FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W3136450069> ?p ?o ?g. }

• W3136450069 abstract "HomeCirculation: Cardiovascular ImagingVol. 14, No. 3Left Ventricular Pathology in Ebstein’s Anomaly—Myocardium in Motion Free AccessEditorialPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyRedditDiggEmail Jump toFree AccessEditorialPDF/EPUBLeft Ventricular Pathology in Ebstein’s Anomaly—Myocardium in MotionCMR Insights Into Left Ventricular Fibrosis, Deformation, and Exercise Capacity Michael Steinmetz, MD Andreas SchusterMD, PhD, MBA Michael SteinmetzMichael Steinmetz Michael Steinmetz, MD, Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University, Robert-Koch-Str. 40, 37075 Goettingen, Germany. Email E-mail Address: [email protected] https://orcid.org/0000-0001-5541-1147 Department of Pediatric Cardiology and Intensive Care Medicine (M.S.), University Medical Center, Georg-August-University, Goettingen, Germany. Department of Cardiology and Pneumology (A.S.), University Medical Center, Georg-August-University, Goettingen, Germany. Search for more papers by this author and Andreas SchusterAndreas Schuster https://orcid.org/0000-0003-1508-1125 Department of Cardiology and Pneumology (A.S.), University Medical Center, Georg-August-University, Goettingen, Germany. DZHK, German Center for Cardiovascular Research (DZHK), partner site Goettingen (M.S., A.S.). Search for more papers by this author Originally published16 Mar 2021https://doi.org/10.1161/CIRCIMAGING.121.012285Circulation: Cardiovascular Imaging. 2021;14:e012285This article is a commentary on the followingMyocardial Fibrosis in Pediatric Patients With Ebstein’s AnomalySee Article by Aly et alEbstein’s anomaly (EA) of the tricuspid valve is a rare congenital cardiac malformation accounting for only 0.5% of all congenital heart disease. It comprises a dysplastic tricuspid valve offset towards the apex, an atrialization of the right ventricle and tricuspid regurgitation. Progressive right heart failure and impaired physical exercise capacity occur in many patients with EA at some stage in their life, which are predominantly associated with abnormal right heart function and morphology.However, left ventricular (LV) alterations appear to be involved in heart failure in EA, as well.1 The left heart has been subject of much less attention as compared with the right heart in EA. Nevertheless, previous studies demonstrated impaired deformation of the LV,2 including the septum1 and the left atrium3 as well as LV dyssynchrony.4,5 All of the latter were also associated with heart failure markers, which suggests direct implications on the clinical course. Cone repair of the tricuspid valve seems to be an effective treatment and evidence suggests that the beneficial effects may be also based on improved basal septal deformation and synchrony of the LV, as detected by cardiovascular magnetic resonance (CMR)6 or echocardiography.7Since the surgical treatment option is mainly reserved for severe or later stages of the disease a better understanding of early pathophysiological mechanisms with a potential for preventive actions is highly desirable. However, the pathogen on a tissue level that may underlie these early alterations especially in LV function and later deterioration of overall cardiac function is still unknown.The study by Aly et al8 published in this issue of Circulation Cardiovascular Imaging helps to shed new light on this enigma in patients with EA by looking at markers of fibrosis and deformation.It touches on an interesting topic, the nature of LV dysfunction in unrepaired EA. The authors studied a group of 12 unrepaired pediatric patients with EA retrospectively with regard to CMR markers of fibrosis, strain and volumes, different severity indices and exercise capacity on cardiopulmonary exercise testing as well as oxygen saturation. The EA group is compared with a group of supposedly healthy controls who had undergone CMR for exclusion of chest pain or ARVC and had no phenotypic disease. Aly et al demonstrate the following new findings:Patients with EA exhibited higher LV T1 and extracellular volume values: (1026±47 versus 956±40 ms, P=0.0004 and 28.5±3.4% versus 22.5±2.6%, P<0.001, respectively).LV deformation parameters were decreased