{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T19:08:17Z","timestamp":1790881697081,"version":"4.1.0"},"reference-count":31,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T00:00:00Z","timestamp":1770336000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Chronic pulmonary regurgitation (PR) after the repair of tetralogy of Fallot (TOF) and other right ventricular outflow tract (RVOT) interventions leads to progressive right ventricular (RV) dilatation, altered ventricular\u2013ventricular interaction, and an increased risk of arrhythmia and heart failure. Pulmonary valve replacement (PVR), whether surgical or transcatheter, effectively eliminates or reduces PR and is associated with short- and mid-term improvement in RV size, symptoms, and electrocardiographic markers. However, the optimal timing of intervention remains unresolved: operating late can result in irreversible myocardial damage and arrhythmogenic substrates, whereas operating early can lead to repeated reinterventions, the impact of which on hard outcomes is uncertain. This review summarizes contemporary evidence on ventricular remodelling after PVR, focusing on cardiovascular magnetic resonance (CMR) and echocardiographic markers, and critically appraises proposed criteria for timing PVR. Classic CMR-derived thresholds (RV end-diastolic volume index [RVEDVi] 150\u2013170 mL\/m2, RV end-systolic volume index [RVESVi] 80\u201390 mL\/m2) and QRS duration cut-offs are discussed alongside emerging markers of risk, including the RV mass-to-volume ratio, diffuse myocardial fibrosis (extracellular volume fraction), strain imaging, and diastolic dysfunction. Meta-analyses show consistent reverse remodelling and symptomatic benefit after PVR, but no conclusive survival benefit has been demonstrated, and data on arrhythmic outcomes remain conflicting. Key gaps include (i) the lack of prospective randomized or carefully matched comparative studies of \u201cearly\u201d versus \u201cdeferred\u201d PVR; (ii) limited understanding of how myocardial fibrosis, RV hypertrophy, and diastolic dysfunction interact with volume load and timing to influence long-term outcomes; (iii) under-representation of adult and older adult TOF cohorts; and (iv) insufficient integration of multiparametric risk scores and machine-learning approaches into clinical decision-making. Future research should prioritize multicentre longitudinal cohorts with standardized imaging, electrophysiological and clinical endpoints, incorporate advanced imaging techniques (e.g., strain, 3D late gadolinium enhancement, and T1 mapping), and explore precision-medicine strategies to individualize PVR timing.<\/jats:p>","DOI":"10.3390\/jcm15031295","type":"journal-article","created":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T10:05:47Z","timestamp":1770372347000},"page":"1295","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Pulmonary Valve Replacement: Update on Timing and Ventricular Remodelling"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0367-1339","authenticated-orcid":false,"given":"Almudena","family":"Ortiz-Garrido","sequence":"first","affiliation":[{"name":"Section of Paediatric Cardiology, Hospital Materno Infantil, Regional Universitario de M\u00e1laga, 29011 Malaga, Spain"},{"name":"Faculty of Medicine, University of M\u00e1laga, 29010 Malaga, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-4494-1388","authenticated-orcid":false,"given":"Monika","family":"R\u00f3\u017cewicz Juraszek","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, 24105 Kiel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominik Daniel","family":"Gabbert","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, 24105 Kiel, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/L\u00fcbeck, 24105 Kiel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6534-087X","authenticated-orcid":false,"given":"Jill","family":"Jussli-Melchers","sequence":"additional","affiliation":[{"name":"Department of Cardiac Surgery, Universitatsklinikum Schleswig-Holstein, 24105 Kiel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Inga","family":"Voges","sequence":"additional","affiliation":[{"name":"Department of Congenital Heart Disease and Paediatric Cardiology, University Hospital Schleswig-Holstein, Campus Kiel, 24105 Kiel, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/L\u00fcbeck, 24105 Kiel, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,2,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1053\/j.pcsu.2006.02.009","article-title":"Indications and timing of pulmonary valve replacement after tetralogy of Fallot repair","volume":"9","author":"Geva","year":"2006","journal-title":"Semin. Thorac. Cardiovasc. Surg. Pediatr. Card. Surg. Annu."