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Ebstein anomoly radiology

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Ebstein anomaly radiology chest X-ray echocardiogram

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Here is a comprehensive overview of Ebstein anomaly radiology, drawn from Grainger & Allison's Diagnostic Radiology, Braunwald's Heart Disease, and supporting literature.

Ebstein Anomaly — Radiology

Anatomy & Pathology (Brief Review)

Ebstein anomaly is a congenital tricuspid valve and right ventricular defect defined by:
  1. Failure of delamination of tricuspid leaflets from the RV endocardium
  2. Apical displacement of the septal (and sometimes inferior) tricuspid leaflet from the AV junction — diagnostic threshold: >8 mm/m² or >20 mm displacement in adults
  3. "Atrialized" RV — the proximal RV is incorporated into the right atrium, creating a large, thin-walled chamber
  4. Sail-like, redundant anterior leaflet — may be fenestrated or tethered
  5. Dilation of the right AV junction; variable RV myopathic change
  6. Commonly associated with ASD/PFO, accessory pathways (WPW), pulmonary stenosis/atresia

Chest Radiograph (CXR)

Classic appearance:
FeatureFinding
Heart sizeMassive cardiomegaly ("box-shaped" or globular heart)
Right heart borderMarkedly prominent, rounded — due to severe RA + atrialized RV enlargement
Pulmonary vascularityDecreased (oligemic lung fields) due to reduced pulmonary flow ± right-to-left shunt
Aortic knobSmall / inconspicuous
Pulmonary trunkSmall shadow
The cardiothoracic ratio ≥0.65 is associated with worse prognosis (increased mortality from biventricular failure or sudden death).
CXR Example:
Ebstein anomaly CXR — massive cardiomegaly with globular heart, prominent right heart border, oligemic lungs
AP CXR: Massive cardiomegaly with globular shape, prominent right atrial border, and decreased pulmonary vascularity — classic for Ebstein anomaly.
Multi-modality Ebstein — ECG, CXR, and CT
Panel A: ECG showing RBBB. Panel B: PA CXR with right heart enlargement. Panel C: Axial CT demonstrating enlarged tricuspid annulus (white arrow).

Echocardiography (Primary Modality)

Echocardiography is the first-line imaging modality and must characterize:
  • Apical displacement of the septal tricuspid leaflet (>8 mm/m²) — the defining criterion
  • Mobility of the anterosuperior and inferior leaflets
  • Eccentric leaflet coaptation
  • Tricuspid regurgitation — quantified by Doppler
  • Right atrial dilation + size of the atrialized RV
  • RV and LV volumes and systolic function
  • Direction of flow across any PFO/ASD (right-to-left shunting causes cyanosis)
  • RVOT obstruction
TEE may be needed to evaluate the anterior leaflet and its distal tethering, which can be distant from the probe.

Cardiac MRI (CMR)

CMR is used when echocardiography is insufficient and is particularly valuable for:
ParameterRole
Degree of TV displacement and rotationAnatomic severity grading
Tricuspid regurgitation quantificationRegurgitant fraction
RV volumes and systolic functionKey prognostic marker
LV volumes (often small)Biventricular assessment
Cardiac outputOften reduced
Qp:QsQuantifies intracardiac shunt
CMR-derived RV and LV systolic dysfunction independently predict mortality and sustained VT.

CT

CT demonstrates:
  • Marked enlargement of the tricuspid annulus (see Panel C above)
  • Right atrial and atrialized RV dilation
  • Useful for pre-operative anatomic planning and when CMR is contraindicated

Key Imaging Goals Summary

(from Grainger & Allison's Diagnostic Radiology)
  1. Describe apical displacement of the septal tricuspid leaflet
  2. Assess mobility of the anterosuperior and inferior leaflets
  3. Note eccentric coaptation
  4. Quantify tricuspid regurgitation
  5. Quantify RA dilation and size of the atrialized RV
  6. Assess RV and LV volume, function, and mass
  7. Quantify right-to-left shunting (Qp:Qs)
  8. Exclude RVOT obstruction

ECG Correlation

Though not imaging per se, the ECG often accompanies radiology workup:
  • Tall, peaked "Himalayan" P waves (>5 mm) — RA enlargement
  • Prolonged PR interval
  • Short PR + delta wave — accessory pathway (WPW, Mahaim)
  • Complete or incomplete RBBB
  • RSR' in V1 — RV conduction delay
  • Atrial flutter/fibrillation common
Sources: Grainger & Allison's Diagnostic Radiology; Braunwald's Heart Disease (10th ed.)
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