Definition and research guide
This page combines a MeSH definition, publication statistics and selected research abstracts. It is a starting point for reading the literature, not a medical textbook or a systematic evidence review.
Content available: 4 complete abstracts (834 words), 10 references. Full article text is not stored or reproduced here; follow the PMID, DOI or PMC links to read the original sources and their access terms.
Definition
Incomplete transposition of the great vessels in which both the aorta and the pulmonary artery arise from the right ventricle. The only outlet of the left ventricle is a large ventricular septal defect (ventricular septal defects or VSD). The various subtypes are classified by the location of the septal defect, such as subaortic, subpulmonary, or noncommitted.
Definition: NLM Medical Subject Headings (MeSH) scope note (Public domain (U.S. National Library of Medicine); attribution requested). View the MeSH record.
Also known as: Double-Outlet Right Ventricle; Double-Outlet Right Ventricles; Right Ventricle, Double-Outlet; Ventricle, Double-Outlet Right; Taussig-Bing Anomaly; Anomaly, Taussig-Bing; Taussig Bing Anomaly; Double Outlet Right Ventricle, Subaortic VSD.
Where it fits in the MeSH classification
- Broader topics
- Heart Septal Defects, Ventricular; Transposition of Great Vessels
Related concepts
- Aorta
- Pulmonary Artery
- Right Ventricle
- Left Ventricle
- Ventricular Septal Defects
Research landscape
PubMed indexes 917 records tagged with the MeSH heading “Double Outlet Right Ventricle” (counts retrieved 2026-10-29). 154 of them (16.8%) were published from 2020 onward, and the busiest period so far is 2010-2019 with 275 records.
Reviews account for 71 records (7.7%) and meta-analyses for 0. Randomized controlled trials number 1 (0.1%) as a share of all records on the topic. Randomized trials make up only a small share of this literature, so much of what is known comes from laboratory, observational or descriptive work.
Show the data
| Period | Records |
|---|---|
| through 1999 | 249 |
| 2000-2009 | 239 |
| 2010-2019 | 275 |
| 2020 onward | 154 |
Publication-type chart data
| Type | Records | Share |
|---|---|---|
| Reviews | 71 | 7.7% |
| Meta-analyses | 0 | 0.0% |
| Randomized controlled trials | 1 | 0.1% |
Method: PubMed query "Double Outlet Right Ventricle"[MeSH Terms] with publication-type and date filters, retrieved 2026-10-29.
Reading the evidence in rare and inherited diseases
Rare conditions are difficult to study: few patients means small studies, case reports and registries are common, and randomized trials are the exception. Small samples limit precision and can exaggerate effects, and cases reported in the literature may be the most severe or unusual. Evidence from many small studies is best read together. Check how patients were identified, how many were included and how long they were followed.
Evidence from open-access studies
These open-access studies come from PubMed’s best-match results for this heading (papers whose main topic it is), ranked here by citation count, with one recent addition. Each abstract is reproduced in full under its recorded open licence so that you can read the authors’ own words. They illustrate the literature; they are not a systematic summary of it. Citation counts measure attention, not reliability or clinical benefit.
Genome and epigenome analysis of monozygotic twins discordant for congenital heart disease [1]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundCongenital heart disease (CHD) is the leading non-infectious cause of death in infants.
- Closing excerpt
- ConclusionsThe results showed a high correlation between hypermethylated promoters at ZIC3 and NR2F2 and down-regulated gene expression levels of these two genes in patients with DORV compared to normal controls, providing new insight into the potential mechanism of this rare form of CHD.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (185 words)
BackgroundCongenital heart disease (CHD) is the leading non-infectious cause of death in infants. Monozygotic (MZ) twins share nearly all of their genetic variants before and after birth. Nevertheless, MZ twins are sometimes discordant for common complex diseases. The goal of this study is to identify genomic and epigenomic differences between a pair of twins discordant for a form of congenital heart disease, double outlet right ventricle (DORV).ResultsA monoamniotic monozygotic (MZ) twin pair discordant for DORV were subjected to genome-wide sequencing and methylation analysis. We identified few genomic differences but 1566 differentially methylated regions (DMRs) between the MZ twins. Twenty percent (312/1566) of the DMRs are located within 2 kb upstream of transcription start sites (TSS), containing 121 binding sites of transcription factors. Particularly, ZIC3 and NR2F2 are found to have hypermethylated promoters in both the diseased twin and additional patients suffering from DORV.ConclusionsThe results showed a high correlation between hypermethylated promoters at ZIC3 and NR2F2 and down-regulated gene expression levels of these two genes in patients with DORV compared to normal controls, providing new insight into the potential mechanism of this rare form of CHD.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 29866040 · DOI · PMC5987557
Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience [2]
Abstract reading summary — source excerpts
- Opening excerpt
- BackgroundWe reviewed our 20-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) or double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) to assess the early and long-term outcomes.
