Anatomical severity of Ebstein’s anomaly - a quantitative analysis based on cardiovascular MRI

Q: What is the main goal of this study on Ebstein’s anomaly?

To evaluate how cardiovascular MRI parameters relate to Carpentier class and heart failure symptoms, and to test whether CMR-derived markers predict postoperative needs and complications.

Q: Which CMR parameters were most strongly linked with Carpentier classes?

Indexed displacement of the septal and inferior leaflets (SLDi and ILDi), Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RV end-diastolic volume, and related ratios between atrialised/functional and anatomical RV.

Q: How did quantitative CMR indices relate to heart failure symptoms and postoperative outcomes?

Lower left ventricular ejection fraction and higher ILDi were associated with heart failure symptoms; ILDi also correlated with the need for bidirectional cavopulmonary anastomosis, haemodynamic complications, and significant postoperative right ventricular dysfunction.

Sep 30, 2026

Uploaded by nayy☆

Ebstein’s anomaly shows marked anatomical and clinical heterogeneity, making systematic risk stratification essential. Carpentier classification (CC) is commonly used qualitatively, while quantitative cardiovascular magnetic resonance (CMR) parameters may enable personalised monitoring and therapy. This retrospective 60-patient study assessed associations between CMR indices, CC, heart failure symptoms, and postoperative outcomes including bidirectional cavopulmonary anastomosis, haemodynamic complications, and right ventricular dysfunction.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages10

Reading Time25 min

AccessFree to download

Abstract

Background

Methods

Results

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What is already known on this topic

What this study adds

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Introduction

Open access Congenital heart disease Anatomical severity of Ebstein’s anomaly: a quantitative analysis based on cardiovascular MRI Giulia Pasqualin,1,2 Francesco Sturla ,3,4 Beatrice Cavazza,1 Lara Tondi,5,6 Massimiliana Abbate,1 Francesca Bevilacqua,1 Federica Torchio,7 Marina Hughes,8 Emanuele Micaglio,9,10 Massimo Lombardi,5 Pietro Spagnolo,11 Carlo Pappone,9,12 Emanuela Angeli,13,14 Massimo Chessa ,1,12 Alessandro Giamberti7,12 ► Additional supplemental material is published online only. To view, please visit the journal online ([https://doi.org/10.1136/](https://doi.org/10.1136/)[ ](https://doi.org/10.1136/)[openhrt-2025-003741](openhrt-2025-003741)) . To cite: Pasqualin G, Sturla F, Cavazza B, et al. Anatomical severity of Ebstein’s anomaly: a quantitative analysis based on cardiovascular MRI. Open Heart 2026;13:e003741 . doi:10 . 1136/ openhrt-2025-003741 Received 19 September 2025 Accepted 15 December 2025 © Author(s) (or their employer(s)) 2026. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ Group. For numbered affiliations see end of article. Correspondence to Dr Francesco Sturla; francesco. [sturla@grupposandonato.it](sturla@grupposandonato.it) ABSTRACT Background Ebstein’s anomaly (EA) exhibits significant anatomical and clinical heterogeneity, warranting a systematic approach to risk stratification. While the Carpentier classification (CC) is commonly employed for qualitative disease assessment, there is increasing interest in quantitative imaging parameters to personalise monitoring strategies and therapeutic interventions. We sought to evaluate the association between cardiovascular magnetic resonance (CMR) parameters, CC and symptoms of heart failure (HF) . Also, we investigated whether CMR-derived markers may predict the need for bidirectional cavopulmonary anastomosis (BCPA) and the occurrence of haemodynamic complications or significant right ventricular (RV) dysfunction in the postoperative stay. Methods This retrospective study evaluated consecutive patients diagnosed with EA undergoing 1.5T CMR acquisition at a tertiary care centre. CC and quantitative indices were extracted from steady-state free precession sequences. Results In a total population of 60 patients (53% male, median age 22 years), CMR parameters most strongly associated (p<0 . 001) with CC included indexed displacement of the septal (SLDi) and inferior (ILDi) leaflets, Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RVend-diastolic volume and the ratio of either atrialisedor functional RV to anatomical RV. Decreasing left ventricular ejection fraction (OR 0. 85, 95% CI 0.75 to 0. 97, p=0 . 01) and increasing ILDi (OR 1. 05, 95% CI 1.00 to 1. 10, p=0 . 04) emerged as the most prominent variables associated with HF symptoms. Additionally, ILDi was significantly linked to the need for BCPA (OR 1. 15, 95% CI 1.03 to 1. 28), the occurrence of haemodynamic complications (OR 1. 09, 95% CI 1.01 to 1. 18) and significant RV dysfunction in the postoperative stay (OR 1. 08, 95% CI 1.01 to 1. 17) . Conclusions Quantitative CMR indices proved to be effective in distinguishing between Carpentier classes and they may be valuable in an integrated CMR-based approach to assess EA severity. Among these, ILDi reflects both the extent of tricuspid valve abnormality and RV atrialisation and may serve as a useful metric in guiding personalised therapeutic strategies. WHAT IS ALREADY KNOWN ON THIS TOPIC | ⇒ Ebstein’s anomaly is a heterogeneous congenital malformation with variable anatomical severity traditionally assessed qualitatively. Cardiovascular MR (CMR) provides accurate chamber quantification, but validated quantitative indices for grading severity and guiding surgical planning remain limited.
WHAT THIS STUDY ADDS | | --- | | ⇒ This study identifies inferior leaflet displacement index (ILDi) and Ebstein valve rotation angle (EVRA) as

FAQs

What is the main goal of this study on Ebstein’s anomaly?

To evaluate how cardiovascular MRI parameters relate to Carpentier class and heart failure symptoms, and to test whether CMR-derived markers predict postoperative needs and complications.

Which CMR parameters were most strongly linked with Carpentier classes?

Indexed displacement of the septal and inferior leaflets (SLDi and ILDi), Ebstein valve rotation angle, functional RV ejection fraction, indexed atrialised RV end-diastolic volume, and related ratios between atrialised/functional and anatomical RV.

How did quantitative CMR indices relate to heart failure symptoms and postoperative outcomes?

Lower left ventricular ejection fraction and higher ILDi were associated with heart failure symptoms; ILDi also correlated with the need for bidirectional cavopulmonary anastomosis, haemodynamic complications, and significant postoperative right ventricular dysfunction.

Sep 30, 2026

Uploaded by nayy☆