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New Kid on the Block: Double-Peak Strain Pattern in Arrhythmic Mitral Valve Prolapse

2023; Lippincott Williams & Wilkins; Volume: 16; Issue: 4 Linguagem: Inglês

10.1161/circimaging.123.015397

1942-0080

Patrizio Lancellotti, Yun Yun Go,

Cardiovascular Function and Risk Factors

HomeCirculation: Cardiovascular ImagingVol. 16, No. 4New Kid on the Block: Double-Peak Strain Pattern in Arrhythmic Mitral Valve Prolapse Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessEditorialPDF/EPUBNew Kid on the Block: Double-Peak Strain Pattern in Arrhythmic Mitral Valve Prolapse Patrizio Lancellotti and Yun Yun Go Patrizio LancellottiPatrizio Lancellotti Correspondence to: Patrizio Lancellotti, MD, PhD, Department of Cardiology, GIGA Cardiovascular Sciences, University of Liège Hospital, Domaine Universitaire du Sart Tilman, B.35, 4000 Liège, Belgium. Email E-mail Address: [email protected] https://orcid.org/0000-0002-0804-8194 Department of Cardiology, University of Liège Hospital, GIGA Cardiovascular Sciences, Heart Valve Clinic, CHU Sart Tilman, Liège, Belgium (P.L.). Division of Cardiovascular Surgery and Research, Gruppo Villa Maria Care and Research, Maria Cecilia Hospital, Cotignola, Italy (P.L.). Anthea Hospital, Bari, Italy (P.L.). and Yun Yun GoYun Yun Go National Heart Research Institute Singapore, National Heart Centre Singapore (Y.Y.G.). Originally published18 Apr 2023https://doi.org/10.1161/CIRCIMAGING.123.015397Circulation: Cardiovascular Imaging. 2023;16This article is a commentary on the followingAbnormal Mechanics Relate to Myocardial Fibrosis and Ventricular Arrhythmias in Patients With Mitral Valve ProlapseMitral valve prolapse (MVP) is the most common valvular heart disease that affects 1% to 3% of the general population. Most patients are asymptomatic with good long-term prognosis. Arrhythmic MVP, defined as MVP in the presence of frequent or complex ventricular arrhythmia (VA), is a rare feature (<1% per year) among MVP patients.1 However, it is overrepresented (up to 7%) in patients with otherwise unexplained sudden cardiac death on autopsy series.2See Article by Nagata and Bertrand et alA typical arrhythmic MVP phenotype would be that of a young female patient with inferior T-wave inversion on ECG, bileaflet prolapsed, and mitral annular disjunction (MAD) on echocardiogram. Other high-risk echocardiographic features that have been suggested include, but are not limited to, the Pickelhaube sign (lateral annular velocity spike of ≥16 cm/s), systolic curling of the posterior wall, marked billowing excursion, mechanical dispersion, and postsystolic shortening.3 The presence of fibrosis on cardiac magnetic resonance (CMR), either in the form of replacement fibrosis on late gadolinium sequences or interstitial fibrosis on T1 mapping, and extracellular volume measurement has also been shown to predict malignant arrhythmias.4 Note that not all the aforementioned high-risk imaging parameters are included in the risk stratification framework of the recently published European Heart Rhythm Association expert consensus statement.1 In asymptomatic patients with MVP, high-risk imaging parameters that warrant further investigations are limited to MAD, redundant mitral leaflets, enlarged left atrium, left ventricular ejection fraction ≤50%, and the presence of late gadolinium enhancement (LGE).1The interaction between the morphology and function of MVP has long been a subject of interest. A common theme that runs through the morphofunctional characteristics of arrhythmic MVP, for example, MAD, systolic curling, and the Pickelhaube sign is a hypercontractile basal inferolateral segment that is subjected to mechanical stretch. Speckle-tracking echocardiography is well suited at assessing myocardial deformation in response to the vectors generated by various components of the mitral valve apparatus during