedial papillary muscle, and Figure 1. Baseline electrocardiogram.Downloaded from https://academic.oup.com/ehjcr/article/7/3/ytad033/7019416 by Universidad de Navarra. Servicio de Bibliotecas user on 24 June 20232                                                                                                                                                                                               A.R. Garcia et al.all over mitral annulus. Diagnosis of arrhythmic mitral valve prolapse was done. Implantable cardioverter defibrillator (ICD) was proposed because high-risk non-sustained ventricular tachycardia (NSVT) finding in Holter, but it was rejected by the patient. The patient accepted implantable loop recorder (ILR) implantation, and began treatment with low-dose beta-blockers, with future uptitration depending on tol-erance. ICD would be considered depending on the ILR findings. Electrocardiogram is an essential tool which can suggest the diagnosis even in the absence of cardiac imaging. ECG is might!Multiple-choice questions 1. The site of origin of the two different PVC seen in the ECG are most consistent with: a. Anterior mitral annulus and posteromedial papillary muscle.b. Posterior mitral annulus and left ventricular apical wall.c. Anterolateral papillary muscle and posterior mitral annulus.d. Anterolateral papillary muscle and posteromedial papillary muscle.e. Posterior mitral annulus and posteromedial papillary muscle.Answer: The first PVC corresponding to the second beat in the ECG shows right bundle branch block morphology, with rS transition in V3, and a rightward axis, suggestive of site of origin in anterolateral papillary muscle. The second PVC (corresponding for instance to the fourth beat of the ECG) shows right bundle branch block morphology with positive concordance in precordial leads, and leftward axis, suggesting a poster-ior mitral annulus site of origin. 2. Which one of the following is not required for the diagnosis of arrhythmic mitral valve prolapse? a. Mitral annular disjunction.b. >5% of total PVC burden.c. The absence of any other well-defined arrhythmic substrate.d. Complex ventricular arrhythmia.e. Mitral valve prolapse.Answer: According to the European Heart Rhythm Association (EHRA) expert consensus on arrhythmic mitral valve prolapse1, the diagnosis criteria are the presence of mitral valve prolapse, frequent burden of PVC (>5%) in Holter monitoring, the presence of complex arrhythmia like NSVT, sustained ventricular tachycardia, or ventricular fibrillation triggered by PVC. The absence of any other well-defined ar-rhythmic substrate is also necessary. However, the presence of mitral annular disjunction2 is not a main criterion for the diagnosis of this entity. 3. According to EHRA expert consensus on arrhythmic mitral valve pro-lapse, which one of the following sentences is not correct? a. Cardiac magnetic resonance (CMR) should be performed in all MVP pa-tients with a history of unexplained syncope.b. ILR may be advised in patients with mitral valve prolapse (MVP) and un-explained syncope in whom non-invasive ECG monitoring was not re-vealing or inconclusive.c. All MVP patients with high-risk ventricular tachycardia (VT) episodes in Holter monitoring should be considered for ICD implantation.d. Reduced left ventricular ejection fraction is the most im-portant echocardiographic predictor of arrhythmias. The progression of mitral valve disease has no role in predicting sudden cardiac death.e. In MVP patients, ablation of PVCs is reasonable if triggering VF, particu-larly if not controlled by medications.Answer: According to the last expert consensus on arrhythmic mitral valve prolapse, CMR must be performed in all patients that have suf-fered unexplained syncope, and implantable loop recorder is advised when prolonged ECG monitoring is not revealing. If there are findings of high-risk VT features, patients should be considered for ICD primary prevention implantation. If there is VF reproducibly triggered by a PVC, the patient should be referred to PVC ablation.3 Although, reduced LVEF is predictor of complex arrhythmias in this entity, the progression of mitral valve disease has a key role in increasing sudden cardiac death risk.Supplementary materialSupplementary material is available at European Heart Journal – Case Reports.Consent: The authors confirm that written consent for submission and publication of this case report including images and associated text has been obtained from the patient in line with COPE guidance.Conflict of interest: None declared.Funding: None declared.References1. Sabbag A, Essayagh B, Ramírez Barrera JD, Basso C, Berni A, Cosyns B, Deharo JC, Deneke T, Di Biase L, Enriquez-Sarano M, Donal E, Imai K, Lim HS, Marsan NA, Turagam MK, Peichl P, Po SS, Haugaa KH, Shah D, de Riva Silva M, Bertrand P, Saba M, Dweck M, Townsend SN, Ngarmukos T, Felenon G, Santangeli P, Sade LE, Corrado D, Lambiase P, Sanders P, Delacrétaz E, Jahangir A, Kaufman ES, Saggu DK, Pierard L, Delgado V, Lancellotti P. EHRA expert consensus statement on arrhythmic mi-tral valve prolapse and mitral annular disjunction complex in collaboration with the ESC Council on valvular heart disease and the European Association of Cardiovascular Imaging endorsed cby the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society. Europace 2022;24:1981–2003.2. Basso C, Iliceto S, Thiene G, Perazzolo Marra M. Mitral valve prolapse, ventricular ar-rhythmias, and sudden death. Circulation 2019;140:952–964.3. Dejgaard LA, Skjølsvik Eystein ET, Lie ØH, Ribe M, Stokke MK, Hegbom F, ScheIrlynck ES, Gjertsen E, Andresen K, Helle-Valle TM, Hopp E, Edvardsen T, Haugaa KH. The mitral annulus disjunction arrhythmic syndrome. J Am Coll Cardiol 2018;72:1600–1609.Downloaded from https://academic.oup.com/ehjcr/article/7/3/ytad033/7019416 by Universidad de Navarra. Servicio de Bibliotecas user on 24 June 2023

ECG, an essential tool to guide arrhythmic mitral valve prolapse diagnosis

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ECG, an essential tool to guide arrhythmic mitral valve prolapse diagnosis

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