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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Pediatric Cardiology

The Anatomy and Subtypes of DORV

At a Glance

Double Outlet Right Ventricle (DORV) is a congenital heart condition where both major arteries exit the right ventricle. It is classified into four main subtypes based on the location of the hole between the ventricles, which helps surgeons determine the best repair approach for your child.

To understand Double Outlet Right Ventricle (DORV), it helps to think of the heart as a house where the plumbing was installed slightly out of place. While a typical heart has one “exit pipe” (artery) for each of the two bottom rooms (ventricles), in DORV, both pipes exit from the right room [1][2]. Because every child’s “house” is built a little differently, DORV is considered a spectrum—meaning the exact layout of the pipes and the holes in the walls varies from child to child [1].

The Role of the “Conal Muscle”

You may hear your doctors talk about the conus or conal muscle (also called the infundibulum). In a typical heart, this special muscle usually disappears under the aortic valve during development, allowing the aorta to sit deep in the heart, tucked right next to the left ventricle [1].

In DORV, this muscle does not disappear; it persists and grows under both the aorta and the pulmonary artery [1]. This extra “floor” of muscle physically pushes the arteries upward and forward, causing them both to sit on top of the right ventricle instead of their usual spots [1][3].

The Four Main Types of DORV

The most important feature for your child’s surgical team is the location of the Ventricular Septal Defect (VSD)—the hole in the wall between the two bottom chambers. Surgeons classify DORV based on which pipe that hole is closest to [4][5]:

  1. Subaortic VSD: The hole is located directly beneath the aortic valve. This is the most common type. It often behaves like a condition called Tetralogy of Fallot, especially if there is narrowing in the path to the lungs [6][5].
  2. Subpulmonary VSD (Taussig-Bing Anomaly): The hole is located beneath the pulmonary valve. This means oxygen-rich blood from the left side is “aimed” at the pipe going to the lungs, while oxygen-poor blood is sent out to the body. This is a more complex type that often requires a surgery similar to what is done for Transposition of the Great Arteries (TGA) [7][6].
  3. Doubly Committed VSD: The hole is large and sits right under both the aortic and pulmonary valves.
  4. Non-committed (Remote) VSD: The hole is not near either valve. It may be tucked away in a corner of the heart wall, making it more challenging for surgeons to build a “tunnel” (called a baffle) from the hole to the aorta [8][9].

Associated Anomalies: The “Company” DORV Keeps

DORV rarely travels alone. Other heart “miswirings” often occur at the same time [10]:

  • Pulmonary Stenosis: A narrowing of the path to the lungs. This limits blood flow to the lungs but can sometimes “protect” the lungs from too much pressure [11][12].
  • Aortic Arch Issues: Sometimes the main pipe to the body (the aorta) is too narrow (coarctation) or is interrupted entirely, requiring surgical repair early in life [13][14].
  • Mitral Valve Issues: The valve on the left side of the heart may be small or abnormally shaped, which can affect how the surgeon plans the repair [5].
  • Heterotaxy Syndrome (Atrial Isomerism): An arrangement where internal organs are located on the wrong side of the body. This is a complex but important association, particularly in cases needing single-ventricle palliation [8][15].
  • Chromosomal Associations: Because the heart develops at the same time as many other systems, DORV is sometimes associated with genetic changes, such as Trisomy 18 or 22q11.2 deletion syndrome [16][17].

Common questions in this guide

What are the four main types of DORV?
The four main types are based on the location of the ventricular septal defect (VSD), the hole between the heart's bottom chambers. The types are subaortic, subpulmonary (Taussig-Bing anomaly), doubly committed, and non-committed or remote VSD.
What is the conal muscle and why does it matter in DORV?
The conal muscle is a band of tissue that normally disappears as a baby's heart develops, but in DORV, it continues to grow. This extra muscle physically pushes the aorta and pulmonary artery out of their normal places, causing them both to sit on top of the right ventricle.
How does the VSD location affect surgery for DORV?
The location of the VSD is the most important factor in surgical planning. It dictates how the surgeon will build a tunnel, called a baffle, to successfully direct oxygen-rich blood from the left side of the heart over to the aorta.
Does DORV usually occur with other heart problems?
Yes, DORV rarely occurs on its own. It is frequently accompanied by other heart conditions, such as pulmonary stenosis (a narrowing of the path to the lungs), a narrow aortic arch, mitral valve issues, or heterotaxy syndrome.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Can you show me on a diagram where the VSD (hole) is located in my child's heart?
  2. 2.Does my child have pulmonary stenosis (narrowing) or any issues with their aortic arch?
  3. 3.How does the presence of the conal muscle under both valves affect the surgical plan?
  4. 4.Will my child's VSD need to be enlarged during surgery to make sure blood can flow easily to the aorta?

