A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction

Q: What is the clinical evidence from this study?

Study design: Cohort. Population: Ebstein anomaly undergoing cone surgical reconstruction (n=20). Intervention: Ebstein valve rotation angle (EVRA) >60 degrees on cardiovascular magnetic resonance vs. Ebstein valve rotation angle (EVRA) ≤60 degrees. Primary outcome: Early surgical dehiscence of the reconstructed valve apparatus within 30 days of surgery (RR 3.2, 95% CI 1.3-4.9, p=0.03).

Q: What does this research mean for the field?

An Ebstein valve rotation angle greater than 60° on pre-operative cardiovascular magnetic resonance is associated with a 3.2-fold increased relative risk of early surgical dehiscence after cone reconstruction. Novelty: ClaimNovelty.NOVEL_FINDING. Consensus alignment: ConsensusAlignment.NEUTRAL.

Marina Hughes; Béatrice Bonello; Preeti Choudhary; Jan Marek; Victor Tsang

doi:10.1186/s12968-019-0546-3

January 1, 2019Journal of Cardiovascular Magnetic ResonanceOpen Access

A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction

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Key result

Ebstein valve rotation angle >60° linked to ~3.2-fold higher risk of early surgical dehiscence.

RR 3.2
95% CI 1.3-4.9
P=0.03
n=20

Figures

Why the study?

Variation in Ebstein valve morphology affects surgical strategy, prompting exploration of practical, easily measurable CMR-derived attributes contributing to the complexity and risk of cone reconstruction.

Does a larger pre-operative CMR-derived Ebstein valve rotation angle (EVRA) and leaflet thickening predict early surgical dehiscence within 30 days in patients older than 12 years undergoing cone reconstruction for Ebstein anomaly?

Key Points

To identify preoperative cardiovascular magnetic resonance (CMR) attributes, particularly the Ebstein valve rotation angle, that correlate with surgical complexity and early failure following cone reconstruction.
Retrospective review of 20 patients older than 12 years with Ebstein anomaly (median age 20 years, range 14–57) who underwent preoperative CMR and surgical cone reconstruction by a single surgeon.
CMR was used to evaluate the Ebstein valve rotation angle (EVRA), functional right ventricular end-diastolic volume index (RVEDVi), chamber area ratios, regurgitant fraction, and anterior leaflet edge thickening.
Tricuspid regurgitation improved in all patients, but 4 of the 20 CMR patients (20%) had early paravalve dehiscence within 30 days of surgery, leading to 1 death.
An EVRA >60 o was associated with repair breakdown, conferring a relative risk of dehiscence of 3.2 (CI 1.3–4.9, p = 0.03; median EVRA 72 o vs 50 o , p = 0.01).
Dehiscence was also significantly associated with thickened anterior leaflet edges (RR 17, CI 3–100, p < 0.01), smaller functional RVEDVi (132 vs 177 mL/m2, p = 0.04), and older patient age (median 38 vs 19 years, p = 0.01).

Study Design

Type

Cohort (n=20)

Blinding

Experienced, independent observers measuring EVRA were blinded to each other's and the surgical results at the time of analysis

Multicenter

No

Structured PICO

Does a larger pre-operative CMR-derived Ebstein valve rotation angle (EVRA) and leaflet thickening predict early surgical dehiscence within 30 days in patients older than 12 years undergoing cone reconstruction for Ebstein anomaly?

P

Population

20 patients with Ebstein anomaly (median age 20 years, range 14-57; 45% female) with pre-operative CMR who underwent primary cone surgical reconstruction and were assessed for 30-day post-operative outcomes.

E

Exposure

Pre-operative cardiovascular magnetic resonance (CMR) assessment measuring the Ebstein valve rotation angle (EVRA >60 degrees), thickened/tethered anterior leaflet edges, and indexed functional right ventricular end-diastolic volume (RVEDVi) prior to cone reconstruction.

C

Comparator

Patients with smaller Ebstein valve rotation angle (EVRA ≤60 degrees), absence of anterior leaflet edge thickening, or intact cone reconstruction (n=16).

