--- title: Double outlet right ventricle description: What double outlet right ventricle is, why this defect has so many variants, how it is found and why surgery cannot be avoided. type: disease aliases: - DORV - Double-outlet right ventricle sources: - tier: guideline org: esc title: 2020 ESC Guidelines for the management of adult congenital heart disease year: 2020 doi: 10.1093/eurheartj/ehaa554 url: https://doi.org/10.1093/eurheartj/ehaa554 - tier: reference org: msd-manuals title: Double Outlet Right Ventricle year: 2025 url: https://www.msdmanuals.com/professional/pediatrics/congenital-cardiovascular-anomalies/double-outlet-right-ventricle - tier: reference org: msd-manuals title: Double Outlet Right Ventricle (MSD Manual Consumer Version) year: 2023 url: https://www.msdmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/double-outlet-right-ventricle - tier: reference org: msd-manuals title: Overview of Heart Defects (MSD Manual Consumer Version) year: 2026 url: https://www.msdmanuals.com/home/children-s-health-issues/birth-defects-of-the-heart/overview-of-heart-defects sources_checked: 2026-10-14 review: null --- Double outlet right ventricle (DORV) is a congenital heart defect in which both large arteries, the aorta and the pulmonary artery, leave the right ventricle, and neither leaves the left ventricle. There is almost always a [ventricular septal defect](../ventricular-septal-defect/) as well, and it is the only way blood can get out of the left ventricle. The defect has many variants: it all depends on the size and position of the hole in the septum, and on whether there is a narrowing (stenosis) on the way into the pulmonary artery and how severe it is. The European guidelines class double-outlet ventricle among congenital heart defects of severe complexity.

Normally the pulmonary artery leaves the right ventricle and the aorta leaves the left.

The diagram shows one variant of the defect: the hole in the septum lies below the aorta; according to the MSD Manual for professionals, this is the most common variant. Both vessels leave the right ventricle, and the left ventricle can pump blood out only through the hole in the septum, so oxygen-rich and oxygen-poor blood mix. In this variant, blood from the left ventricle goes through the hole into both the aorta and the pulmonary artery; if there is no narrowing on the way into the pulmonary artery, the lungs get a lot of blood, almost as with a large ventricular septal defect.

