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Abnormal right ventricle morphology

MedGen UID:
91036
•Concept ID:
C0344887
•
Congenital Abnormality
Synonyms: Abnormality of the right ventricle; Right ventricle abnormality; Right ventricular abnormality; RV - Right ventricular abnormality
SNOMED CT: Right ventricle abnormality (253516002); RV - Right ventricular abnormality (253516002); Right ventricular abnormality (253516002)
 
HPO: HP:0001707

Definition

An abnormality of the right ventricle of the heart. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal right ventricle morphology

Professional guidelines

PubMed

Surgical Strategies in Single Ventricle Management of Neonates and Infants.
Haller C, Barron DJ
Can J Cardiol 2022 Jul;38(7):909-920. Epub 2022 May 2 doi: 10.1016/j.cjca.2022.04.021. PMID: 35513174
Molecular Approaches in Single Ventricle Management.
Toubat O, Kumar SR
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020;23:77-85. doi: 10.1053/j.pcsu.2020.03.003. PMID: 32354551Free PMC Article
Lymphatic Management in Single-Ventricle Patients.
Itkin M, Pizarro C, Radtke W, Spurrier E, Rabinowitz DA
Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2020;23:41-47. doi: 10.1053/j.pcsu.2020.03.001. PMID: 32354546

Recent clinical studies

Etiology

Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.
Garcia AM, Beatty JT, Nakano SJ
Am J Physiol Heart Circ Physiol 2020 Apr 1;318(4):H947-H965. Epub 2020 Feb 28 doi: 10.1152/ajpheart.00518.2019. PMID: 32108525Free PMC Article
The Forgotten Ventricle? The Left Ventricle in Right-Sided Congenital Heart Disease.
Tretter JT, Redington AN
Circ Cardiovasc Imaging 2018 Mar;11(3):e007410. doi: 10.1161/CIRCIMAGING.117.007410. PMID: 29855426
Origins and consequences of congenital heart defects affecting the right ventricle.
Woudstra OI, Ahuja S, Bokma JP, Bouma BJ, Mulder BJM, Christoffels VM
Cardiovasc Res 2017 Oct 1;113(12):1509-1520. doi: 10.1093/cvr/cvx155. PMID: 28957538
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
Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions.
Ohye RG, Schranz D, D'Udekem Y
Circulation 2016 Oct 25;134(17):1265-1279. doi: 10.1161/CIRCULATIONAHA.116.022816. PMID: 27777296Free PMC Article

Diagnosis

Outcomes in Hypoplastic Left Heart Syndrome.
Metcalf MK, Rychik J
Pediatr Clin North Am 2020 Oct;67(5):945-962. doi: 10.1016/j.pcl.2020.06.008. PMID: 32888691
The Forgotten Ventricle? The Left Ventricle in Right-Sided Congenital Heart Disease.
Tretter JT, Redington AN
Circ Cardiovasc Imaging 2018 Mar;11(3):e007410. doi: 10.1161/CIRCIMAGING.117.007410. PMID: 29855426
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
Quadrichambered ventricles.
Akdemir R, Balci MM, Ozer C, Karcaaltincaba M, Acikel S
Cardiol J 2010;17(3):303-5. PMID: 20535723
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

Therapy

The Secrets of the Frogs Heart.
Corno AF, Zhou Z, Uppu SC, Huang S, Marino B, Milewicz DM, Salazar JD
Pediatr Cardiol 2022 Oct;43(7):1471-1480. Epub 2022 Mar 15 doi: 10.1007/s00246-022-02870-8. PMID: 35290490
Failing systemic right ventricle: to treat or not to treat.
Dos-Subirà L
Heart 2021 Sep;107(17):1360-1361. Epub 2021 Jun 4 doi: 10.1136/heartjnl-2021-319337. PMID: 34088765
Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations.
Garcia AM, Beatty JT, Nakano SJ
Am J Physiol Heart Circ Physiol 2020 Apr 1;318(4):H947-H965. Epub 2020 Feb 28 doi: 10.1152/ajpheart.00518.2019. PMID: 32108525Free PMC Article
Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management.
Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757
Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions.
Ohye RG, Schranz D, D'Udekem Y
Circulation 2016 Oct 25;134(17):1265-1279. doi: 10.1161/CIRCULATIONAHA.116.022816. PMID: 27777296Free PMC Article