the cardiac cycle.In this issue of Circulation: Cardiovascular Imaging, Nagata and Bertrand et al5 reported a novel double-peak pattern (DPP) on speckle-tracking echocardiography–derived longitudinal strain curves in patients with MVP. As the name implies, DPP is characterized by 2 distinct peaks. The first peak denotes normal systolic shortening in early systole. This is followed by paradoxical systolic expansion of the mitral annulus in late systole, tugging the basal posterior segment and causing it to lengthen in late systole. The second peak represents postsystolic shortening, as the stretched basal posterior segment recoils to release the tension that was built up earlier. In this study cohort of 113 patients with MVP, DPP was reported in slightly less than half, 47% (n=53) of the study population, mostly in those with fibrosis on CMR (n=35). DPP was also more frequently found in those with superior leaflet displacement, MAD, systolic curling, and papillary muscle displacement.These findings, specifically the postsystolic shortening, concur with that reported by Huttin et al in an earlier study. In the study by Huttin et al6 that included patients with MVP and normal LV ejection fraction, pathological early systolic shortening and postsystolic deformation was observed. The present article by Nagata et al offers further insights into the underlying mechanisms of the strain pattern and provides outcome correlation.Interestingly, this study has shown that DPP is an independent predictor of VAs, regardless of the presence of fibrosis. In fact, the effect of fibrosis on VAs was rendered statistically insignificant after adjusting for DPP (model 7 in Table 3 and Table S4B) or after adjusting for both DPP and history of VA (model 9 in Table 3 and Table S4B). Whether DPP in itself is arrhythmogenic still requires further validation in larger, prospective studies. However, this echoes previous observation that the pathogenesis of arrhythmias in some MVP patients is independent of replacement fibrosis detected by CMR as LGE. Studies have shown that arrhythmias can occur in patients without the evidence of LGE on CMR, for example, those with focal basal posterior hypertrophy or ballerina-foot pattern.7 VAs in the absence of LGE might be attributed to interstitial fibrosis or other disease processes, for example, inflammation or ischemia.8–10 Late gadolinium sequence strength lies in its ability to detect replacement fibrosis. However, it lacks sensitivity at detecting interstitial fibrosis compared with parametric mapping techniques, for example, T1 and extracellular volume. Interstitial fibrosis on parametric mapping has been associated with increased risk of cardiac arrest in the absence of LGE.4 Study using fluorine 18-labeled fluorodeoxyglucose positron emission tomography imaging in patients with MVP and ventricular ectopy showed metabolic activity, a surrogate for myocardial inflammation, or ischemia, which suggests ongoing occult disease activities beyond replacement fibrosis.11In the context of arrhythmia prediction, DPP adds to the expanding list of risk markers in patients with MVP. However, it is unclear whether DPP provides incremental prognostic values beyond the current established risk markers or whether DPP still has a role when all things are considered. Further, larger longitudinal outcome studies are required to better define the role of DPP in arrhythmic MVP risk stratification. Also, more data are required to make sense of the relationship between DPP and other imaging markers that are also a result of a hypercontractile inferolateral segment and whether they are indeed different angles that describe the same phenomenon.Nagata et al also reported decreased regional peak systolic longitudinal strain in the basal inferior and posterior segments, both in MVP patients with and without LGE. This contrasts previous study that reported supranormal