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Health condition · Clinically reviewed

Double outlet right ventricle, a rare heart defect with tailored, specialist repair.

Both great arteries arise from the right ventricle. Anatomy varies widely, and each child needs a plan built for them by a specialist congenital cardiac team.

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Why trust this guide

  • 01

    Clinically reviewed

    Written by our editorial team and reviewed by a UK-registered clinician before publication.

  • 02

    Sourced from guidance

    Checked against UK specialist congenital cardiac guidance, BCS/BCCA and peer-reviewed sources cited at the end.

  • 03

    Current for 2026

    Reflects modern UK practice across specialist commissioned paediatric cardiac surgery centres.

Key facts

DORV at a glance.

The essentials in plain English: what it is, why anatomy matters and how the UK specialist system treats it.

  • What it is

    A rare congenital heart defect where both great arteries, the aorta and pulmonary artery, arise from the right ventricle.

  • How common

    Accounts for roughly 1 to 3 percent of all congenital heart disease. Highly variable anatomy from case to case.

  • Anatomy varies

    Classified by the position of the ventricular septal defect (VSD), the great vessel relationship, and the presence of pulmonary stenosis.

  • Physiology

    Ranges from tetralogy-like (subaortic VSD with pulmonary stenosis) to transposition-like (Taussig-Bing anomaly) to VSD-like.

  • When picked up

    Often at the 20-week antenatal anomaly scan, or shortly after birth on pulse oximetry screening and echocardiography.

  • Treatment

    Individualised surgical repair at a specialist commissioned UK congenital cardiac centre, followed by lifelong ACHD follow-up.

Why this guide matters

A rare defect, but a well-mapped pathway.

DORV is rare and complex, but the UK has a well-organised specialist network. Three ideas shape everything on this page.

  • Anatomy drives the plan

    The position of the VSD and the great vessel relationship determines whether repair is tetralogy-like, transposition-like or something else.

  • Care is centralised on purpose

    DORV is repaired at specialist commissioned UK centres, where teams see enough cases to know the nuances of each variant.

  • Follow-up is lifelong

    Repair is a beginning, not an end. Adult congenital heart disease services watch for arrhythmia, dysfunction and conduit problems for life.

How the diagnosis is made

From the 20-week scan to a surgical plan.

The steps a UK fetal cardiology and paediatric cardiac team will normally follow, so you know what to expect and why.

  1. 01

    Antenatal

    Antenatal anomaly scan

    The 20-week ultrasound is the most common point of first suspicion, with concerning views of the outflow tracts.

  2. 02

    Antenatal

    Fetal echocardiography

    A detailed fetal echo confirms the diagnosis, defines VSD position and looks for associated defects.

  3. 03

    Antenatal

    Fetal cardiac MRI

    Used selectively when echo windows are limited or anatomy is unusually complex.

  4. 04

    Postnatal

    Newborn pulse oximetry screen

    Routine UK newborn screening flags babies with low oxygen saturations for urgent cardiac assessment.

  5. 05

    Postnatal

    Postnatal echocardiography

    The gold-standard postnatal test. Confirms DORV, defines the VSD, great vessels, pulmonary stenosis and any associated lesions.

  6. 06

    Planning

    Cardiac MRI, CT and catheter

    Cross-sectional imaging and cardiac catheterisation help plan the surgical approach in complex or borderline anatomy.

  7. 07

    Planning

    Genetic testing and MDT

    Testing for 22q11.2 deletion (DiGeorge) and other syndromes. All cases are discussed in a specialist paediatric cardiac MDT.

Typical timeline: antenatal suspicion, postnatal confirmation, then a surgical plan built for your child.

Symptoms

What DORV looks like in a baby.

A mix of cyanosis, heart failure signs and murmurs. The exact pattern depends on the anatomy and how much pulmonary stenosis is present.

  • Cyanosis

    Blue lips, tongue or nail beds. Severity depends on the degree of pulmonary stenosis and mixing.

  • Heart failure signs

    Fast breathing, sweating with feeds, poor weight gain and hepatomegaly in babies with high pulmonary flow.

  • Failure to thrive

    Poor feeding and slow weight gain are common when unrepaired physiology overloads the heart or lungs.

  • Heart murmur

    A pansystolic VSD murmur, an ejection murmur from pulmonary stenosis, or both, heard on newborn examination.

  • Low oxygen saturations

    Detected on newborn pulse oximetry screening or later on routine observation.

  • Recurrent chest infections

    Older infants with high pulmonary blood flow can present with repeated respiratory illness.

  • Older-child presentation

    A minority with balanced physiology present later with a murmur, breathlessness or reduced exercise tolerance.

  • Red flag - collapse or deep cyanosis

    A neonate who is deeply blue, mottled or collapsing needs emergency cardiac assessment and prostaglandin.

Treatment

How DORV is treated in the UK.

Surgical, always individualised, and delivered by specialist commissioned paediatric cardiac centres. Some children need staged operations; some need a single, definitive repair.

  • Antenatal counselling and planning

    Once diagnosed on fetal echo, families are seen by a specialist team to plan delivery, neonatal care and early surgical assessment.

  • Neonatal stabilisation

    Prostaglandin infusion keeps the duct open when needed, alongside careful oxygen, fluid and inotrope management on a specialist unit.

  • Palliative shunt or PA banding

    Temporary measures in the newborn period. A modified Blalock-Taussig shunt improves pulmonary blood flow; a PA band protects the lungs from over-circulation.

  • Tetralogy-like DORV repair

    For subaortic VSD with pulmonary stenosis: intracardiac VSD closure and relief of right ventricular outflow tract obstruction.

