Daily Radiology Case

Explanation

The entity is defined by apical displacement of the septal and posterior tricuspid leaflets, producing atrialization of the right ventricle, a shortened functional chamber, and massive right atrial enlargement. The enlarged, tethered anterior leaflet usually drives severe tricuspid regurgitation, and an associated ASD or PFO permits right-to-left shunting and cyanosis. Echocardiography is diagnostic, apical leaflet displacement ≥8 mm/m² is the hallmark, while MRI quantifies functional RV volume and the atrialized segment. Management spans medical therapy for heart failure and arrhythmias (with ablation for accessory pathways) through surgical valve repair such as cone reconstruction in severe disease.

Source: Yarmola I, Ebstein anomaly. Case study, Radiopaedia.org (Accessed on 04 Jun 2026) https://doi.org/10.53347/rID-236876

Hints

• A 12-year-old girl presents with progressive exercise intolerance and signs of heart failure.
• Examination reveals a holosystolic murmur at the left lower sternal border that augments with inspiration, along with hepatomegaly, elevated jugular venous pressure, and a triple gallop.
• The ECG shows tall, peaked right atrial P waves, right bundle branch block, and a delta wave indicating ventricular pre-excitation.
• Chest radiography demonstrates marked "box-shaped" cardiomegaly with a massively dilated right atrium, a narrow vascular pedicle, and reduced pulmonary vascularity.
• This rare congenital lesion is linked to maternal lithium exposure and coexists with an atrial septal defect or patent foramen ovale in the majority of cases.
• Cross-sectional imaging reveals the tricuspid valve abnormally displaced apically into the right ventricle with atrialization of its inlet portion, though in this case no significant regurgitant flow void is identified.