- Closing excerpt
- Close long-term surveillance is mandatory to detect structural or hemodynamic changes.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (259 words)
BackgroundWe reviewed our 20-year experience with arterial switch operation (ASO) for transposition of the great arteries (TGA) or double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly) to assess the early and long-term outcomes.MethodsBetween January 1995 and December 2014, 139 consecutive patients who underwent ASO for TGA or Taussig-Bing anomaly were included in this retrospective study. The median age at the operation was 9 (0-485) days, and 97 patients (70 %) underwent ASO less than 2 weeks. The median weight was 3.3 (2.1-10.3) kg. The patients were divided into three groups; simple TGA (n = 78) included patients with TGA with intact ventricular septum, complex TGA (n = 46) included those who had TGA with ventricular septal defect or other anomalies, and Taussig-Bing anomaly (n = 15). Median follow-up duration was 72.5 (0.4-230) months.ResultsThere were 3(2.2 %) in-hospital deaths. One patient (0.7 %) underwent early reoperation due to coronary insufficiency. Late deaths occurred in 3 (2.2 %) of 136 survivors. The Kaplan-Meier's survival rate was 97.6 ± 1.4 % at 15 years. Twenty-three patients (16.9 %) required 26 reintervention. The freedom from reintervention rates were 82.5 ± 3.7 % at 5 years and 75.8 ± 4.7 % at 10 years, respectively. Median interval between ASO and first reintervention was 22.8 (6.4-89.2) months. The multivariate analysis showed that diagnosis of Taussig-Bing anomaly (hazard ratio, 7.09; P ConclusionsASO can be performed with a low risk of early mortality and satisfactory long-term outcomes even in a small volume center. Close long-term surveillance is mandatory to detect structural or hemodynamic changes.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 26911711 · DOI · PMC4766671
Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography [3]
Abstract reading summary — source excerpts
- Opening excerpt
- PurposeTo evaluate the feasibility and diagnostic accuracy of retrospective electrocardiographically (ECG)-gated dual-source computed tomography (DSCT) for the assessment of double outlet right ventricle (DORV) and associated multiple malformations in pediatric patients.
- Closing excerpt
- Combined with TTE, it may reduce or even obviate the use of invasive cardiac catheterization, and thus expose the patients to a much lower radiation dose.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (174 words)
PurposeTo evaluate the feasibility and diagnostic accuracy of retrospective electrocardiographically (ECG)-gated dual-source computed tomography (DSCT) for the assessment of double outlet right ventricle (DORV) and associated multiple malformations in pediatric patients.Materials and methodsForty-seven patients ResultsApart from DORV, 109 associated malformations were confirmed postoperatively. There was excellent agreement (κ = 0.90) for the diagnosis of associated malformations between DSCT and TTE. However, DSCT was superior to TTE in demonstrating paracardiac anomalies (sensitivity, coronary artery anomalies: 100% vs. 80.00%, anomalies of great vessels: 100% vs. 88.57%, separate thoracic and abdominal anomalies: 100% vs. 76.92%, respectively). Combined with TTE, DSCT can achieve excellent diagnostic performance in intracardiac anomalies (sensitivity, 91.30% vs. 100%). The mean image quality score was 3.70 ± 0.46 (κ = 0.76). The estimated mean effective dose was ConclusionsRetrospective ECG-gated DSCT is a better diagnostic tool than TTE for pediatric patients with complex congenital heart disease such as DORV. Combined with TTE, it may reduce or even obviate the use of invasive cardiac catheterization, and thus expose the patients to a much lower radiation dose.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 26115034 · DOI · PMC4482600
Printed Models for Better Prediction of Surgery in Patients with Double Outlet Right Ventricle [4]
Abstract reading summary — source excerpts
- Opening excerpt
- The complex and variable anatomy of complex double outlet right ventricle makes it imperative to understand the spatial anatomic structures to determine whether it is feasible to repair the anomaly in a biventricular or univentricular fashion.
- Closing excerpt
- 3D-prints are strongly recommended in patients with complex double outlet right ventricle.
- Interpretation limits
- Verbatim excerpts from the authors’ abstract; the closing excerpt may describe a finding, limitation or recommendation. Design, population, effect size and uncertainty have not been independently extracted or appraised. Read the complete abstract and linked paper before interpreting the findings.