  • Arterial switch (Taussig-Bing)

    For subpulmonary VSD (Taussig-Bing anomaly): arterial switch combined with VSD closure to route the left ventricle to the aorta.

  • Rastelli or Nikaidoh procedure

    Complex biventricular repairs used when the VSD and pulmonary stenosis anatomy suit a tunnel plus conduit approach.

  • Univentricular pathway (Fontan)

    Staged single-ventricle palliation when biventricular repair is not feasible. Delivered at a specialist commissioned centre.

  • Lifelong ACHD follow-up

    Every child transitions into adult congenital heart disease services for lifelong surveillance of arrhythmia, function and conduits.

What this guide is based on

The sources behind every claim on this page.

UK national guidance, specialist congenital cardiac standards and international consensus, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your specialist congenital cardiac team knows your child’s anatomy and history and can tell you which parts apply to you. If in doubt, ask them.

  • NHS England. Specialised services for congenital heart disease: service specifications.

  • British Congenital Cardiac Association (BCCA). Standards and consensus documents.

  • British Cardiovascular Society (BCS). Guidance on adult congenital heart disease.

  • European Society of Cardiology (ESC). Guidelines for the management of adult congenital heart disease.

  • Royal College of Obstetricians and Gynaecologists (RCOG). Fetal cardiology and anomaly screening standards.

Red flags

When DORV needs urgent attention.

Once a plan is in place, most children are well between visits. These are the situations that need earlier contact with the team.

  • Deep or worsening cyanosis

    A neonate with rapidly deepening cyanosis needs immediate cardiac assessment. Prostaglandin may be needed to reopen the duct.

  • Collapse or shock

    Poor perfusion, mottling and low output in a newborn is a medical emergency and needs a resuscitation team and cardiac input.

  • Poor feeding and failure to thrive

    Progressive breathlessness with feeds and static or falling weight suggests worsening heart failure.

  • Arrhythmia after repair

    Atrial or ventricular arrhythmias after DORV repair are common in later life and need urgent ACHD review.

  • Prosthetic conduit dysfunction

    Conduits and valves used in repair wear out. New murmurs, breathlessness or falling exercise tolerance need imaging.

  • Ventricular dysfunction

    Late right or left ventricular dysfunction can develop silently. Regular echo and cardiac MRI pick it up early.

  • Infective endocarditis risk

    Fevers, malaise or embolic features after any cardiac surgery need blood cultures and echo urgently.

  • Pregnancy planning

    Adults with repaired DORV should have pre-pregnancy counselling in a specialist ACHD-obstetric clinic.

  • Missed antenatal diagnosis

    Persistent murmur, cyanosis or breathlessness in a baby needs echo, even when the 20-week scan was reported as normal.

Living with it

A rare defect, a full life with the right team.

Modern repair of DORV has transformed outcomes. The routine after surgery is regular follow-up, sensible activity and knowing who to call.

A quiet reminder

Follow-up is the treatment.

Even a child who is well after repair benefits from lifelong specialist review. Small changes picked up early are much easier to manage.

  1. 01 Team

    Stay linked to your specialist centre

    Children and adults with DORV are followed in specialist commissioned UK centres. Keep every appointment and share records with your GP.

  2. 02 Transition

    Move smoothly into ACHD

    From around 16, care transitions to adult congenital heart disease teams. This is lifelong follow-up, not a graduation.

  3. 03 Family

    Support networks help

    Little Hearts Matter, the British Heart Foundation and the Down Heart Group offer information, peer support and practical help.

  4. 04 Everyday

    Live well, live carefully

    Most children do well after repair. Activity, school and work are usually possible, with tailored advice on exercise, dental care and pregnancy.

Frequently asked

Everything families ask about DORV.

Quick answers on diagnosis, surgery, UK specialist centres and long-term outlook.

  • What is double outlet right ventricle?

    Double outlet right ventricle (DORV) is a rare congenital heart defect in which both great arteries, the aorta and the pulmonary artery, arise mostly or entirely from the right ventricle. There is almost always an associated ventricular septal defect. The anatomy varies widely from child to child.

  • How is DORV diagnosed?

    Most cases are picked up on the routine 20-week antenatal anomaly scan and confirmed with fetal echocardiography. After birth, postnatal echocardiography is the gold standard. Cardiac MRI, CT and catheterisation are used in selected cases to plan surgery, and genetic testing looks for conditions such as 22q11.2 deletion (DiGeorge syndrome).

  • How is DORV treated?

    Treatment is surgical and individualised to the anatomy. Tetralogy-like DORV usually needs an intracardiac repair; Taussig-Bing anomaly often needs an arterial switch with VSD closure; complex anatomy may need Rastelli, Nikaidoh or, if biventricular repair is not possible, a staged Fontan pathway. Some babies need a temporary shunt or pulmonary artery band first.

  • Which UK centres treat DORV?

    DORV is managed at specialist commissioned paediatric cardiac surgery centres, including Great Ormond Street, Birmingham Children’s, Southampton, Bristol, Leeds, Newcastle, Alder Hey and Glasgow. Adults are followed in adult congenital heart disease (ACHD) services.

  • Will my child live a normal life?

    Outcomes have improved substantially. Many children with DORV grow up to lead active lives with school, work and family. Lifelong follow-up is essential because arrhythmia, ventricular dysfunction and conduit or valve problems can develop years after surgery.

  • Where can we find support?

    Little Hearts Matter, the British Heart Foundation and the Down Heart Group are well established UK charities offering information, peer support and family services for children and adults with complex congenital heart disease.

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