in patients with EA: circumferential strain and strain rate (−22±12% versus -27±14%, P<0.001 and 4.5±2.7%/s versus 6.5±3.2%/s, P<0.001, respectively) and radial strain and strain rate (39±19% versus 45±8%, P=0.003, and 9±5%/s versus 14±11%/s, P=0.01, respectively).Higher extracellular volume and T1 values were associated with age, higher severity index values, lower exercise capacity (VO2 max.), lower resting oxygen saturation (SaO2), and lower circumferential strain.The authors are to be commended to have focused their attention on the normally rather overlooked LV in a right heart malformation.Fibrotic changes of both ventricles in EA have been reported previously—before the times of routine CMR—in pathology studies.9 Using T1 mapping, CMR can now supply surrogate markers for histologically assessed fibrosis of the heart.10 With this technique, myocardial fibrosis in EA has been found in CMR studies by different groups.11,12 Ciepłucha et al11 focused on LGE and fibrosis in the obviously malformed right heart and did not report any association with exercise capacity in patients with EA. However, Yang et al12 have described myocardial fibrosis in the LV of patients with EA. They showed an association with right heart volume, LV EF, disease severity (ie, right/left volume index), hematocrit and New York Heart Association class, but did not look at exercise capacity or wall motion in their cohort.This is where Aly et al take up the thread and help us to understand, what LV fibrosis means for cardiac function and for the EA patients’ exercise capacity. They depict very much to the point the impact of fibrosis on LV myocardial deformation, that is, its correlation with impaired strain values and EF which in turn are the basis for impaired exercise capacity. Moreover, they discuss the origin of diffuse fibrosis: The groups hypothesis that a hypoxic stimulus may explain ventricular fibrosis. They attribute this stimulus to impaired pulmonary perfusion—the effect of which is also measured indirectly by increased hematocrit values. This is in keeping with previous studies by Hoesch et al, who also reported the value of hematocrit in the evaluation of EA severity, clinical parameters of heart failure and exercise capacity.13The study by Aly et al opens up an interesting path to be followed further: Fibrosis—as detected by T1 mapping appears to be the basis for LV dysfunction—as expressed by decreased deformation markers from feature tracking. Are fibrosis and deformation 2 entities signaling different ends of cardiac deterioration14 that come together in patients with EA early due to the unique nature of the anomaly? The association with a hypoxic stimulus seems likely due to the correlation of T1 times and extracellular volume with SaO2. However, a direct link and mechanism behind this observation is still to be presented. This shortcoming of the presented study cannot be overcome in a pilot-study setting. Larger cohorts and—as one should not grow tired to say—multicentric Ebstein’s studies would be necessary to find out more about the pathophysiology of this extremely interesting association of hypoxic stimulus, fibrosis, and impaired LV deformation. In which EA patient at what time do increased LV fibrosis and decreased LV deformation occur and are they associated with EA severity? It is furthermore extremely interesting to speculate whether or not subtle changes could be detected at an early stage and be improved by medical therapy increasing pulmonary perfusion including antipulmonary hypertensive agents such as sildenafil to prevent disease progression.15 Conversely, CMR may help us with risk stratification of patients and the decision for tricuspid valve surgery at later disease stages.As shown previously in patients with hypertrophic cardiomyopathy,16 advanced CMR studies using Blood Oxygen Level Dependent (BOLD) sequences to investigate tissue oxygen content and its impact on fibrosis may even better determine the aforementioned changes in EA. What is also missing in the study by Aly et al. is a look at diastolic LV function in EA. Do early fibrotic changes impact diastolic function and may in turn determine exercise capacity? In adult heart failure with preserved ejection fraction patients, impaired LA