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Abnormal right ventricle morphology

MedGen UID:
91036
•Concept ID:
C0344887
•
Anatomical Abnormality; 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]

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

Being born with a single cardiac ventricle: What do we tell prospective parents.
d'Udekem Y, Hutchinson D
Prenat Diagn 2022 Apr;42(4):411-418. Epub 2022 Apr 7 doi: 10.1002/pd.6121. PMID: 35278231
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

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
Cat eye syndrome.
Sharma D, Murki S, Pratap T, Vasikarla M
BMJ Case Rep 2014 May 19;2014 doi: 10.1136/bcr-2014-203923. PMID: 24842361Free PMC Article
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
Evaluating the Longevity of the Fontan Pathway.
Kelly JM, Mirhaidari GJM, Chang YC, Shinoka T, Breuer CK, Yates AR, Hor KN
Pediatr Cardiol 2020 Dec;41(8):1539-1547. Epub 2020 Nov 8 doi: 10.1007/s00246-020-02452-6. PMID: 33161457Free PMC Article
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
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

STRUCTURAL HEART ANOMALIES (REVIEW).
Osovska N, Kuzminova N, Ovcharuk M, Serhiychuk O
Georgian Med News 2016 Jun;(255):66-77. PMID: 27441539
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
Cat eye syndrome.
Sharma D, Murki S, Pratap T, Vasikarla M
BMJ Case Rep 2014 May 19;2014 doi: 10.1136/bcr-2014-203923. PMID: 24842361Free PMC Article
Double-chambered right ventricle: a review.
Loukas M, Housman B, Blaak C, Kralovic S, Tubbs RS, Anderson RH
Cardiovasc Pathol 2013 Nov-Dec;22(6):417-23. Epub 2013 May 20 doi: 10.1016/j.carpath.2013.03.004. PMID: 23701985
Uhl's anomaly.
Vecht RJ, Carmichael DJ, Gopal R, Philip G
Br Heart J 1979 Jun;41(6):676-82. doi: 10.1136/hrt.41.6.676. PMID: 465242Free PMC Article

Clinical prediction guides

Outcomes After Hybrid Palliation for Infants With Critical Left Heart Obstruction.
Argo MB, Barron DJ, Eghtesady P, Yerebakan C, DeCampli WM, Alsoufi B, Honjo O, Jacobs JP, Paramananthan T, Rahman M, Lambert LM, Jegatheeswaran A, Carrillo SA, Husain SA, Ramakrishnan K, Caldarone CA, Karamlou T, Nelson J, Mannie C, Romano JC, Turek JW, Blackstone EH, Galantowicz ME, Kirklin JK, Mitchell ME, McCrindle BW; Congenital Heart Surgeons Society Critical Left Heart Obstruction Work Group
J Am Coll Cardiol 2023 Oct 3;82(14):1427-1441. doi: 10.1016/j.jacc.2023.07.020. PMID: 37758438
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
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

Arrhythmogenic Right Ventricular Cardiomyopathy Post-Mortem Assessment: A Systematic Review.
Cianci V, Forzese E, Sapienza D, Cianci A, Ieni A, Germanà A, Guerrera MC, Omero F, Speranza D, Cracò A, Asmundo A, Gualniera P, Mondello C
Int J Mol Sci 2024 Feb 20;25(5) doi: 10.3390/ijms25052467. PMID: 38473714Free PMC Article
Cardiac ventricular false tendons: A meta-analysis.
Carrasco M, Cabrito TMS, Montalbano MJ, Hołda MK, Walocha J, Tubbs RS, Loukas M
Clin Anat 2024 Jan;37(1):114-129. Epub 2023 Oct 11 doi: 10.1002/ca.24116. PMID: 37819143
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
A Systematic Review of Acquired Left Ventricle to Right Atrium Shunts (Gerbode Defects).
Yuan SM
Hellenic J Cardiol 2015 Sep-Oct;56(5):357-72. PMID: 26429364

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