O

Outcome

Short-term primary outcome measures within 30 days of surgery: early death, early surgical dehiscence of the reconstructed valve apparatus, and severe residual tricuspid valve regurgitation.hard clinical

Main Result

Relative Risk: 3.2 (95% CI 1.3–4.9)

p-value: p=0.03

Pre-operative CMR measurement of the Ebstein valve rotation angle (EVRA >60°), smaller functional RVEDVi, and anterior leaflet edge thickening identify patients at high risk of early repair dehiscence after cone reconstruction, guiding surgical modifications such as leaflet patch augmentation.

Limitations

Retrospective clinical analysis design
Small sample size (n=20) precluding multivariate regression methods
Possible confounding due to an era effect and evolution over time of the surgeon's skill, experience, and technique
Inter-observer variability in EVRA measurements of up to 25 degrees, particularly at lower angle values
Retrospective clinical analysis
Small sample size (n=20) precluding valid use of multivariate regression methods
Observers differed in EVRA angle measurements by up to 25 degrees (95% limits of agreement -13° to +25°), greatest at lower angle values

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Implication

May identify high-risk Ebstein patients for modified repair; leaves open whether EVRA should guide surgery pending prospective validation.

Abstract

Once surgical management is indicated, variation of Ebstein valve morphology affects surgical strategy. This study explored practical, easily measureable, cardiovascular magnetic resonance (CMR)-derived attributes that may contribute to the complexity and risk of cone reconstruction. A retrospective assessment was performed of Ebstein anomaly patients older than 12 years age, with pre-operative CMR, undergoing cone surgical reconstruction by one surgeon. In addition to clinical data, the CMR-derived Ebstein valve rotation angle (EVRA), area ratios of chamber size, indexed functional RV (RVEDVi) and left ventricular (LV) volumes, tricuspid valve regurgitant fraction (TR%) and other valve attributes were related to early surgical outcome; including death, significant residual TR% or breakdown of repair. Of 26 operated patients older than 12 years age, since program start, 20 had pre-op CMR and underwent surgery at median (range) age 20 (14–57) years. TR% was improved in all patients. Four of the 20 CMR patients (20%) experienced early surgical dehiscence of the paravalve tissue, with cone-shaped tricuspid valve intact; one of whom died. A larger EVRA correlated with Carpentier category and was significantly related to dehiscence. If EVRA >60 o , relative risk of dehiscence was 3.2 (CI 1.3–4.9, p = 0.03). Those with dehiscence had thickened, more tethered anterior leaflet edges (RR 17, CI 3–100, p < 0.01), smaller pre-operative functional RVEDVi; (132 vs 177 mL/m2, p = 0.04), and were older (median 38 vs 19 years, p = 0.01). TR %, chamber area ratios and LV parameters were not different. Comprehensive CMR assessment characterizes patients prior to cone surgical reconstruction of Ebstein anomaly. Pragmatic observation of larger EVRA, smaller RVEDVi and leaflet thickening, suggests risk of repair tension and dehiscence, and may require specific modification of cone surgical technique, such as leaflet augmentation.

Discussion

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Cite This Study

Hughes et al. (2019) conducted a cohort in Ebstein anomaly undergoing cone surgical reconstruction (n=20). Ebstein valve rotation angle (EVRA) >60 degrees on cardiovascular magnetic resonance vs. Ebstein valve rotation angle (EVRA) ≤60 degrees was evaluated on Early surgical dehiscence of the reconstructed valve apparatus within 30 days of surgery (RR 3.2, 95% CI 1.3-4.9, p=0.03). An Ebstein valve rotation angle >60° on pre-operative CMR was associated with a 3.2-fold increased relative risk of early surgical dehiscence after cone reconstruction (RR 3.2, 95% CI 1.3-4.9, p=0.03).

synapsesocial.com/papers/6a7a29c5756812e395897325 https://doi.org/10.1186/s12968-019-0546-3

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A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction

Figures

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

A simple measure of the extent of Ebstein valve rotation with cardiovascular magnetic resonance gives a practical guide to feasibility of surgical cone reconstruction

Figures

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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