function, which is interlinked with ventricular diastolic function, has been shown to be the best predictor of exercise capacity independent of invasively determined ventricular relaxation and stiffness.17 Whether this may precede or aggravate the changes in LV function and fibrosis in patients with EA as observed by Aly et al will need to be determined.What else can we learn from the study: First, let us not forget the LV in right heart congenital heart disease! Second, ejection fraction and volumes may sometimes not be enough to tell the whole story; let us also look at changes in tissue composition and morphology as well as myocardial deformation.14 T1 mapping and feature tracking myocardial deformation parameters are able to detect structural and functional impairment earlier and predict outcome more accurately as previously and repeatedly reported in adult and congenital heart disease.14,18–20 Why not explore and use these valuable markers further in rare congenital malformations such as EA, too?A new door has been opened by the Aly study: It should be of interest to physicians and researchers concerned with Ebstein’s patients to follow the notion of LV fibrosis and impaired deformation in further studies. Possibly, novel therapeutic ideas such as improving pulmonary perfusion pharmacologically might be pursued in this context to prevent LV fibrosis and strain based deterioration. Let us not hesitate to use these newer techniques in clinical settings whenever possible.Sources of FundingNone.Disclosures None.FootnotesThe opinions expressed in this article are not necessarily those of the editors or of the American Heart Association.Michael Steinmetz, MD, Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University, Robert-Koch-Str. 40, 37075 Goettingen, Germany. Email michael.[email protected]uni-goettingen.deReferences1. Goleski PJ, Sheehan FH, Chen SS, Kilner PJ, Gatzoulis MA. The shape and function of the left ventricle in Ebstein’s anomaly.Int J Cardiol. 2014; 171:404–412. doi: 10.1016/j.ijcard.2013.12.037CrossrefMedlineGoogle Scholar2. Liu X, Zhang Q, Yang ZG, Shi K, Xu HY, Xie LJ, Jiang L, Diao KY, Guo YK. Assessment of left ventricular deformation in patients with Ebstein’s anomaly by cardiac magnetic resonance tissue tracking.Eur J Radiol. 2017; 89:20–26. doi: 10.1016/j.ejrad.2017.01.013CrossrefMedlineGoogle Scholar3. Steinmetz M, Broder M, Hösch O, Lamata P, Kutty S, Kowallick JT, Staab W, Ritter CO, Hasenfuß G, Paul T, et al.. Atrio-ventricular deformation and heart failure in Ebstein’s anomaly - a cardiovascular magnetic resonance study.Int J Cardiol. 2018; 257:54–61. doi: 10.1016/j.ijcard.2017.11.097CrossrefMedlineGoogle Scholar4. Park SJ, Chung S, On YK, Kim JS, Yang JH, Jun TG, Jang SY, Lee OJ, Song J, Kang IS, et al.. Fragmented QRS complex in adult patients with Ebstein anomaly and its association with arrhythmic risk and the severity of the anomaly.Circ Arrhythm Electrophysiol. 2013; 6:1148–1155. doi: 10.1161/CIRCEP.113.000636LinkGoogle Scholar5. Steinmetz M, Usenbenz S, Kowallick JT, Hösch O, Staab W, Lange T, Kutty S, Lotz J, Hasenfuß G, Paul T, et al.. Left ventricular synchrony, torsion, and recoil mechanics in Ebstein’s anomaly: insights from cardiovascular magnetic resonance.J Cardiovasc Magn Reson. 2017; 19:101. doi: 10.1186/s12968-017-0414-yCrossrefMedlineGoogle Scholar6. Beroukhim RS, Jing L, Harrild DM, Fornwalt BK, Mejia-Spiegeler A, Rhodes J, Emani S, Powell AJ. Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein anomaly: a cardiovascular magnetic resonance study.J Cardiovasc Magn Reson. 2018; 20:32. doi: 10.1186/s12968-018-0452-0CrossrefMedlineGoogle Scholar7. Perdreau E, Tsang V, Hughes ML, Ibrahim M, Kataria S, Janagarajan K, Iriart X, Khambadkone S, Marek J. Change in biventricular function after cone reconstruction of Ebstein’s anomaly: an echocardiographic study.Eur Heart J Cardiovasc Imaging. 2018; 19:808–815. doi: 10.1093/ehjci/jex186CrossrefMedlineGoogle Scholar8. Aly S, Seed M, Yoo SJ, Lam C, Lars GW. Myocardial fibrosis in pediatric patients with Ebstein’s anomaly.Circ Cardiovasc Imaging. 2021; 14:e011136. doi: 10.1161/CIRCIMAGING.120.011136LinkGoogle Scholar9. Celermajer DS, Dodd SM, Greenwald SE, Wyse RK, Deanfield JE. Morbid anatomy in neonates with Ebstein’s anomaly of the tricuspid valve: pathophysiologic and clinical implications.J Am Coll Cardiol. 