Overview
Double outlet right ventricle with subaortic or doubly committed ventricular septal defect (DORV-subaortic VSD) is a rare congenital heart defect present from birth. In a normal heart, the aorta carries blood from the left ventricle to the body, and the pulmonary artery carries blood from the right ventricle to the lungs. In DORV, both of these major blood vessels arise from the right ventricle instead of their normal positions. The 'subaortic' or 'doubly committed' type refers to the location of a hole in the wall between the two lower chambers of the heart (the ventricular septal defect, or VSD). When the VSD sits just below the aorta or beneath both great vessels, it creates a specific pattern of blood flow that makes this subtype somewhat more surgically favorable than other forms of DORV. Because of the abnormal connections, oxygen-rich and oxygen-poor blood mix inside the heart. This means the body does not get enough oxygen, leading to symptoms like bluish skin color (cyanosis), rapid breathing, poor feeding, and slow growth in infants. The heart works harder than normal, which can lead to heart failure if not treated. Some children also have other heart defects alongside DORV, such as narrowing of the pulmonary artery. Treatment is surgical and is usually performed in infancy or early childhood. The goal of surgery is to redirect blood flow so that the left ventricle pumps oxygen-rich blood to the body through the aorta. With timely surgery and ongoing cardiac care, many children with this condition can lead active lives, though lifelong follow-up with a heart specialist is important.
Also known as:
Key symptoms:
Bluish or grayish tint to the skin, lips, or fingernails (cyanosis)Rapid or labored breathingPoor feeding or tiring easily during feedingSlow weight gain or poor growth in infancySweating during feeding or activitySwelling in the legs, abdomen, or around the eyesFatigue and low energyHeart murmur detected by a doctorFrequent respiratory infectionsReduced exercise tolerance in older children
Multifactorial
Caused by a mix of several genes and environmental factors
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Double outlet right ventricle with subaortic or doubly committed ventricular septal defect.
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Specialists
View all specialists →No specialists are currently listed for Double outlet right ventricle with subaortic or doubly committed ventricular septal defect.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Double outlet right ventricle with subaortic or doubly committed ventricular septal defect.
Community
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Caregiver Resources
NORD Caregiver Resources
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the exact anatomy of my child's heart defect and how does it affect blood flow?,When should surgery be performed and what type of repair is planned?,What are the risks of surgery and what is the expected recovery time?,Will my child need more than one surgery, and what might trigger the need for future procedures?,Should we have genetic testing to look for an underlying syndrome or chromosomal condition?,What activity restrictions should my child follow before and after surgery?,What signs should prompt me to call the doctor or go to the emergency room?
Common questions about Double outlet right ventricle with subaortic or doubly committed ventricular septal defect
What is Double outlet right ventricle with subaortic or doubly committed ventricular septal defect?
Double outlet right ventricle with subaortic or doubly committed ventricular septal defect (DORV-subaortic VSD) is a rare congenital heart defect present from birth. In a normal heart, the aorta carries blood from the left ventricle to the body, and the pulmonary artery carries blood from the right ventricle to the lungs. In DORV, both of these major blood vessels arise from the right ventricle instead of their normal positions. The 'subaortic' or 'doubly committed' type refers to the location of a hole in the wall between the two lower chambers of the heart (the ventricular septal defect, or
How is Double outlet right ventricle with subaortic or doubly committed ventricular septal defect inherited?
Double outlet right ventricle with subaortic or doubly committed ventricular septal defect follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Double outlet right ventricle with subaortic or doubly committed ventricular septal defect typically begin?
Typical onset of Double outlet right ventricle with subaortic or doubly committed ventricular septal defect is neonatal. Age of onset can vary across affected individuals.