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Updated: May 12, 2026
09:22
Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Ebstein's anomaly with subpulmonary obstruction--a rare association
Bhupinder Singh1, Anand Subramanyan, M Jayaranganath
1Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India. dr_bhupinders@yahoo.in
Echocardiography (Mount Kisco, N.Y.)
|April 6, 2013
Summary
This case report details Ebstein's anomaly in a child with severe subpulmonic obstruction due to an abnormal tricuspid valve leaflet and a ventricular septal defect. Surgical palliation was planned due to the right ventricle's poor function.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Ebstein's anomaly is a spectrum of congenital heart defects affecting the tricuspid valve and right ventricle.
- Accurate diagnosis and management are crucial for pediatric patients with complex cardiac anomalies.
Observation:
- A 3-year-old boy presented with Ebstein's anomaly.
- The anomaly was uniquely associated with severe subpulmonic obstruction caused by a tethered and dysplastic anterior tricuspid leaflet.
- A large ventricular septal defect was also noted.
Findings:
- The patient exhibited a poorly functioning right ventricle.
- The anatomical findings presented a complex surgical challenge.
Implications:
- This case highlights a rare presentation of Ebstein's anomaly requiring tailored surgical planning.
- Management involved a staged approach with bidirectional Glenn shunt and planned Fontan completion.
- Understanding such unique associations is vital for improving outcomes in complex congenital heart disease.
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