peak systolic strain in the posterolateral trident.3 Nagata et al added that the areas of decreased regional longitudinal strain mirrored the distribution of fibrosis on CMR, thus the decreased longitudinal strain in patients with LGE. However, it does not explain the decreased regional longitudinal strain in patients without fibrosis. On the other hand, the supranormal peak systolic strain parallels the Pickelhaube sign, both the result of a hypercontractile basal posterior segment. Further studies are required to reconcile these contradictory findings.Risk stratification in arrhythmic MVP is challenging. A multitude of parameters, ranging from imaging to ECG to genetics to electrophysiologic study, have been associated with arrhythmias, although none conclusively and most limited by the size of the studies or the lack of longitudinal follow-up. Compared with hypertrophic cardiomyopathy that has similar annual incidence of sudden cardiac death, risk stratification in arrhythmic MVP is still in its infancy. The European Heart Rhythm Association expert consensus statement is the start of a long journey that calls for constant review of emerging literature and iterative refinement of the recommendations. There are still many gray areas, from screening to risk stratification to management, that could benefit from further guidance and clarification. One area would be the role of surgery in arrhythmic MVP. In patients with severe mitral regurgitation, the decision for surgery is relatively straightforward. However, in patients with less than severe mitral regurgitation, whether arrhythmic MVP lowers the threshold for surgery is unclear. Small studies have suggested that mitral valve repair may reduce the burden of malignant arrhythmias. However, surgery has not been shown to decrease the risk of sudden cardiac death among patients with MVP.12Another area that requires further guidance is the role of multimodality imaging in patients with suspected arrhythmic MVP. All patients in this study underwent CMR as part of the study entry criteria. Thus, the present study was not poised to answer the perennial question of who among the MVP patients should undergo a CMR study. The European Heart Rhythm Association expert consensus suggests that CMR may be useful in patients with MVP who have at least 1 phenotypic risk feature, namely palpitations, inferior lead T-wave inversion, polymorphic premature ventricular complexes, MAD, redundant mitral leaflets, enlarged left atrium, or ejection fraction ≤50%.1Arrhythmic MVP is a complex disease entity that has been much talked about but not fully understood or at least not just yet. Collaboration between the imagers, electrophysiologists, pathologists, and the surgeons is needed for us to stand the best chance at resolving the many uncertainties surrounding the most common valve disease in the community—the MVP (Figure).Download figureDownload PowerPointFigure. Features associated with arrhythmic mitral valve prolapse. ICD indicates implantable cardioverter-defibrillator; LA, left atrium; LGE, late gadolinium enhancement; LS, longitudinal strain; LVEF, left ventricular ejection fraction; MAD, mitral annular disjunction; MR, mitral regurgitation; MRI, magnetic resonance imaging; MV, mitral valve; PL, posterior-lateral; PVC, premature ventricular contraction; SCD, sudden cardiac death; TWI, T-wave inversion; and VT, ventricular tachycardia.Disclosures None.FootnotesFor Disclosures, see page 342.The opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.https://www.ahajournals.org/journal/circimagingCorrespondence to: Patrizio Lancellotti, MD, PhD, Department of Cardiology, GIGA Cardiovascular Sciences, University of Liège Hospital, Domaine Universitaire du Sart Tilman, B.35, 4000 Liège, Belgium. Email plancellotti@chuliege.beReferences1. Sabbag A, Essayagh B, Barrera JDR, Basso C, Berni A, Cosyns B, Deharo JC, Deneke T, Di Biase L, Enriquez-Sarano M, et al. EHRA expert consensus statement on arrhythmic mitral 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 by the Heart Rhythm Society, by the Asia Pacific Heart Rhythm Society, and by the Latin American Heart Rhythm Society.Europace. 