Read complete abstract (216 words)
The complex and variable anatomy of complex double outlet right ventricle makes it imperative to understand the spatial anatomic structures to determine whether it is feasible to repair the anomaly in a biventricular or univentricular fashion. Biventricular repair should be aimed for but is not always feasible. Choosing the correct surgical technique is of great importance in surgical planning of biventricular repair. Conventional imaging is typically insufficient to predict feasibility and technique of biventricular repair. The gap between virtual images and spatial reality can be filled using 3D prints. Retrospective observational study of all available imaging including 3D prints and operative reports in 13 patients. 3D-prints enabled accurate prediction of biventricular repair in 8 cases and of univentricular repair in 2 cases. In 2 patients, no precise prediction was possible. One 3D-print was created post repair. 3D-prints accurately predicted the optimal technique for achieving biventricular repair in 8 cases. Conventional imaging could not accurately predict biventricular repair of optimal surgical technique in any patient. In complex double outlet right ventricle, 3D-printing can predict feasibility of biventricular repair better than conventional imaging. Additionally, 3D-prints can predict the type of surgical technique better. 3D-printing is also helpful in preoperative discussion with parents, caretakers, and pediatric cardiologists. 3D-prints are strongly recommended in patients with complex double outlet right ventricle.
Abstract reproduced from the open-access article under the recorded cc by licence; © the authors. PMID 39762516 · DOI · PMC12827401
Key reviews and meta-analyses
The most cited review-type papers among PubMed’s best-match results for this heading, listed with bibliographic details only. Follow the links for the full text and access terms.
- Research article Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle [5]
- Research article Double outlet right ventricle: aetiologies and associations [6]
- Research article Essential Modifiers of Double Outlet Right Ventricle: Revisit With Endocardial Surface Images and 3-Dimensional Print Models [7]
- Research article Evans syndrome in a patient with chromosome 22q11.2 deletion syndrome: a case report [8]
- Research article Anatomy, echocardiography, and surgical approach to double outlet right ventricle [9]
- Research article Continuing medical education. Double outlet right ventricle [10]
References
- Lyu G, Zhang C, Ling T, Liu R, Zong L, Guan Y, et al.. Genome and epigenome analysis of monozygotic twins discordant for congenital heart disease. . 2018. PMID 29866040 · DOI · PMC5987557
- Shim MS, Jun TG, Yang JH, Park PW, Kang IS, Huh J, et al.. Current expectations of the arterial switch operation in a small volume center: a 20-year, single-center experience. . 2016. PMID 26911711 · DOI · PMC4766671
- Shi K, Yang ZG, Chen J, Zhang G, Xu HY, Guo YK. Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography. . 2015. PMID 26115034 · DOI · PMC4482600
- Hoogerbeets SF, Roest AAW, Valverde I, Gomez-Ciriza G, Kroft L, Hazekamp MG. Printed Models for Better Prediction of Surgery in Patients with Double Outlet Right Ventricle. . 2026. PMID 39762516 · DOI · PMC12827401
- Walters HL, Mavroudis C, Tchervenkov CI, Jacobs JP, Lacour-Gayet F, Jacobs ML. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. 2000. PMID 10798433 · DOI
- Obler D, Juraszek AL, Smoot LB, Natowicz MR. Double outlet right ventricle: aetiologies and associations. J Med Genet. 2008. PMID 18456715 · DOI
- Yim D, Dragulescu A, Ide H, Seed M, Grosse-Wortmann L, van Arsdell G, et al.. Essential Modifiers of Double Outlet Right Ventricle: Revisit With Endocardial Surface Images and 3-Dimensional Print Models. Circ Cardiovasc Imaging. 2018. PMID 29855425 · DOI
- Kratz CP, Niehues T, Lyding S, Heusch A, Janssen G, Göbel U. Evans syndrome in a patient with chromosome 22q11.2 deletion syndrome: a case report. Pediatr Hematol Oncol. 2003. PMID 12554529 · DOI
- Mahle WT, Martinez R, Silverman N, Cohen MS, Anderson RH. Anatomy, echocardiography, and surgical approach to double outlet right ventricle. Cardiol Young. 2008. PMID 19094378 · DOI
- Anderson RH, McCarthy K, Cook AC. Continuing medical education. Double outlet right ventricle. Cardiol Young. 2001. PMID 11388629 · DOI
About this article
- Sources
- NLM Medical Subject Headings (definition, synonyms); NCBI PubMed (counts, identifiers); Europe PMC (open-access abstracts and citation counts).
- Verification
- Every reference was resolved against PubMed on 2026-10-31; title, year and DOI matched.
- Review state
- automated source verified; no human expert review has been recorded yet.
- Version
- 1, last updated 2026-10-29.
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Evidence strength
EvidenceHelix's computed view of the study designs behind this topic: how common meta-analyses, randomized trials and reviews are, how recent the literature is, and a plain-language reading. It describes the design mix of the literature. It is not a quality appraisal of any study, and it is not medical advice.