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1186\/1532-429X-13-9","article-title":"Repaired tetralogy of Fallot: The roles of cardiovascular magnetic resonance in evaluating pathophysiology and for pulmonary valve replacement decision support","volume":"13","author":"Geva","year":"2011","journal-title":"J. Cardiovasc. Magn. Reson."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1264","DOI":"10.1016\/j.amjcard.2009.06.043","article-title":"Impact of right ventricular dilation on left ventricular myocardial deformation in patients after surgical repair of tetralogy of Fallot","volume":"104","author":"Cheung","year":"2009","journal-title":"Am. J. Cardiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"975","DOI":"10.1016\/S0140-6736(00)02714-8","article-title":"Risk factors for arrhythmia and sudden cardiac death late after repair of tetralogy of Fallot: A multicentre study","volume":"356","author":"Balaji","year":"2000","journal-title":"Lancet"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2227","DOI":"10.1016\/j.jacc.2013.04.107","article-title":"Pulmonary valve replacement after operative repair of tetralogy of Fallot: A meta-analysis and meta-regression of 3,118 patients from 48 studies","volume":"62","author":"Santos","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"680","DOI":"10.1161\/CIRCINTERVENTIONS.113.000690","article-title":"Impact of transcatheter pulmonary valve replacement on biventricular strain and synchrony assessed by cardiac magnetic resonance feature tracking","volume":"6","author":"Harrild","year":"2013","journal-title":"Circ. Cardiovasc. Interv."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1016\/j.athoracsur.2020.11.040","article-title":"Pulmonary valve replacement in tetralogy of Fallot: An updated meta-analysis","volume":"113","author":"Vervoort","year":"2022","journal-title":"Ann. Thorac. Surg."},{"key":"ref_8","first-page":"2915","article-title":"ESC guidelines for the management of grown-up congenital heart disease","volume":"31","author":"Baumgartner","year":"2010","journal-title":"Eur. Heart J."},{"key":"ref_9","first-page":"2473","article-title":"Optimal timing of pulmonary valve replacement\u2014The holy grail in tetralogy of Fallot","volume":"40","author":"Pula","year":"2024","journal-title":"Can. J. Cardiol."},{"key":"ref_10","first-page":"77","article-title":"Myocardial extracellular volume fraction adds prognostic information in adults with repaired tetralogy of Fallot","volume":"18","author":"Chen","year":"2016","journal-title":"J. Cardiovasc. Magn. Reson."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Leonardi, B., Stambach, D., and B\u00fcchel, E.V. (2024). Repaired tetralogy of Fallot: Have we understood the right timing of pulmonary valve replacement?. J. Clin. Med., 13.","DOI":"10.3390\/jcm13092682"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"e004270","DOI":"10.1161\/CIRCIMAGING.116.004270","article-title":"How to image repaired tetralogy of Fallot","volume":"10","author":"Valente","year":"2017","journal-title":"Circ. Cardiovasc. Imaging"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1703","DOI":"10.1161\/CIRCULATIONAHA.117.027402","article-title":"Immediate and mid-term cardiac remodeling after surgical pulmonary valve replacement in adults with repaired tetralogy of Fallot","volume":"136","author":"Heng","year":"2017","journal-title":"Circulation"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1136\/heartjnl-2013-304958","article-title":"Contemporary predictors of death and sustained ventricular tachycardia in patients with repaired tetralogy of Fallot enrolled in the INDICATOR cohort","volume":"100","author":"Valente","year":"2014","journal-title":"Heart"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"2044","DOI":"10.1016\/S0735-1097(02)02566-4","article-title":"Right ventricular function in adults with repaired tetralogy of Fallot assessed with cardiovascular magnetic resonance imaging","volume":"40","author":"Davlouros","year":"2002","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"420","DOI":"10.1016\/j.amjcard.2008.09.101","article-title":"Adverse left ventricular mechanics in adults with repaired tetralogy of Fallot","volume":"103","author":"Tzemos","year":"2009","journal-title":"Am. J. Cardiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"e008321","DOI":"10.1161\/CIRCEP.119.008321","article-title":"Three-dimensional late gadolinium enhancement cardiovascular magnetic resonance predicts inducibility of ventricular tachycardia in adults with repaired tetralogy of Fallot","volume":"13","author":"Ghonim","year":"2020","journal-title":"Circ. Arrhythmia Electrophysiol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.ijcard.2020.02.067","article-title":"Echocardiographic predictors of severe right ventricular diastolic dysfunction in tetralogy of Fallot: Relations to patient outcomes","volume":"306","author":"Egbe","year":"2020","journal-title":"Int. J. