In double outlet right ventricle, both the aorta and pulmonary valve connect to the right ventricle. Normally the pulmonary valve connects to the right ventricle, and the aorta connects to the left ventricle. A ventricular septal defect is virtually always present and provides the only outlet of blood flow from the left ventricle.
Symptoms depend on the specific anatomy and location of the ventricular defect and associated abnormalities.
Diagnosis is by electrocardiography, imaging tests, and cardiac catheterization.
Medication is helpful, but surgery is usually required.
(See also Overview of Heart Defects.)
In double outlet right ventricle, both the aorta and the pulmonary artery are connected to the right ventricle and no artery attaches to the left ventricle. Infants with double outlet right ventricle also have a hole between the ventricles (ventricular septal defect), which causes oxygen-rich blood to mix with the oxygen-poor blood. Therefore, infants may not get a normal amount of oxygen going to the body resulting in cyanosis. If too much blood flows through the pulmonary artery to the lungs, heart failure may develop.
Symptoms of Double Outlet Right Ventricle
Symptoms vary depending on the location of the ventricular septal defect and what additional abnormalities the infant has.
Infants may have rapid breathing, poor feeding, easy tiring, and/or bluish color of the lips, nail beds, and skin (cyanosis).
Diagnosis of Double Outlet Right Ventricle
Chest x-rays and electrocardiography (ECG)
Echocardiography (ultrasonography of the heart)
Magnetic resonance imaging (MRI) and computed tomography (CT)
Cardiac catheterization
Diagnosis is confirmed by echocardiography.
Chest x-rays and ECG are typically done. Cardiac catheterization is occasionally needed. Other imaging studies may be helpful.
Treatment of Double Outlet Right Ventricle
Medications to treat cyanosis and heart failure
Surgery
During the newborn period, medications called prostaglandins may be needed to keep the ductus arteriosus open if the infant has severe cyanosis. If too much blood flows to the lungs, medications are needed to improve heart function and treat the resulting heart failure.
Surgery is required to repair the defect.
More Information
The following English-language resources may be useful. Please note that The Manual is not responsible for the content of these resources.
American Heart Association: Common Heart Defects: Provides overview of common congenital heart defects for parents and caregivers
American Heart Association: Infective Endocarditis: Provides an overview of infective endocarditis, including summarizing prophylactic antibiotic use, for patients and caregivers