2022; 24:1981–2003. doi: 10.1093/europace/euac125CrossrefMedlineGoogle Scholar2. Basso C, Perazzolo Marra M, Rizzo S, de Lazzari M, Giorgi B, Cipriani A, Frigo AC, Rigato I, Migliore F, Pilichou K, et al. Arrhythmic mitral valve prolapse and sudden cardiac death.Circulation. 2015; 132:556–566. doi: 10.1161/CIRCULATIONAHA.115.016291LinkGoogle Scholar3. Muthukumar L, Jahangir A, Jan MF, Perez Moreno AC, Khandheria BK, Tajik AJ. Association between malignant mitral valve prolapse and sudden cardiac death.JAMA Cardiol. 2020; 5:1053–1061. doi: 10.1001/jamacardio.2020.1412CrossrefMedlineGoogle Scholar4. Pavon AG, Arangalage D, Pascale P, Hugelshofer S, Rutz T, Porretta AP, Le Bloa M, Muller O, Pruvot E, Schwitter J, et al. Myocardial extracellular volume by T1 mapping: a new marker of arrhythmia in mitral valve prolapse.J Cardiovasc Magn Reson. 2021; 123:1–13.Google Scholar5. Nagata Y, Bertrand PB, Baliyan V, Kochav J, Kagan RD, Ujka K, Alfraidi H, van Kampen A, Morningstar JE, Dal-Bianco JP, et al. Abnormal mechanics relate to myocardial fibrosis and ventricular arrhythmias in patients with mitral valve prolapse.Circ Cardiovasc Imaging. 2023; 16:e014963. doi: 10.1161/CIRCIMAGING.122.014963LinkGoogle Scholar6. Huttin O, Pierre S, Venner C, Voilliot D, Sellal JM, Aliot E, Sadoul N, Juillière Y, Brembilla-Perrot B, Selton-Suty C. Interactions between mitral valve and left ventricle analysed by 2D speckle tracking in patients with mitral valve prolapse: one more piece to the puzzle.Eur Heart J Cardiovasc Imaging. 2017; 18:323–331. doi: 10.1093/ehjci/jew075CrossrefMedlineGoogle Scholar7. Zia MI, Valenti V, Cherston C, Criscito M, Uretsky S, Wolff S. Relation of mitral valve prolapse to basal left ventricular hypertrophy as determined by cardiac magnetic resonance imaging.Am J Cardiol. 2012; 109:1321–1325. doi: 10.1016/j.amjcard.2011.12.029CrossrefMedlineGoogle Scholar8. Garbi M, Lancellotti P, Sheppard MN. Mitral valve and left ventricular features in malignant mitral valve prolapse.Open Heart. 2018; 5:e000925. doi: 10.1136/openhrt-2018-000925CrossrefMedlineGoogle Scholar9. Lancellotti P, Garbi M. Malignant mitral valve prolapse: substrates to ventricular remodeling and arrhythmias.Circ Cardiovasc Imaging. 2016; 9:e005248. doi: 10.1161/CIRCIMAGING.116.005248LinkGoogle Scholar10. Kubala M, de Chillou C, Bohbot Y, Lancellotti P, Enriquez-Sarano M, Tribouilloy C. Arrhythmias in patients with valvular heart disease: gaps in knowledge and the way forward.Front Cardiovasc Med. 2022; 9:1–19.CrossrefGoogle Scholar11. Miller MA, Adams DH, Pandis D, Robson PM, Pawale A, Pyzik R, Liao SL, El-Eshmawi A, Boateng P, Garg J, et al. Hybrid positron emission tomography/magnetic resonance imaging in arrhythmic mitral valve prolapse.JAMA Cardiol. 2020; 5:1000–1005. doi: 10.1001/jamacardio.2020.1555CrossrefMedlineGoogle Scholar12. Basso C, Iliceto S, Thiene G, Perazzolo Marra M. Mitral valve prolapse, ventricular arrhythmias, and sudden death.Circulation. 2019; 140:952–964. doi: 10.1161/CIRCULATIONAHA.118.034075LinkGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsRelated articlesAbnormal Mechanics Relate to Myocardial Fibrosis and Ventricular Arrhythmias in Patients With Mitral Valve ProlapseYasufumi Nagata, et al. Circulation: Cardiovascular Imaging. 2023;16 April 2023Vol 16, Issue 4 Advertisement Article InformationMetrics © 2023 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.123.015397PMID: 37071715 Originally publishedApril 18, 2023 KeywordsEditorialsfibrosismitral valve prolapseprognosisprolapsePDF download Advertisement SubjectsEchocardiographyMagnetic Resonance Imaging (MRI)Valvular Heart Disease