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PubMed This is a summary of 20 peer-reviewed journal articles Updated
Pediatric Cardiology

Surgical Strategies and Treatment Pathways

At a Glance

Surgery is necessary to treat Double Outlet Right Ventricle (DORV) so oxygen-rich and oxygen-poor blood flow correctly. Treatment depends on a child's specific heart anatomy and may involve a single biventricular repair or a staged single-ventricle pathway.

The ultimate goal of surgery for Double Outlet Right Ventricle (DORV) is to ensure that oxygen-rich blood can reach the body and oxygen-poor blood can reach the lungs without mixing. Because every child’s heart is unique, surgeons have developed several different “pathways” to reach this goal.

Surgical Timing

When surgery happens depends heavily on your child’s specific anatomy and how well they are tolerating the altered blood flow [1][2].

  • Neonatal (Days to Weeks Old): If the baby’s circulation is completely dependent on fetal connections remaining open, or if there is severe heart failure, intervention is required soon after birth [1][3].
  • Infancy (3 to 6 Months Old): Many children with stable anatomy wait a few months to grow and gain weight before surgery [4][5].

Choosing the Pathway: Biventricular vs. Single Ventricle

Your surgical team will determine whether your child’s heart can be repaired to function with two pumping chambers (biventricular repair) or if a single-ventricle pathway is safer [6][7].

  • Biventricular Repair (BVR): This involves creating a permanent “tunnel” inside the heart to guide blood to the correct artery [4]. For BVR to work, the left ventricle must be large enough to pump blood to the entire body, and the VSD must be in a position where it can be “routed” to the aorta [8][9].
  • Single-Ventricle (SV) Pathway: If vital heart valves are in the way or a ventricle is too small, a biventricular repair might be too risky [10][11]. While it sounds intimidating, this is a calculated safety decision. Surgeons use a staged series of operations (often leading to the Fontan procedure) to allow the right ventricle to do the work for the whole heart [8][12]. Many children on a Fontan pathway go on to have excellent, active qualities of life.

Common Surgical Procedures

If your child is a candidate for a biventricular repair, the specific operation depends on their subtype:

  • Intraventricular Tunnel Repair: Often used for the “subaortic” type. The surgeon creates a tunnel (baffle) from the VSD to the aorta, connecting the left ventricle to the body’s main pipe [4][6].
  • Arterial Switch Operation (ASO): Frequently used for the Taussig-Bing type. Surgeons “switch” the positions of the two great arteries so the aorta is correctly connected [13][14].
  • Rastelli Procedure: Surgeons build a tunnel to the aorta and then use a separate tube (conduit) to connect the right ventricle to the lungs [15][16].
  • Nikaidoh Procedure: The surgeon physically moves the aortic root to sit over the left ventricle, which can be a better option than the Rastelli for some cases because it uses the heart’s own valves [17][18].

Why Staged Repairs Are Sometimes Needed

Not every heart can be fully repaired in a single surgery. Sometimes, a child needs “palliative” (temporary) procedures to stabilize their blood flow while they grow [19][5].

  • Shunt: If not enough blood is reaching the lungs, a small tube (like a modified Blalock-Taussig-Thomas shunt) is placed to provide more flow [20][19].
  • Pulmonary Artery Banding (PAB): If too much blood is rushing to the lungs, a band is placed around the artery to “tighten” it and protect the lung tissue from high pressure [20].

These early steps ensure your child is as strong as possible before their definitive repair [5].

Common questions in this guide

Is a biventricular or single-ventricle repair better for DORV?
The best pathway depends on your child's specific anatomy. Biventricular repair uses two pumping chambers, but if vital heart structures are too small or blocked, a single-ventricle pathway using staged surgeries is often the safest option.
When does a baby with DORV need surgery?
Timing depends on the child's heart anatomy and how well they tolerate altered blood flow. Newborns with severe heart failure or circulation dependency may need surgery within days, while those with stable anatomy often wait 3 to 6 months to grow.
What is the Rastelli procedure for DORV?
The Rastelli procedure is a type of biventricular repair. Surgeons build a tunnel inside the heart to route blood to the aorta and use a separate tube, or conduit, to connect the right ventricle to the lungs.
Why might my child need multiple surgeries for DORV?
Not every heart can be fully repaired in a single operation. Surgeons may use temporary procedures, like shunts or pulmonary artery banding, to stabilize blood flow and allow your child to grow stronger before their definitive repair.
What is the Arterial Switch Operation?
Surgeons switch the positions of the two great arteries so the aorta is correctly connected. This approach is frequently used for the Taussig-Bing type of DORV.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Is our child a candidate for a biventricular (two-ventricle) repair, or is a single-ventricle pathway safer for their specific anatomy?
  2. 2.What is our expected timeline for intervention?
  3. 3.If a biventricular repair is possible, which procedure do you recommend (e.g., Intraventricular Tunnel, Rastelli, Nikaidoh)?
  4. 4.Will my child need a conduit that requires future replacement?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (20)
  1. 1

    Successful surgical repair of Taussig Bing anomaly with pulmonary artery and pulmonary valve stenosis in a neonate patient: A case report.

