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Updated: Jun 3, 2025
Morphological and Functional Assessment of the Right Ventricle Using 3D Echocardiography
Published on: October 28, 2020
Double Outlet Right Ventricle: A Rare Finding in a 15-Month-Old Female With Failure to Thrive
Bernadette Pedun1,2, Ivaan Pitua3, Felix Bongomin4,5
1Uganda Cancer Institute Kampala Uganda.
Insights
This case highlights a rare congenital heart defect, double outlet right ventricle (DORV), in an infant with recurrent respiratory issues. Early diagnosis and intervention are crucial for managing this complex pediatric cardiac condition.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Defects
- Medical Imaging
Background:
- Double outlet right ventricle (DORV) is a rare congenital heart defect.
- It often presents with complex intracardiac anomalies and circulatory imbalances.
- Compensatory mechanisms in DORV frequently prove insufficient.
Purpose of the Study:
- To report a case of DORV in an infant with recurrent respiratory infections.
- To emphasize the importance of considering cardiac pathology in pediatric respiratory symptoms.
- To illustrate diagnostic findings using computed tomography angiography.
Main Methods:
- Case report of a 15-month-old infant.
- Clinical examination, echocardiography, and computed tomography angiography.
- Review of diagnostic imaging and clinical presentation.
Main Results:
- The infant presented with recurrent respiratory infections, poor weight gain, pallor, tachypnea, and finger clubbing.
- Imaging confirmed DORV with additional defects: double superior vena cavae, hypoplastic aortic arch, hypertrophic right ventricle, and patent ductus arteriosus.
- Surgical correction was delayed due to respiratory complications.
Conclusions:
- Recurrent respiratory symptoms in pediatric patients can indicate underlying cardiac pathology like DORV.
- Prompt diagnosis and management of DORV are essential to prevent high mortality.
- Multimodality imaging is critical for delineating complex congenital heart defects.
Abstract:
Double outlet right ventricle (DORV) is a rare congenital heart defect where both the aorta and pulmonary artery originate from the right ventricle, often accompanied by additional cardiac anomalies to mitigate circulatory imbalance, though such compensations usually fail. We report a 15-month-old infant with recurrent respiratory infections and poor weight gain, referred for computed tomography angiography. Physical examination showed a small, non-syndromic infant with pallor, tachypnea, irritability, and finger clubbing. Initial imaging revealed cardiomegaly and lung infiltrates; echocardiography and computed tomography angiography confirmed additional intracardiac defects of double superior vena cavae, a hypoplastic aortic arch, hypertrophic right ventricular wall, and a patent ductus arteriosus. Corrective surgery was delayed due to respiratory complications. This case emphasizes the critical need to consider cardiac pathology in pediatric patients with recurrent respiratory symptoms, as untreated DORV can lead to high mortality.