1992; 19:1049–1053. doi: 10.1016/0735-1097(92)90293-vCrossrefMedlineGoogle Scholar10. Backhaus SJ, Lange T, Beuthner BE, Topci R, Wang X, Kowallick JT, Lotz J, Seidler T, Toischer K, Zeisberg EM, et al.. Real-time cardiovascular magnetic resonance T1 and extracellular volume fraction mapping for tissue characterisation in aortic stenosis.J Cardiovasc Magn Reson. 2020; 22:46. doi: 10.1186/s12968-020-00632-0CrossrefMedlineGoogle Scholar11. Ciepłucha A, Trojnarska O, Kociemba A, Łanocha M, Barczynski M, Rozmiarek S, Kramer L, Pyda M. Clinical aspects of myocardial fibrosis in adults with Ebstein’s anomaly.Heart Vessels. 2018; 33:1076–1085. doi: 10.1007/s00380-018-1141-5CrossrefMedlineGoogle Scholar12. Yang D, Li X, Sun JY, Cheng W, Greiser A, Zhang TJ, Liu H, Wan K, Luo Y, An Q, et al.. Cardiovascular magnetic resonance evidence of myocardial fibrosis and its clinical significance in adolescent and adult patients with Ebstein’s anomaly.J Cardiovasc Magn Reson. 2018; 20:69. doi: 10.1186/s12968-018-0488-1CrossrefMedlineGoogle Scholar13. Hösch O, Ngyuen TT, Lauerer P, Schuster A, Kutty S, Staab W, Unterberg-Buchwald C, Sohns JM, Paul T, Lotz J, et al.. BNP and haematological parameters are markers of severity of Ebstein’s anomaly: correlation with CMR and cardiopulmonary exercise testing.Eur Heart J Cardiovasc Imaging. 2015; 16:670–675. doi: 10.1093/ehjci/jeu312MedlineGoogle Scholar14. Fröjdh F, Fridman Y, Bering P, Sayeed A, Maanja M, Niklasson L, Olausson E, Pi H, Azeem A, Wong TC, et al.. Extracellular volume and global longitudinal strain both associate with outcomes but correlate minimally.JACC Cardiovasc Imaging. 2020; 13:2343–2354. doi: 10.1016/j.jcmg.2020.04.026CrossrefMedlineGoogle Scholar15. Pham P, Hoyer A, Shaughnessy R, Law YM. A novel approach incorporating sildenafil in the management of symptomatic neonates with Ebstein’s anomaly.Pediatr Cardiol. 2006; 27:614–617. doi: 10.1007/s00246-006-1203-9CrossrefMedlineGoogle Scholar16. Ando K, Nagao M, Watanabe E, Sakai A, Suzuki A, Nakao R, Ishizaki U, Sakai S, Hagiwara N. Association between myocardial hypoxia and fibrosis in hypertrophic cardiomyopathy: analysis by T2* BOLD and T1 mapping MRI.Eur Radiol. 2020; 30:4327–4336. doi: 10.1007/s00330-020-06779-9CrossrefMedlineGoogle Scholar17. von Roeder M, Rommel KP, Kowallick JT, Blazek S, Besler C, Fengler K, Lotz J, Hasenfuss G, Lucke C, Gutberlet M, et al.. Influence of left atrial function on exercise capacity and left ventricular function in patients with heart failure and preserved ejection fraction.Circ Cardiovasc Imaging. 2017; 10:e005467. doi: 10.1161/CIRCIMAGING.116.005467LinkGoogle Scholar18. Schuster A, Hor KN, Kowallick JT, Beerbaum P, Kutty S. Cardiovascular magnetic resonance myocardial feature tracking: concepts and clinical applications.Circ Cardiovasc Imaging. 2016; 9:e004077. doi: 10.1161/CIRCIMAGING.115.004077LinkGoogle Scholar19. Eitel I, Stiermaier T, Lange T, Rommel KP, Koschalka A, Kowallick JT, Lotz J, Kutty S, Gutberlet M, Hasenfuß G, et al.. Cardiac magnetic resonance myocardial feature tracking for optimized prediction of cardiovascular events following myocardial infarction.JACC Cardiovasc Imaging. 2018; 11:1433–1444. doi: 10.1016/j.jcmg.2017.11.034CrossrefMedlineGoogle Scholar20. Orwat S, Diller GP, Kempny A, Radke R, Peters B, Kühne T, Boethig D, Gutberlet M, Dubowy KO, Beerbaum P, et al.; German Competence Network for Congenital Heart Defects Investigators. Myocardial deformation parameters predict outcome in patients with repaired tetralogy of Fallot.Heart. 2016; 102:209–215. doi: 10.1136/heartjnl-2015-308569CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsRelated articlesMyocardial Fibrosis in Pediatric Patients With Ebstein’s AnomalySafwat Aly, et al. Circulation: Cardiovascular Imaging. 2021;14 March 2021Vol 14, Issue 3Article InformationMetrics Download: 82 © 2021 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.121.012285PMID: 33722058 Originally publishedMarch 16, 2021 KeywordsfibrosisEbstein’s Anomalytricuspid valveexerciseCMREditorialsPDF download SubjectsCongenital Heart DiseaseContractile FunctionMyocardial BiologyFibrosisMagnetic Resonance Imaging (MRI)" @default.