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","unstructured":"Nonaka, H., R\u00e4tsep, I., Obonyo, N.G., Suen, J.Y., Fraser, J.F., and Chan, J. (2024). Current trends and latest developments in echocardiographic assessment of right ventricular function: Load dependency perspective. Front. Cardiovasc. Med., 11.","DOI":"10.3389\/fcvm.2024.1365798"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Burkhardt, B.E.U., Forte, M.N.V., Durairaj, S., Rafiq, I., Valverde, I., and Tandon, A. (2017). Timely pulmonary valve replacement may allow preservation of left ventricular circumferential strain in patients with tetralogy of Fallot. Front. Pediatr., 5.","DOI":"10.3389\/fped.2017.00039"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"2721","DOI":"10.1093\/eurheartj\/ehi581","article-title":"Remodelling of the right ventricle after early pulmonary valve replacement in children with repaired tetralogy of Fallot: Assessment by cardiovascular magnetic resonance","volume":"26","author":"Dave","year":"2005","journal-title":"Eur. Heart J."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"660","DOI":"10.1016\/j.athoracsur.2014.09.015","article-title":"Right ventricular remodeling after pulmonary valve replacement: Early gains, late losses","volume":"99","author":"Hallbergson","year":"2015","journal-title":"Ann. Thorac. Surg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1161\/CIRCULATIONAHA.106.659664","article-title":"Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance","volume":"116","author":"Oosterhof","year":"2007","journal-title":"Circulation"},{"key":"ref_24","first-page":"1619","article-title":"Preoperative predictors of death and sustained ventricular tachycardia after pulmonary valve replacement in repaired tetralogy of Fallot","volume":"138","author":"Bokma","year":"2018","journal-title":"Circulation"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1067\/mtc.2001.111209","article-title":"Late pulmonary valve replacement after repair of tetralogy of Fallot","volume":"121","author":"Discigil","year":"2001","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1093\/icvts\/ivx118","article-title":"Early versus late pulmonary valve replacement in patients with transannular patch-repaired tetralogy of Fallot","volume":"25","author":"Dobbels","year":"2017","journal-title":"Interact. Cardiovasc. Thorac. Surg."},{"key":"ref_27","first-page":"ivad105","article-title":"Pulmonary valve reconstruction for acquired pulmonary regurgitation in patients with treated congenital heart disease","volume":"37","author":"Hansen","year":"2023","journal-title":"Interdiscip. Cardiovasc. Thorac. Surg."},{"key":"ref_28","first-page":"495","article-title":"Myocardial T1 mapping: Methods, applications, and limitations","volume":"105","author":"Bing","year":"2019","journal-title":"Heart"},{"key":"ref_29","first-page":"1619","article-title":"Identification and assessment of diffuse myocardial fibrosis in cardiomyopathies using T1 mapping","volume":"59","author":"Sado","year":"2012","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1016\/j.amjcard.2004.11.037","article-title":"Optimal timing for pulmonary valve replacement in adults after tetralogy of Fallot repair","volume":"95","author":"Therrien","year":"2005","journal-title":"Am. J. Cardiol."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"507","DOI":"10.1161\/CIRCULATIONAHA.109.921692","article-title":"Short- and medium-term outcomes after transcatheter pulmonary valve replacement in the US Melody valve investigational trial","volume":"122","author":"McElhinney","year":"2010","journal-title":"Circulation"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/3\/1295\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,6]],"date-time":"2026-02-06T10:30:07Z","timestamp":1770373807000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/15\/3\/1295"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,6]]},"references-count":31,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026,2]]}},"alternative-id":["jcm15031295"],"URL":"https:\/\/doi.org\/10.3390\/jcm15031295","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,6]]}}}