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September 29, 2026The Egyptian Heart JournalOpen Access

A rare congenital triad: ebsteinoid tricuspid valve, giant right atrial aneurysm, and atrial septal defect

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Key result

Surgical repair of Ebsteinoid tricuspid valve, ASD, and giant RA aneurysm preserves RV function at one year.

  • n=1

Why the study?

The coexistence of an Ebsteinoid tricuspid valve anomaly, giant right atrial aneurysm, and atrial septal defect is exceptionally rare and requires detailed anatomical delineation for surgical planning.

Population

A 15-year-old girl with Ebsteinoid tricuspid valve anomaly, giant right atrial aneurysm, and ostium secundum ASD

Comparison

Tricuspid valve repair, ASD closure, and right atrial reduction atrioplasty

Design

Case report

Follow-up

One year

Authors

UKUma Devi KaruruSASunitha ArumullaNDNeusha Doddi

Discussion

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Overview

Supports individualized surgical repair in this rare triad; leaves open need for multicenter data on long-term outcomes.

Key Points

  • To describe the presentation, multimodality imaging, and successful surgical management of a rare congenital triad consisting of an Ebsteinoid tricuspid valve, a giant right atrial aneurysm, and an atrial septal defect.
  • Evaluated a 15-year-old girl presenting with exertional dyspnea (NYHA class II) and palpitations using chest radiography, transthoracic and four-dimensional echocardiography, cardiac CT, and cardiac catheterization.
  • Performed surgical intervention comprising tricuspid valve repair, ostium secundum atrial septal defect closure, and right atrial reduction atrioplasty, followed by clinical evaluation at one year.
  • Diagnostic imaging identified severe tricuspid regurgitation, an ostium secundum atrial septal defect, and an Ebsteinoid tricuspid valve with elongated anterior and tethered septal leaflets, while catheterization confirmed pulmonary artery pressure at one-third systemic pressure.
  • At one-year follow-up, the patient was asymptomatic (NYHA class I) with preserved right ventricular function and only trivial residual tricuspid regurgitation.

Study Design

Type

Case Report (n=1)

Structured PICO

P
Population
A 15-year-old girl with progressive exertional dyspnoea and intermittent palpitations diagnosed with an Ebsteinoid tricuspid valve, giant right atrial aneurysm, and an ostium secundum ASD, followed for one year.
I
Intervention
Tricuspid valve repair, ASD closure, and right atrial reduction atrioplasty
O
Outcome
Clinical and echocardiographic outcomes (symptoms, right ventricular function, residual tricuspid regurgitation) at one-year follow-up

Early surgical correction of a rare triad of Ebsteinoid tricuspid valve, giant right atrial aneurysm, and ASD yielded excellent clinical and echocardiographic outcomes at one year.

Cite This Study

Karuru et al. (2026) conducted a case report in Ebsteinoid tricuspid valve anomaly, giant right atrial aneurysm, and ostium secundum ASD (n=1). Tricuspid valve repair, ASD closure, and right atrial reduction atrioplasty was evaluated on Clinical and echocardiographic outcomes. Surgical correction of an Ebsteinoid tricuspid valve, giant right atrial aneurysm, and ASD resulted in asymptomatic status (NYHA class I) and preserved right ventricular function at one year.

synapsesocial.com/papers/6abb3e235eb570e077ea1629https://doi.org/10.1186/s43044-026-00787-8
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Also Consider

Synapse has enriched 4 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Displacement of the anterior leaflet of the tricuspid valve: Rare variant of Ebstein's anomaly2016 · 8 citations
  2. 2The Congenital Tricuspid Valve Spectrum: From Ebstein to Dysplasia2020 · 29 citations
  3. 3Ebstein’s anomaly: contemporary management strategies2020 · 62 citations
  4. 4Ebstein anomaly review: what’s now, what’s next?2015 · 101 citations