    Barakat L, Alabdullah S, Alezzo A, et al.

    International journal of surgery case reports 2024; (125()):110462 doi:10.1016/j.ijscr.2024.110462.

    PMID: 39503103
  2. 2

    Aortic Arch Phenotypes in Double Outlet Right Ventricle (DORV)-Implications for Surgery and Multi-Modal Imaging.

    Gressani A, Aynetdinova R, Kostolny M, et al.

    Journal of cardiovascular development and disease 2022; (9(8)) doi:10.3390/jcdd9080262.

    PMID: 36005426
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    Single-Stage Correction for Taussig-Bing Anomaly Associated With Aortic Arch Obstruction.

    Luo K, Zheng J, Wang S, et al.

    Pediatric cardiology 2017; (38(8)):1548-1555 doi:10.1007/s00246-017-1694-6.

    PMID: 28752325
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    Biventricular repair of double-outlet right ventricle with noncommitted ventricular septal defect using intraventricular conduit.

    Lu T, Li J, Hu J, et al.

    The Journal of thoracic and cardiovascular surgery 2020; (159(6)):2397-2403 doi:10.1016/j.jtcvs.2019.07.084.

    PMID: 31564538
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    Repair of double outlet right ventricle: Midterm outcomes.

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    The Journal of thoracic and cardiovascular surgery 2020; (159(1)):254-264 doi:10.1016/j.jtcvs.2019.06.120.

    PMID: 31597616
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    Double Outlet Right Ventricle: In-Depth Anatomic Review Using Three-Dimensional Cardiac CT Data.

    Goo HW

    Korean journal of radiology 2021; (22(11)):1894-1908 doi:10.3348/kjr.2021.0248.

    PMID: 34564964
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    MRA-based 3D-printed heart model-an effective tool in the pre-surgical planning of DORV.

    Bharati A, Garekar S, Agarwal V, et al.

    BJR case reports 2016; (2(3)):20150436 doi:10.1259/bjrcr.20150436.

    PMID: 30459986
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    Half-turned truncal switch operation after single ventricle palliation in a patient with borderline left heart hypoplasia.

    Oh TH, Jung H, Cho JY, Lee Y

    Journal of cardiothoracic surgery 2020; (15(1)):308 doi:10.1186/s13019-020-01357-y.

    PMID: 33036641
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    Case Report: Parachute tricuspid valve in association with cyanotic congenital heart disease and juxtaposed atrial appendages in a 1-year-old boy-a rare anatomical association.

    Sangwan D, Marwah A, Jain V

    Frontiers in cardiovascular medicine 2026; (13()):1683429 doi:10.3389/fcvm.2026.1683429.

    PMID: 41798609
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    [Successful Staged Surgical Management for Double Outlet Right Ventricle with Ebstein's Anomaly and Aortic Coarctation;Report of a Case].

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    Kyobu geka. The Japanese journal of thoracic surgery 2018; (71(8)):615-621.

    PMID: 30185760
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    Functionally univentricular heart with systemic venous anomalies: surgical palliation and pulmonary arterial reconstruction with a roll of left atrial appendage.

    Talwar S, Anderson RH, George N, et al.

    Indian journal of thoracic and cardiovascular surgery 2019; (35(2)):203-207 doi:10.1007/s12055-018-0741-0.

    PMID: 33061006
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    Practical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.

    Fitzgerald NM, Singh AA, Barron DJ, et al.

    Pediatric radiology 2025; (55(11)):2272-2285 doi:10.1007/s00247-025-06233-2.

    PMID: 40227499
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    Arterial Switch Operation in Patients With Taussig-Bing Anomaly and Aortic Arch Obstruction.

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    The Annals of thoracic surgery 2022; (114(3)):834-840 doi:10.1016/j.athoracsur.2021.05.082.

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    Outcomes of arterial switch operation for Taussig-Bing anomaly versus transposition of great arteries and ventricular septal defect.

    Erek E, Suzan D, Aydın S, et al.

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    Nikaidoh vs Réparation à l'Etage Ventriculaire vs Rastelli.

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    Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual 2018; (21()):58-63 doi:10.1053/j.pcsu.2017.10.001.

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    Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis.

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    Successful Modified Nikaidoh Procedure (Pivot Rotation) in a Patient with Double Outlet Right Ventricle and Pulmonary Atresia: Case Report.

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This page provides general information about surgical strategies for Double Outlet Right Ventricle (DORV). It is not a substitute for professional medical advice; always consult your pediatric cardiologist and surgical team regarding your child's specific treatment plan.

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