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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]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVAbnormal right ventricle morphology

Phenotypic abnormality
- Abnormality of the cardiovascular system
  - Abnormal cardiovascular system morphology
    - Abnormal heart morphology
      - Abnormal cardiac ventricle morphology
        Abnormal right ventricle morphology
        Anomalous muscle bundle of the right ventricle
        Cor pulmonale
        Fibrofatty replacement of right ventricular myocardium
        Hypoplasia of right ventricle
        Right ventricular aneurysm
        Right ventricular cardiomyopathy
        Right ventricular dilatation

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: 32354551 Free 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

See all (176)

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: 32108525 Free 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

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

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

See all (3508)

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-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

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: 17922908 Free PMC Article

See all (4045)

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: 33161457 Free 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: 27777296 Free PMC Article

See all (1153)

Prognosis

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

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: 24842361 Free 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

See all (2548)

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

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.

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

Aorto-ventricular tunnel.

McKay R
Orphanet J Rare Dis 2007 Oct 8;2:41. doi: 10.1186/1750-1172-2-41. PMID: 17922908 Free PMC Article

See all (1929)

Recent systematic reviews

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: 37092499 Free PMC Article

Sickle cell disease and ventricular myocardial strain: A systematic review.

Whipple NS, Joshi VM, Naik RJ, Mentnech T, McFarland MM, Nolan VG, Hankins JS
Pediatr Blood Cancer 2021 Jun;68(6):e28973. Epub 2021 Mar 19 doi: 10.1002/pbc.28973. PMID: 33742492 Free 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

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

See all (24)

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Abnormal right ventricle morphology

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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