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Updated: May 2, 2026
07:11
Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
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Double outlet right ventricle with intact ventricular septum
Senem Özgür1, Özben Ceylan1, Vehbi Doğan1
1Department of Cardiology, Dr. Sami Ulus Maternity, Children's Health and Diseases Training and Research Hospital, Ankara, Turkey.
Summary
A rare congenital heart defect, double outlet right ventricle with intact ventricular septum, was diagnosed in a cyanotic newborn. This case highlights the diagnostic challenges of this extremely uncommon cardiac malformation.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Malformations
Background:
- Double outlet right ventricle (DORV) is a complex congenital heart defect.
- In DORV, both the aorta and pulmonary artery originate from the right ventricle.
- DORV with an intact ventricular septum is exceptionally rare.
Observation:
- A 9-day-old male infant presented with cyanosis and a heart murmur.
- Physical examination revealed low oxygen saturation (84%) and a systolic murmur.
- Electrocardiography showed deep Q waves; echocardiography identified left atrial isomerism, dextrocardia, atrial septal defect, DORV with intact ventricular septum, great artery malposition, and pulmonary stenosis.
Findings:
- Diagnostic confirmation via catheter angiography.
- The patient was diagnosed with double outlet right ventricle with intact ventricular septum.
- Associated anomalies included left atrial isomerism, dextrocardia, ASD, and pulmonary stenosis.
Implications:
- This case underscores the rarity and diagnostic complexity of DORV with intact ventricular septum.
- Early diagnosis and management are crucial for infants with complex cyanotic heart disease.
- Reporting such rare cases contributes to a better understanding of congenital cardiac malformations.
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