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Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis

MedGen UID:: 408331
•Concept ID:: C1956413
•: Congenital Abnormality

Synonyms:	Anomaly, Taussig-Bing; Taussig Bing Anomaly; Taussig-Bing Anomaly
SNOMED CT:	Taussig-Bing syndrome (7484005); Taussig-Bing defect (7484005)

HPO:	HP:0011658

Definition

A double outlet right ventricle with a ventricular spetal defect (a hole between the two bottom chambers (ventricles) of the heart), that is considered to be closely related to the pulmonary origin. There is not associated pulmonary stenosis, the abnormal narrowing or constriction of the pulmonary artery, in the main pulmonary artery and/or in the left or right pulmonary artery branches. [from HPO]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDouble outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Congenital cardiovascular disorder
        Double outlet right ventricle
        Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis

Professional guidelines

PubMed

Does a prenatal diagnosis affect mortality and morbidity for neonatal arterial switch operation.

Guvenc O, Beken S, Inamlik A, Albayrak E, Temur B, Basgoze S, Aydin S, Saygi M, Ödemiş E, Erek E, Toygar AK
Cardiol Young 2022 Oct;32(10):1644-1648. Epub 2022 Jan 6 doi: 10.1017/S1047951121004819. PMID: 34986909

Prenatal Diagnosis and Postnatal Outcome of Eight Cases with Criss-Cross Heart - A Multicenter Case Series.

Vorisek CN, Kurkevych A, Kuhn V, Stessig R, Ritgen J, Degenhardt J, Enzensberger C, Wolter A, Götte M, Khalil M, Akintürk H, Axt-Fliedner R
Ultraschall Med 2022 Dec;43(6):e90-e97. Epub 2020 Jul 16 doi: 10.1055/a-1205-0289. PMID: 32674186

Current options and outcomes for the management of atrioventricular septal defect.

Shuhaiber JH, Ho SY, Rigby M, Sethia B
Eur J Cardiothorac Surg 2009 May;35(5):891-900. Epub 2009 Feb 24 doi: 10.1016/j.ejcts.2009.01.009. PMID: 19243971

See all (29)

Recent clinical studies

Etiology

Double outlet ventricles: review of anatomic and imaging characteristics.

Xu Z, Semple T, Gu H, McCarthy KP, Yen Ho S, Li W
Heart 2023 May 26;109(12):905-912. doi: 10.1136/heartjnl-2022-321955. PMID: 36539269

Essential Modifiers of Double Outlet Right Ventricle: Revisit With Endocardial Surface Images and 3-Dimensional Print Models.

Yim D, Dragulescu A, Ide H, Seed M, Grosse-Wortmann L, van Arsdell G, Yoo SJ
Circ Cardiovasc Imaging 2018 Mar;11(3):e006891. doi: 10.1161/CIRCIMAGING.117.006891. PMID: 29855425

How should we diagnose and differentiate hearts with double-outlet right ventricle?

Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710

Perioperative Management of DORV.

Spaeth JP
Semin Cardiothorac Vasc Anesth 2014 Sep;18(3):281-9. Epub 2014 Mar 21 doi: 10.1177/1089253214528048. PMID: 24659409

Arterial switch.

Planche C, Lacour-Gayet F, Serraf A
Pediatr Cardiol 1998 Jul-Aug;19(4):297-307. doi: 10.1007/s002469900313. PMID: 9636253

See all (693)

Diagnosis

Essential Modifiers of Double Outlet Right Ventricle: Revisit With Endocardial Surface Images and 3-Dimensional Print Models.

Yim D, Dragulescu A, Ide H, Seed M, Grosse-Wortmann L, van Arsdell G, Yoo SJ
Circ Cardiovasc Imaging 2018 Mar;11(3):e006891. doi: 10.1161/CIRCIMAGING.117.006891. PMID: 29855425

Double-outlet right ventricle revisited.

Ebadi A, Spicer DE, Backer CL, Fricker FJ, Anderson RH
J Thorac Cardiovasc Surg 2017 Aug;154(2):598-604. Epub 2017 Mar 23 doi: 10.1016/j.jtcvs.2017.03.049. PMID: 28528718

How should we diagnose and differentiate hearts with double-outlet right ventricle?

Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710

Ivemark Syndrome.

Masiwal P, Chenthil KS, Priyadarsini B, Gnanaprakasam J, Srihari I
J Assoc Physicians India 2016 May;64(5):73-75. PMID: 27735157

Ventricular septal defect.

Spicer DE, Hsu HH, Co-Vu J, Anderson RH, Fricker FJ
Orphanet J Rare Dis 2014 Dec 19;9:144. doi: 10.1186/s13023-014-0144-2. PMID: 25523232 Free PMC Article

See all (711)

Therapy

Double outlet right ventricle in adults: Anatomic variability, surgical treatment, and late postoperative complications.

