U.S. flag

An official website of the United States government


Send to:

Choose Destination

Hypoplasia of right ventricle

MedGen UID:
Concept ID:
Anatomical Abnormality
Synonym: Right ventricular hypoplasia
HPO: HP:0004762


Underdevelopment or reduced size of the heart right ventricle, often due to a reduced number of cells. [from HPO]

Term Hierarchy

Conditions with this feature

Isolated right ventricular hypoplasia
MedGen UID:
Concept ID:
Congenital Abnormality
A rare congenital heart malformation with characteristics of underdevelopment of the right ventricle associated with patent foramen ovale or interauricular communication and normally developed tricuspid and pulmonary valves. Manifests with severe cyanosis, congestive heart failure, and in severe cases, death in early infancy.
Heterotaxy, visceral, 7, autosomal
MedGen UID:
Concept ID:
Disease or Syndrome
Autosomal visceral heterotaxy-7 is an autosomal recessive developmental disorder characterized by complex congenital heart malformations and/or situs inversus and caused by defects in the normal left-right asymmetric positioning of internal organs. The phenotype is variable (summary by Guimier et al., 2015). For a discussion of the genetic heterogeneity of visceral heterotaxy, see HTX1 (306955).
Cardiac valvular defect, developmental
MedGen UID:
Concept ID:
Disease or Syndrome
Cardiac valvular dysplasia-1 (CVDP1) is characterized by congenital malformations of the pulmonic, tricuspid, and mitral valves. Structural cardiac defects, including atrial and ventricular septal defects, single left ventricle, and hypoplastic right ventricle have also been observed in affected individuals (Ta-Shma et al., 2017). Genetic Heterogeneity of Cardiac Valvular Dysplasia CVDP2 (620067) is caused by mutation in the ADAMTS19 gene (607513) on chromosome 5q23.

Professional guidelines


Corrado D, Perazzolo Marra M, Zorzi A, Beffagna G, Cipriani A, Lazzari M, Migliore F, Pilichou K, Rampazzo A, Rigato I, Rizzo S, Thiene G, Anastasakis A, Asimaki A, Bucciarelli-Ducci C, Haugaa KH, Marchlinski FE, Mazzanti A, McKenna WJ, Pantazis A, Pelliccia A, Schmied C, Sharma S, Wichter T, Bauce B, Basso C
Int J Cardiol 2020 Nov 15;319:106-114. Epub 2020 Jun 16 doi: 10.1016/j.ijcard.2020.06.005. PMID: 32561223
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
Thenappan T, Ormiston ML, Ryan JJ, Archer SL
BMJ 2018 Mar 14;360:j5492. doi: 10.1136/bmj.j5492. PMID: 29540357Free PMC Article

Recent clinical studies


Tamisier D, Ouaknine R, Pouard P, Mauriat P, Lefebvre D, Sidi D, Vouhé PR
Eur J Cardiothorac Surg 1997 May;11(5):810-7. doi: 10.1016/s1010-7940(97)01178-0. PMID: 9196293


Joy MV, Venugopalan P, Sapru A, Subramanyan R
Indian Heart J 1999 Jul-Aug;51(4):440-3. PMID: 10547948


Tamisier D, Ouaknine R, Pouard P, Mauriat P, Lefebvre D, Sidi D, Vouhé PR
Eur J Cardiothorac Surg 1997 May;11(5):810-7. doi: 10.1016/s1010-7940(97)01178-0. PMID: 9196293

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...