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 Overview of Heart Defects About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more .)
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 Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more ), 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
Echocardiography (ultrasonography of the heart)
Magnetic resonance imaging (MRI) and computed tomography (CT)
Diagnosis is confirmed by echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more .
Chest x-rays X-Rays of the Chest Anyone thought to have a heart disorder has chest x-rays taken from the front and the side. Typically, the person is standing upright, but chest x-rays can be done with people lying in bed if... read more 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
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.
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