## Why it happens Normally the pulmonary artery starts from the right ventricle and the aorta from the left. With this defect both vessels start from the right ventricle. Blood from the left ventricle reaches them through a hole in the wall between the ventricles, and oxygen-rich blood mixes with oxygen-poor blood. Because of this, the body's organs may get less oxygen than they need, and the baby's skin turns bluish; this is called cyanosis. And if too much blood flows through the pulmonary artery to the lungs, [heart failure](../heart-failure/) can develop. How low the oxygen level in the blood is depends on which way blood flows through the hole, and on whether there is an obstruction on the way into the pulmonary artery. The anatomy and physiology of DORV vary widely depending on the size and location of the ventricular septal defect (VSD), which is virtually always present, and on the presence and degree of pulmonary stenosis. Because both great arteries arise from one ventricle, there is some degree of mixing of desaturated systemic venous blood with highly saturated pulmonary venous return, which enters the right ventricle (RV) via the left atrium, the left ventricle (LV) and the VSD. The MSD Manual for professionals describes the major variants by the position of the VSD: - **Subaortic VSD** (the most common variant): the VSD shunt goes to both the aorta and the pulmonary artery, resulting in an effective large left-to-right shunt, and the physiology and clinical findings are nearly identical to those of a large VSD. If pulmonary stenosis is also present, more desaturated blood from the RV is directed to the aorta, leading to cyanosis and a picture similar to [tetralogy of Fallot](../tetralogy-of-fallot/). - **Subpulmonary VSD**: highly saturated blood from the LV flows predominantly to the pulmonary artery and desaturated blood from the RV goes mainly to the aorta; cyanosis is moderate to severe, with the same physiology as [transposition of the great arteries](../transposition-of-the-great-arteries/). When the pulmonary valve lies immediately next to the defect or partially overrides it, the defect is called the Taussig-Bing anomaly. - **Muscular VSD uncommitted to either great artery**: the clinical findings fall in the middle of the spectrum, with both excessive pulmonary blood flow and systemic desaturation. Various other congenital defects can be associated with DORV, most commonly [coarctation of the aorta](../coarctation-of-the-aorta/) with the Taussig-Bing anomaly. ## Symptoms Symptoms depend on where the hole in the septum is and what other abnormalities the baby has. A baby may breathe fast, feed poorly and tire easily, and the lips, nail beds and skin may turn bluish. Clinical manifestations range from severe cyanosis (with the Taussig-Bing anomaly or any VSD type with pulmonary stenosis) to congestive heart failure and [pulmonary hypertension](../pulmonary-hypertension/) (with a subaortic VSD and no pulmonary outflow obstruction). Examination may reveal a single loud second heart sound (S2) with pulmonary hypertension, a systolic ejection murmur at the base due to pulmonary stenosis, cyanosis with clubbing and, if coarctation is present, decreased femoral pulses. ## How it is diagnosed The diagnosis is confirmed with an [ultrasound scan of the heart (echocardiography)](../../diagnostic/echocardiography/). A chest X-ray and an [ECG](../../diagnostic/ecg/) are usually done as well. MRI or CT helps plan the operation, and cardiac catheterisation is needed only occasionally. Echocardiography demonstrates the anatomy well: the size and location of the VSD, the arrangement of the aorta and pulmonary artery, and the presence and degree of pulmonary stenosis, either below or at the valve level. The ECG consistently shows right ventricular hypertrophy with right axis deviation. If the pulmonary outflow and pulmonary valve are unobstructed, the chest X-ray shows cardiomegaly and increased pulmonary vascular markings; with pulmonary stenosis, heart size may be normal and pulmonary vascular markings normal or decreased. MRI or CT is valuable in planning surgery, giving details of the spatial relationships of the VSD and the great arteries. Cardiac catheterisation may help define the haemodynamics: any gradient across the VSD, pulmonary artery pressure and vascular resistance. ## How it is treated Medicines help with this defect, but surgery cannot be avoided: the defect is repaired with an operation. A newborn with marked cyanosis (blueness) may need medicines that keep the [ductus arteriosus](../patent-ductus-arteriosus/) from closing. If too much blood flows to the lungs, medicines are needed to improve how the heart works and to treat heart failure. Which operation is needed depends on the variant of the defect: in the most common variant a complete repair is possible, while in the Taussig-Bing anomaly and when the hole is not related to either of the large arteries the operation is more complex. So the type of operation is chosen according to which variant of the defect the child has. The European guidelines stress that care for people with congenital heart defects is lifelong. They recommend that every adult with any congenital heart defect, including this one, be seen at least once in a specialist centre, where the care and follow-up they need are decided. In the newborn period, medical maintenance of ductal patency may be needed for severe cyanosis with critical pulmonary stenosis; if pulmonary blood flow is greatly increased, heart failure treatment is required. However, surgical intervention is invariably required. According to the MSD Manual for professionals, in the most common variant, with a subaortic VSD, a complete repair is possible: the VSD is closed with an intracardiac patch so as to direct left ventricular outflow to the aorta, and pulmonary stenosis, if present, is relieved. In the Taussig-Bing anomaly the repair is more complex and involves an arterial switch along with closure of the VSD, directing left ventricular outflow to the aorta. When the VSD is uncommitted to either great artery, surgical correction is more difficult and may require a palliative procedure, such as an aortopulmonary shunt, or staged procedures leading to a Fontan operation (see [single ventricle](../single-ventricle/)). In the ESC classification, double-outlet ventricle (repaired or unrepaired) is a defect of severe complexity; any cyanotic congenital heart defect, unoperated or palliated, is in the same group. The complexity of the defect should not be the only criterion for assigning a level of care, although patients with complex defects can easily be assigned to a high level; the ESC recommends that every adult with congenital heart disease be seen at least once in a specialist centre, where the level of care and follow-up intervals are determined. ## When to call an ambulance Heart failure or cyanosis in a baby in the first week of life is a medical emergency. Call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK) if a baby's lips, nails or skin turn blue, or if the baby breathes fast or with effort, including during feeds. If the baby feeds poorly, tires quickly or sweats during feeds, take them to a doctor without delay.