Karev E, Stovpyuk OF
J Clin Ultrasound 2022 Oct;50(8):1151-1165. doi: 10.1002/jcu.23319. PMID: 36218204

Double root translocation: Early experience on performance of translocated aortic and pulmonary root.

Agarwal V, Vaidyanathan S
Asian Cardiovasc Thorac Ann 2022 Oct;30(8):916-923. Epub 2022 Aug 15 doi: 10.1177/02184923221120751. PMID: 35971235

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

Lu T, Li J, Hu J, Huang C, Tan L, Wu Q, Wu Z
J Thorac Cardiovasc Surg 2020 Jun;159(6):2397-2403. Epub 2019 Aug 24 doi: 10.1016/j.jtcvs.2019.07.084. PMID: 31564538

Double-Outlet Right Ventricle With Noncommitted Ventricular Septal Defect and 2 Adequate Ventricles: Is Anatomical Repair Advantageous?

Villemain O, Bonnet D, Houyel L, Vergnat M, Ladouceur M, Lambert V, Jalal Z, Vouhé P, Belli E
Semin Thorac Cardiovasc Surg 2016 Spring;28(1):69-77. Epub 2016 May 11 doi: 10.1053/j.semtcvs.2016.01.007. PMID: 27568139

Pitfalls in repair of conotruncal anomalies.

Raisky O, Vouhé PR
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2013;16(1):7-12. doi: 10.1053/j.pcsu.2013.02.001. PMID: 23561812

See all (204)

Prognosis

Current outcomes of live-born children with double outlet right ventricle in Norway.

Holten-Andersen M, Lippert M, Holmstrøm H, Brun H, Døhlen G
Eur J Cardiothorac Surg 2022 Dec 2;63(1) doi: 10.1093/ejcts/ezac560. PMID: 36472441 Free PMC Article

Long-Term Results after Fallot Repair.

Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793

Double-chambered Left Ventricle: Clinical Features Comparison between Children and Adults.

Yuan SM
J Coll Physicians Surg Pak 2019 Nov;29(11):1087-1091. doi: 10.29271/jcpsp.2019.11.1087. PMID: 31659968

Arterial switch.

Planche C, Lacour-Gayet F, Serraf A
Pediatr Cardiol 1998 Jul-Aug;19(4):297-307. doi: 10.1007/s002469900313. PMID: 9636253

See all (518)

Clinical prediction guides

Current outcomes of live-born children with double outlet right ventricle in Norway.

Holten-Andersen M, Lippert M, Holmstrøm H, Brun H, Døhlen G
Eur J Cardiothorac Surg 2022 Dec 2;63(1) doi: 10.1093/ejcts/ezac560. PMID: 36472441 Free PMC Article

Double outlet right ventricle in adults: Anatomic variability, surgical treatment, and late postoperative complications.

Karev E, Stovpyuk OF
J Clin Ultrasound 2022 Oct;50(8):1151-1165. doi: 10.1002/jcu.23319. PMID: 36218204

Long-Term Results after Fallot Repair.

Kleinöder JM, Purbojo A, Blumauer R, Cuomo M, Alkassar M, Dittrich S, Cesnjevar R
Thorac Cardiovasc Surg 2022 Dec;70(S 03):e7-e14. Epub 2022 Jun 25 doi: 10.1055/s-0042-1749098. PMID: 35752174

Fetal double outlet right ventricle without heterotaxy syndrome: Diagnostic spectrum, associated extracardiac pathology and clinical outcomes.

Young AA, McBrien A, Caluseriu O, Haberer K, Wewala G, Eckersley L, Hornberger LK
Prenat Diagn 2021 Aug;41(9):1118-1126. Epub 2021 Jun 27 doi: 10.1002/pd.5979. PMID: 34118793

How should we diagnose and differentiate hearts with double-outlet right ventricle?

Bharucha T, Hlavacek AM, Spicer DE, Theocharis P, Anderson RH
Cardiol Young 2017 Jan;27(1):1-15. Epub 2016 Sep 19 doi: 10.1017/S1047951116001190. PMID: 27641710

See all (286)

Recent systematic reviews

Risk of congenital heart defects in offspring exposed to maternal diabetes mellitus: an updated systematic review and meta-analysis.

Chen L, Yang T, Chen L, Wang L, Wang T, Zhao L, Ye Z, Zhang S, Luo L, Zheng Z, Qin J
Arch Gynecol Obstet 2019 Dec;300(6):1491-1506. Epub 2019 Nov 12 doi: 10.1007/s00404-019-05376-6. PMID: 31713644

Double-chambered Left Ventricle: Clinical Features Comparison between Children and Adults.

Yuan SM
J Coll Physicians Surg Pak 2019 Nov;29(11):1087-1091. doi: 10.29271/jcpsp.2019.11.1087. PMID: 31659968

See all (2)

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Double outlet right ventricle with subpulmonary ventricular septal defect without pulmonary stenosis

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

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