Prognosis

Cardiac imaging in Ebstein anomaly.
Qureshi MY, O'Leary PW, Connolly HM
Trends Cardiovasc Med 2018 Aug;28(6):403-409. Epub 2018 Jan 12 doi: 10.1016/j.tcm.2018.01.002. PMID: 29409687
Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management.
Brida M, Diller GP, Gatzoulis MA
Circulation 2018 Jan 30;137(5):508-518. doi: 10.1161/CIRCULATIONAHA.117.031544. PMID: 29378757
Current Therapy for Hypoplastic Left Heart Syndrome and Related Single Ventricle Lesions.
Ohye RG, Schranz D, D'Udekem Y
Circulation 2016 Oct 25;134(17):1265-1279. doi: 10.1161/CIRCULATIONAHA.116.022816. PMID: 27777296Free PMC Article
Right heart and pulmonary vessels structure and function.
D'Alto M, Scognamiglio G, Dimopoulos K, Bossone E, Vizza D, Romeo E, Vonk-Noordergraaf A, Gaine S, Peacock A, Naeije R
Echocardiography 2015 Jan;32 Suppl 1:S3-10. Epub 2014 Sep 19 doi: 10.1111/echo.12227. PMID: 25244348
Double-chambered right ventricle.
Cabrera A, Martinez P, Rumoroso JR, Alcibar J, Arriola J, Pastor E, Galdeano JM
Eur Heart J 1995 May;16(5):682-6. doi: 10.1093/oxfordjournals.eurheartj.a060973. PMID: 7588901

Clinical prediction guides

Coronary-Cameral Fistula.
Boyle S, Jesuthasan LSB, Jenkins C, Challa P, Ranjan S, Dahiya A
Circ Cardiovasc Imaging 2019 May;12(5):e008691. doi: 10.1161/CIRCIMAGING.118.008691. PMID: 31088151
Cardiac imaging in Ebstein anomaly.
Qureshi MY, O'Leary PW, Connolly HM
Trends Cardiovasc Med 2018 Aug;28(6):403-409. Epub 2018 Jan 12 doi: 10.1016/j.tcm.2018.01.002. PMID: 29409687
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
Right heart and pulmonary vessels structure and function.
D'Alto M, Scognamiglio G, Dimopoulos K, Bossone E, Vizza D, Romeo E, Vonk-Noordergraaf A, Gaine S, Peacock A, Naeije R
Echocardiography 2015 Jan;32 Suppl 1:S3-10. Epub 2014 Sep 19 doi: 10.1111/echo.12227. PMID: 25244348
Aorto-ventricular tunnel.
McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908Free PMC Article

Recent systematic reviews

Electrocardiographic Predictors of Atrial Fibrillation.
Chousou PA, Chattopadhyay R, Tsampasian V, Vassiliou VS, Pugh PJ
Med Sci (Basel) 2023 Apr 7;11(2) doi: 10.3390/medsci11020030. PMID: 37092499Free PMC Article
Double-chambered Right Ventricle in Children.
Yuan SM
J Coll Physicians Surg Pak 2019 Dec;29(12):1193-1198. doi: 10.29271/jcpsp.2019.12.1193. PMID: 31839094
Comparison of extracardiac conduit and lateral tunnel for functional single-ventricle patients: A meta-analysis.
Lin Z, Ge H, Xue J, Wu G, Du J, Hu X, Zhao Q
Congenit Heart Dis 2017 Dec;12(6):711-720. Epub 2017 Aug 28 doi: 10.1111/chd.12503. PMID: 28845580
Impact of Era, Type of Isomerism, and Ventricular Morphology on Survival in Heterotaxy: Implications for Therapeutic Management.
Loomba RS, Nijhawan K, Anderson R
World J Pediatr Congenit Heart Surg 2016 Jan;7(1):54-62. doi: 10.1177/2150135115601831. PMID: 26714995
Complex Ebstein's malformation: defining preoperative cardiac anatomy and function.
Negoi RI, Ispas AT, Ghiorghiu I, Filipoiu F, Negoi I, Hostiuc M, Hostiuc S, Ginghina C
J Card Surg 2013 Jan;28(1):70-81. doi: 10.1111/jocs.12032. PMID: 23330581

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