{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T04:50:36Z","timestamp":1787028636333,"version":"build-2736575974"},"reference-count":36,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2023,7,10]],"date-time":"2023-07-10T00:00:00Z","timestamp":1688947200000},"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>The aim of the present review is to highlight the strengths and limitations of echocardiographic parameters and scores employed to predict favorable outcome in complex congenital heart diseases (CHDs) with borderline right ventricle (RV), with a focus on pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS\/CPS). A systematic search in the National Library of Medicine using Medical Subject Headings and free-text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords \u201cPAIVS\/CPS\u201d, Ebstein\u2019s anomaly, and unbalanced atrioventricular septal defect with left dominance. A total of 22 studies were selected for final analysis; 12 of them were focused on parameters to predict biventricular repair (BVR)\/pulmonary blood flow augmentation in PAIVS\/CPS. All of these studies presented numerical (the limited sample size) and methodological limitations (retrospective design, poor definition of inclusion\/exclusion criteria, variability in the definition of outcomes, differences in adopted surgical and interventional strategies). There was heterogeneity in the echocardiographic parameters employed and cut-off values proposed, with difficultly in establishing which one should be recommended. Easy scores such as TV\/MV (tricuspid\/mitral valve) and RV\/LV (right\/left ventricle) ratios were proven to have a good prognostic accuracy; however, the data were very limited (only two studies with &lt;40 subjects). In larger studies, RV end-diastolic area and a higher degree of tricuspid regurgitation were also proven as accurate predictors of successful BVR. These measures, however, may be either operator and\/or load\/pressure dependent. TV Z-scores have been proposed by several authors, but old and heterogenous nomograms sources have been employed, thus producing discordant results. In summary, we provide a review of the currently available echocardiographic parameters for risk prediction in CHDs with a diminutive RV that may serve as a guide for use in clinical practice.<\/jats:p>","DOI":"10.3390\/jcm12144599","type":"journal-article","created":{"date-parts":[[2023,7,11]],"date-time":"2023-07-11T01:58:14Z","timestamp":1689040694000},"page":"4599","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Echocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis"],"prefix":"10.3390","volume":"12","author":[{"given":"Massimiliano","family":"Cantinotti","sequence":"first","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"},{"name":"Institute of Clinical Physiology, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0337-9338","authenticated-orcid":false,"given":"Colin Joseph","family":"McMahon","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology, Childrens Health Ireland, D12 N512 Dublin, Ireland"},{"name":"School of Medicine, University College Dublin, D04 V1W8 Dublin, Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Marchese","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"},{"name":"Istituto di Scienze Della Vita (ISV), Scuola Superiore Sant\u2019Anna, 56127 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1766-7859","authenticated-orcid":false,"given":"Martin","family":"K\u00f6stenberger","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Cardiology, Medical University Graz, 8036 Graz, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7470-2422","authenticated-orcid":false,"given":"Marco","family":"Scalese","sequence":"additional","affiliation":[{"name":"Istituto di Scienze Della Vita (ISV), Scuola Superiore Sant\u2019Anna, 56127 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliana","family":"Franchi","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6342-7843","authenticated-orcid":false,"given":"Giuseppe","family":"Santoro","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia","family":"Assanta","sequence":"additional","affiliation":[{"name":"Fondazione G. Monasterio CNR-Regione Toscana, 56124 Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xander","family":"Jacquemyn","sequence":"additional","affiliation":[{"name":"Helen B. Taussig Heart Center, Department of Pediatrics, Johns Hopkins Hospital, Baltimore, MD 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelby","family":"Kutty","sequence":"additional","affiliation":[{"name":"Helen B. Taussig Heart Center, Department of Pediatrics, Johns Hopkins Hospital, Baltimore, MD 21205, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Giordano","sequence":"additional","affiliation":[{"name":"Adult and Pediatric Cardiac Surgery, Department Advanced Biomedical Sciences, University of Naples \u201cFederico II\u201d, 80131 Naples, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,10]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"892","DOI":"10.1111\/chd.12671","article-title":"Echocardiographic parameters associated with biventricular circulation and right ventricular growth following right ventricular decompression in patients with pulmonary atresia and intact ventricular septum: Results from a multicenter study","volume":"13","author":"Maskatia","year":"2018","journal-title":"Congenit. 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