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 (17)
  1. 1

    Double outlet right ventricle - the 50% rule has always been about the conus.

    Josowitz R, Rogers LS

    Current opinion in cardiology 2024; (39(4)):348-355 doi:10.1097/HCO.0000000000001131.

    PMID: 38391276
  2. 2

    Conotruncal Anomalies of the Fetus.

    Gottschalk I, Menzel T, Berg C

    Ultraschall in der Medizin (Stuttgart, Germany : 1980) 2025; (46(6)):536-559 doi:10.1055/a-2620-4784.

    PMID: 40921165
  3. 3

    ETS1 loss in mice impairs cardiac outflow tract septation via a cell migration defect autonomous to the neural crest.

    Lin L, Pinto A, Wang L, et al.

    Human molecular genetics 2022; (31(24)):4217-4227 doi:10.1093/hmg/ddac174.

    PMID: 35899771
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    Congenital Conundrum: Unraveling the Puzzle of Double Outlet Right Ventricle for the Neonatologist.

    Urbina T, Graham B, Tang R, et al.

    NeoReviews 2025; (26(7)):e477-e489 doi:10.1542/neo.26-7-034.

    PMID: 40588272
  5. 5

    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
  6. 6

    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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    Patient after correction Taussig-Bing anomaly with severe neo-aortic regurgitation after sudden cardiac arrest.

    Bielecka-Dabrowa A, Bikiewicz A, Rybak M, et al.

    Clinical case reports 2021; (9(12)):e05085 doi:10.1002/ccr3.5085.

    PMID: 34925830
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    Impact of anatomic characteristics and initial biventricular surgical strategy on outcomes in various forms of double-outlet right ventricle.

    Villemain O, Belli E, Ladouceur M, et al.

    The Journal of thoracic and cardiovascular surgery 2016; (152(3)):698-706.e3.

    PMID: 27345579
  9. 9

    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
  10. 10

    Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center.

    Gottschalk I, Abel JS, Menzel T, et al.

    Journal of perinatal medicine 2019; (47(3)):354-364.

    PMID: 30676006
  11. 11

    Double-Outlet Right Ventricle, Pulmonary Atresia, and Discontinuous Branch Pulmonary Arteries Supplied by Bilateral Ducti.

    Shibbani K, Thattaliyath B, Bunker M, et al.

    JACC. Case reports 2021; (3(9)):1236-1240 doi:10.1016/j.jaccas.2021.04.020.

    PMID: 34401767
  12. 12

    Born to be Bad: Combination of Aorticopulmonary Window With Double-Outlet Right Ventricle-Pulmonary Atresia.

    Öztürk M, Ertuğrul İ, Alpat Ş, et al.

    Echocardiography (Mount Kisco, N.Y.) 2025; (42(5)):e70170 doi:10.1111/echo.70170.

    PMID: 40285522
  13. 13

    [Ross-Konnno Procedure for the Patient of Intractable Subaortic Stenosis after Operation of Double Outlet Right Ventricle with Coarctation of the Aorta and Subaortic Stenosis:Report of a Case].

    Suzuki A, Aoki M, Hagino I

    Kyobu geka. The Japanese journal of thoracic surgery 2021; (74(12)):1004-1007.

    PMID: 34795142
  14. 14

    Aortopulmonary Window With Pulmonary Atresia and Interrupted Aortic Arch: A Very Rare Triad.

    Shahbah DA, Herrick NL, El-Said H, et al.

    World journal for pediatric & congenital heart surgery 2019; (10(6)):791-792 doi:10.1177/2150135119872199.

    PMID: 31701829
  15. 15

    Long-Term Surgical Outcomes in Double Outlet Right Ventricle Based on Detailed Anatomical Sub-Typology.

    Pang K, Yang K, Wang R, et al.

    European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery 2025; (67(10)) doi:10.1093/ejcts/ezaf334.

    PMID: 41056413
  16. 16

    Double outlet right ventricle in the setting of hypoplastic left ventricle, mitral atresia, interruption of aortic arch, and uncommon intra-atrial anomalies in Trisomy 18.

    Farruggio S, Caruso E

    Echocardiography (Mount Kisco, N.Y.) 2021; (38(8)):1359-1364 doi:10.1111/echo.15142.

    PMID: 34286883
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    The accuracy of prenatal diagnosis of major congenital heart disease is increasing.

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    PMID: 31455124

This page provides educational information about DORV anatomy and subtypes. Always consult your child's pediatric cardiologist and surgical team for specific anatomical details and treatment plans.

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