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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 anomaly of cardiovascular system
        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

Prenatal Diagnosis of Double Aortic Arch: Associated Findings and Postnatal Clinical Outcomes.

Bornaun H, Süzen Çaypınar S, Gedik Özköse Z, Topbaş NF, Behram M
J Ultrasound Med 2021 Dec;40(12):2721-2726. Epub 2021 Mar 3 doi: 10.1002/jum.15669. PMID: 33656187

See all (29)

Recent clinical studies

Etiology

Human Genetics of Tetralogy of Fallot and Double-Outlet Right Ventricle.

Dorn C, Perrot A, Grunert M, Rickert-Sperling S
Adv Exp Med Biol 2024;1441:629-644. doi: 10.1007/978-3-031-44087-8_36. PMID: 38884738

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

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 (718)

Diagnosis

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

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

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

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

See all (725)

Therapy

Congenital heart defects caused by FOXJ1.

Padua MB, Helm BM, Wells JR, Smith AM, Bellchambers HM, Sridhar A, Ware SM
Hum Mol Genet 2023 Jul 4;32(14):2335-2346. doi: 10.1093/hmg/ddad065. PMID: 37158461 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

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

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 (213)

Prognosis

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

Josowitz R, Rogers LS
Curr Opin Cardiol 2024 Jul 1;39(4):348-355. Epub 2024 Feb 23 doi: 10.1097/HCO.0000000000001131. PMID: 38391276

Growth of the Neo-Aortic Root and Prognosis of Transposition of the Great Arteries.

Sengupta A, Carreon CK, Gauvreau K, Lee JM, Sanders SP, Colan SD, Del Nido PJ, Mayer JE Jr, Nathan M
J Am Coll Cardiol 2024 Jan 30;83(4):516-527. Epub 2023 Nov 6 doi: 10.1016/j.jacc.2023.10.023. PMID: 37939977

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

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 (529)

Clinical prediction guides

Human Genetics of Defects of Situs.

Perrot A, Rickert-Sperling S
Adv Exp Med Biol 2024;1441:705-717. doi: 10.1007/978-3-031-44087-8_42. PMID: 38884744

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

Josowitz R, Rogers LS
Curr Opin Cardiol 2024 Jul 1;39(4):348-355. Epub 2024 Feb 23 doi: 10.1097/HCO.0000000000001131. PMID: 38391276

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

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 (300)

Recent systematic reviews

Cardiovascular anomalies in patients with Tessier syndrome: a systematic review.

Nazari S, Vaezi A, Mossavarali S, Ghanavati K, Shafiee A
Eur J Pediatr 2024 Jan;183(1):73-82. Epub 2023 Nov 4 doi: 10.1007/s00431-023-05322-4. PMID: 37924347

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 (3)

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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

Consumer resources

Reviews

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