• W3136450069 created "2021-03-29" @default.
• W3136450069 creator A5037173235 @default.
• W3136450069 creator A5067454034 @default.
• W3136450069 date "2021-03-01" @default.
• W3136450069 modified "2023-10-16" @default.
• W3136450069 title "Left Ventricular Pathology in Ebstein’s Anomaly—Myocardium in Motion" @default.
• W3136450069 cites W2040461747 @default.
• W3136450069 cites W2106121260 @default.
• W3136450069 cites W2146504286 @default.
• W3136450069 cites W2204818956 @default.
• W3136450069 cites W2546447965 @default.
• W3136450069 cites W2571764778 @default.
• W3136450069 cites W2740799920 @default.
• W3136450069 cites W2765719257 @default.
• W3136450069 cites W2774649350 @default.
• W3136450069 cites W2785678869 @default.
• W3136450069 cites W2790024577 @default.
• W3136450069 cites W2790074029 @default.
• W3136450069 cites W2807633283 @default.
• W3136450069 cites W2893503434 @default.
• W3136450069 cites W3013870362 @default.
• W3136450069 cites W3036442196 @default.
• W3136450069 cites W3036568020 @default.
• W3136450069 cites W3138854050 @default.
• W3136450069 cites W4245692967 @default.
• W3136450069 doi "https://doi.org/10.1161/circimaging.121.012285" @default.
• W3136450069 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/33722058" @default.
• W3136450069 hasPublicationYear "2021" @default.
• W3136450069 type Work @default.
• W3136450069 sameAs 3136450069 @default.
• W3136450069 citedByCount "1" @default.
• W3136450069 countsByYear W31364500692022 @default.
• W3136450069 crossrefType "journal-article" @default.
• W3136450069 hasAuthorship W3136450069A5037173235 @default.
• W3136450069 hasAuthorship W3136450069A5067454034 @default.
• W3136450069 hasBestOaLocation W31364500691 @default.
• W3136450069 hasConcept C121332964 @default.
• W3136450069 hasConcept C126322002 @default.
• W3136450069 hasConcept C12997251 @default.
• W3136450069 hasConcept C164705383 @default.
• W3136450069 hasConcept C26873012 @default.
• W3136450069 hasConcept C2778658428 @default.
• W3136450069 hasConcept C2779946567 @default.
• W3136450069 hasConcept C2910183347 @default.
• W3136450069 hasConcept C71924100 @default.
• W3136450069 hasConceptScore W3136450069C121332964 @default.
• W3136450069 hasConceptScore W3136450069C126322002 @default.
• W3136450069 hasConceptScore W3136450069C12997251 @default.
• W3136450069 hasConceptScore W3136450069C164705383 @default.
• W3136450069 hasConceptScore W3136450069C26873012 @default.
• W3136450069 hasConceptScore W3136450069C2778658428 @default.
• W3136450069 hasConceptScore W3136450069C2779946567 @default.
• W3136450069 hasConceptScore W3136450069C2910183347 @default.
• W3136450069 hasConceptScore W3136450069C71924100 @default.
• W3136450069 hasIssue "3" @default.
• W3136450069 hasLocation W31364500691 @default.
• W3136450069 hasOpenAccess W3136450069 @default.
• W3136450069 hasPrimaryLocation W31364500691 @default.
• W3136450069 hasRelatedWork W2012641352 @default.
• W3136450069 hasRelatedWork W2035044797 @default.
• W3136450069 hasRelatedWork W2149294284 @default.
• W3136450069 hasRelatedWork W2198135068 @default.
• W3136450069 hasRelatedWork W2370189922 @default.
• W3136450069 hasRelatedWork W2411180437 @default.
• W3136450069 hasRelatedWork W2789251001 @default.
• W3136450069 hasRelatedWork W3208290810 @default.
• W3136450069 hasRelatedWork W4322704030 @default.
• W3136450069 hasRelatedWork W2509168764 @default.
• W3136450069 hasVolume "14" @default.
• W3136450069 isParatext "false" @default.
• W3136450069 isRetracted "false" @default.
• W3136450069 magId "3136450069" @default.
• W3136450069 workType "article" @default.