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

MedGen UID:
868832
•Concept ID:
C4023242
•
Congenital Abnormality
Synonym: DORV with subpulmonary VSD and pulmonary stenosis
 
HPO: HP:0011657

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 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 subpulmonary 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
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
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
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

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
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
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
Surgical Treatment for Double Outlet Right Ventricle With Pulmonary Outflow Tract Obstruction.
Wu Q, Jin Y, Li H, Zhang M
World J Pediatr Congenit Heart Surg 2016 Nov;7(6):696-699. doi: 10.1177/2150135116674440. PMID: 27834760
Double Outlet Right Ventricle With Intact Ventricular Septum: Avulsion or Exclusion.
Menon S, Kumar CJ, Mathew T, Venkateshwarn S, Jayakumar K, Dharan BS
World J Pediatr Congenit Heart Surg 2016 Mar;7(2):220-2. Epub 2015 Nov 24 doi: 10.1177/2150135115586813. PMID: 26603090

Therapy

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
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
Ventricular Tachycardia Following Surgical Repair of Complex Congenital Heart Disease.
Baysa SJ, Kanter RJ
Card Electrophysiol Clin 2016 Mar;8(1):201-4. Epub 2016 Jan 19 doi: 10.1016/j.ccep.2015.10.029. PMID: 26920195
An additional patient with mycophenolate mofetil embryopathy: cardiac and facial analyses.
Lin AE, Singh KE, Strauss A, Nguyen S, Rawson K, Kimonis VE
Am J Med Genet A 2011 Apr;155A(4):748-56. Epub 2011 Mar 15 doi: 10.1002/ajmg.a.33934. PMID: 21594997

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
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
Early results for anatomic correction of transposition of the great arteries and for double-outlet right ventricle with subpulmonary ventricular septal defect.
Brawn WJ, Mee RB
J Thorac Cardiovasc Surg 1988 Feb;95(2):230-8. PMID: 3339890

Clinical prediction guides

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
Intraoperative Stenting of Pulmonary Artery Stenosis in Children With Congenital Heart Disease.
Meot M, Lefort B, El Arid JM, Soulé N, Lothion-Boulanger J, Lengellé F, Chantepie A, Neville P
Ann Thorac Surg 2017 Jul;104(1):190-196. Epub 2017 Mar 6 doi: 10.1016/j.athoracsur.2016.12.012. PMID: 28274523
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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