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What is double outlet right ventricle?

Double outlet right ventricle is a rare birth defect of the heart.

The heart has two upper collecting chambers (right and left atrium), two lower pumping chambers (right and left ventricle), one artery leaving the right ventricle (pulmonary artery) and one leaving the left ventricle (aorta). In a normal heart, the aorta leaves the left ventricle, carrying oxygen-rich (red) blood to the body. The pulmonary artery leaves the right ventricle, carrying oxygen-poor (blue) blood to the lungs.

In double outlet right ventricle, both of the heart’s “outlets” – the pulmonary artery and the aorta – exit abnormally from the right ventricle. This causes the oxygen-rich and oxygen-poor blood to mix together before leaving the heart.

Many kinds of heart defects fall into the category of double outlet right ventricle. They all share the feature that the aorta and pulmonary artery both exit from the right ventricle. In addition, other parts of the heart may be different.

In double outlet right ventricle, there is almost always a hole in the septum, or wall, between the right and left ventricles, called a ventricular septal defect, that allows blood to mix within the heart. Some children also have a hole in the septum between the right and left atria called an atrial septal defect. They can have other defects too, such as abnormal heart valves (pulmonary stenosis, aortic stenosis or mitral valve abnormalities) or abnormal arteries (coarctation of the aorta or pulmonary atresia).

Double Outlet Right Ventricle in Children

This is a congenital defect. Doctors do not know why it happens. They think it occurs very early in the pregnancy, when the baby’s heart is forming, before a woman may even know she is pregnant.

Double outlet right ventricle is rare. About one in every 10,000 babies has this condition.

Double Outlet Right Ventricle at Seattle Children’s

Our heart team has treated many children with double outlet right ventricle. We have extensive experience with the surgery these patients require. We also have a pediatric cardiac anesthesia team and a cardiac intensive care unit ready to care for children who undergo heart surgery.

When you come to Seattle Children's, a team of people will take care of your child. Along with your child's cardiologist, you are connected with surgeons, cardiac intensive care unit doctors, cardiac anesthesiologists, neonatologists, nurses, cardiac sonographers, dietitians, child life specialists, social workers and others, if their expertise is needed. We work together to meet all of your child's health needs and help your family through this experience.

Seattle Children's has been treating children since 1907. Our team members are trained in their fields and also in meeting the unique needs of children. For example, the doctors who give your child anesthesia are board certified in pediatric anesthesiology. This means they have extra years of training in how to take care of kids. Our child life specialists know how to help children understand their illnesses and treatments in ways that make sense for their age. 

The Adult Congenital Heart Disease Program shared by Seattle Children’s and the University of Washington can help with care throughout your child’s life.

Contact Us

Contact the Heart Center at 206-987-2015 for a cardiac referral, a second opinion or more information.

Who Treats This at Seattle Children's?

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Summer 2014: Good Growing Newsletter

In This Issue

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Download Summer 2014 (PDF)