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Double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis

MedGen UID:
868834
•Concept ID:
C4023244
•
Congenital Abnormality
Synonym: DORV with subaortic VSD and pulmonary stenosis
 
HPO: HP:0011655

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 aortic origin. There is 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

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDouble outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis

Professional guidelines

PubMed

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
Pattern and clinical aspects of congenital heart diseases and their management in Cameroon.
Tantchou Tchoumi JC, Ambassa JC, Chelo D, Djimegne FK, Giamberti A, Cirri S, Kingue S, Butera G
Bull Soc Pathol Exot 2011 Feb;104(1):25-8. Epub 2011 Feb 1 doi: 10.1007/s13149-010-0091-7. PMID: 21287371
Double outlet left ventricle. Morphology, cineangiocardiographic diagnosis and surgical treatment.
Brandt PW, Calder AL, Barratt-Boyes BG, Neutze JM
Am J Cardiol 1976 Dec;38(7):897-909. doi: 10.1016/0002-9149(76)90803-1. PMID: 998525

Recent clinical studies

Etiology

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
Bi-ventricular repair of double outlet left ventricle: Experience and review of the literature.
Raja J, Menon S, Ramanan S, Dutta Baruah S, Devarakonda BV, Gopalakrishnan A, Dharan BS
J Card Surg 2020 Aug;35(8):1865-1870. Epub 2020 Jul 11 doi: 10.1111/jocs.14768. PMID: 32652736
Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension.
Wu QY, Li DH, Li HY, Zhang MK, Xu ZH, Xue H
Chin Med J (Engl) 2017 Feb 20;130(4):409-413. doi: 10.4103/0366-6999.199827. PMID: 28218213Free PMC Article
Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect.
Li S, Ma K, Hu S, Hua Z, Yan J, Pang K, Wang X, Yan F, Liu J, Zhang S, Chen Q
Eur J Cardiothorac Surg 2015 Oct;48(4):580-7; discussion 587. Epub 2015 Jan 21 doi: 10.1093/ejcts/ezu523. PMID: 25612745
Arterial switch.
Planche C, Lacour-Gayet F, Serraf A
Pediatr Cardiol 1998 Jul-Aug;19(4):297-307. doi: 10.1007/s002469900313. PMID: 9636253

Diagnosis

Surgical repair of peripheral pulmonary artery stenosis: A 2-decade experience with 145 patients.
Felmly LM, Mainwaring RD, Collins RT, Lechich K, Martin E, Ma M, Hanley FL
J Thorac Cardiovasc Surg 2023 Apr;165(4):1493-1502.e2. Epub 2022 Aug 10 doi: 10.1016/j.jtcvs.2022.07.037. PMID: 36088147
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
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
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
Prenatally Diagnosed Ventricular Inversion, Restrictive Ventricular Septal Defect, Pulmonary Stenosis, Hypertensive Left Ventricle and Double Outlet Right Ventricle: Case Report and Literature Review.
El-Asmar JM, Degheili JA, Al-Halabi E, Sklansky M
Pediatr Cardiol 2019 Jan;40(1):234-236. Epub 2018 Dec 19 doi: 10.1007/s00246-018-2048-8. PMID: 30569250

Therapy

Multicentre study on late outcomes of biventricular repair of double outlet right ventricle.
Lacour-Gayet F, Zoghbi J, Gouton M, Roussin R, Bical O, Lucet V, Saint-Pick M, Leca F
Eur J Cardiothorac Surg 2024 Jan 2;65(1) doi: 10.1093/ejcts/ezad423. PMID: 38134423
Comprehensive outcomes after primary arterial switch operation.
Nakamura M, Kanno K, Nishioka M
Gen Thorac Cardiovasc Surg 2023 Aug;71(8):447-454. Epub 2022 Dec 30 doi: 10.1007/s11748-022-01902-7. PMID: 36583825
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
Arterial Switch Operation in Patients With Taussig-Bing Anomaly and Aortic Arch Obstruction.
Fricke TA, Thungathurthi K, Naimo PS, Buratto E, d'Udekem Y, Brizard CP, Konstantinov IE
Ann Thorac Surg 2022 Sep;114(3):834-840. Epub 2021 Jun 29 doi: 10.1016/j.athoracsur.2021.05.082. PMID: 34197825
Tetralogy of Fallot in the Current Era.
Mouws EMJP, de Groot NMS, van de Woestijne PC, de Jong PL, Helbing WA, van Beynum IM, Bogers AJJC
Semin Thorac Cardiovasc Surg 2019 Autumn;31(3):496-504. Epub 2018 Nov 2 doi: 10.1053/j.semtcvs.2018.10.015. PMID: 30395964

Prognosis

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
Absent pulmonary valve with double-outlet left ventricle: a case report.
Sakai W, Asai H, Ebuoka N
Cardiol Young 2022 Nov;32(11):1864-1865. Epub 2022 Feb 28 doi: 10.1017/S1047951122000786. PMID: 35225204
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
Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect.
Li S, Ma K, Hu S, Hua Z, Yan J, Pang K, Wang X, Yan F, Liu J, Zhang S, Chen Q
Eur J Cardiothorac Surg 2015 Oct;48(4):580-7; discussion 587. Epub 2015 Jan 21 doi: 10.1093/ejcts/ezu523. PMID: 25612745
Arterial switch.
Planche C, Lacour-Gayet F, Serraf A
Pediatr Cardiol 1998 Jul-Aug;19(4):297-307. doi: 10.1007/s002469900313. PMID: 9636253

Clinical prediction guides

Comprehensive outcomes after primary arterial switch operation.
Nakamura M, Kanno K, Nishioka M
Gen Thorac Cardiovasc Surg 2023 Aug;71(8):447-454. Epub 2022 Dec 30 doi: 10.1007/s11748-022-01902-7. PMID: 36583825
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
Criss-cross heart: Transthoracic echocardiographic features.
Manuel D, Ghosh G, Joseph G, Lahiri A, George PV
Indian Heart J 2018 Jan-Feb;70(1):71-74. Epub 2017 Mar 23 doi: 10.1016/j.ihj.2017.03.008. PMID: 29455791Free PMC Article
The concept of double inlet-double outlet right ventricle: a distinct congenital heart disease.
Spadotto V, Frescura C, Ho SY, Thiene G
Cardiovasc Pathol 2017 Jan-Feb;26:39-44. Epub 2016 Sep 25 doi: 10.1016/j.carpath.2016.09.003. PMID: 27866077

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