Double outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis

MedGen UID:: 868835
•Concept ID:: C4023245
•: Congenital Abnormality

Synonyms:	DORV with non-committed VSD without pulmonary stenosis; Double outlet right ventricle, noncommitted ventricular septal defect

HPO:	HP:0011654

Definition

A double outlet right ventricle with a non-committed ventricular septal defect (VSD), which is a VSD that is anatomically related to, or close to, neither great vessel, being separated from both by considerable muscle, but there is not accompanying 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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVDouble outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis

Phenotypic abnormality
- Abnormality of prenatal development or birth
  - Fetal anomaly
    - Congenital Systemic Disorder
      - Congenital anomaly of cardiovascular system
        Double outlet right ventricle
        Double outlet right ventricle with non-committed ventricular septal defect without pulmonary stenosis

Recent clinical studies

Etiology

Multicentre study on late outcomes of biventricular repair of double outlet right ventricle.

Lacour-Gayet F, Zoghbi J, Gouton M, Roussin R, Bical O, Lucet V, Saint-Pick M, Leca F
Eur J Cardiothorac Surg 2024 Jan 2;65(1) doi: 10.1093/ejcts/ezad423. PMID: 38134423

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

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

Artrip JH, Sauer H, Campbell DN, Mitchell MB, Haun C, Almodovar MC, Hraska V, Lacour-Gayet F
Eur J Cardiothorac Surg 2006 Apr;29(4):545-50. Epub 2006 Feb 21 doi: 10.1016/j.ejcts.2005.12.038. PMID: 16495067

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch.

Lacour-Gayet F, Haun C, Ntalakoura K, Belli E, Houyel L, Marcsek P, Wagner F, Weil J
Eur J Cardiothorac Surg 2002 Jun;21(6):1042-8. doi: 10.1016/s1010-7940(02)00105-7. PMID: 12048084

Double-outlet right ventricle with non-committed ventricular septal defect.

Belli E, Serraf A, Lacour-Gayet F, Hubler M, Zoghby J, Houyel L, Planche C
Eur J Cardiothorac Surg 1999 Jun;15(6):747-52. doi: 10.1016/s1010-7940(99)00100-1. PMID: 10431853

See all (6)

Therapy

Multicentre study on late outcomes of biventricular repair of double outlet right ventricle.

Lacour-Gayet F, Zoghbi J, Gouton M, Roussin R, Bical O, Lucet V, Saint-Pick M, Leca F
Eur J Cardiothorac Surg 2024 Jan 2;65(1) doi: 10.1093/ejcts/ezad423. PMID: 38134423

See all (1)

Prognosis

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

Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification.

Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery.

Busquet J, Fontan F, Choussat A, Caianiello G, Fernandez G
Eur J Cardiothorac Surg 1988;2(3):176-84. doi: 10.1016/1010-7940(88)90068-1. PMID: 3272219

See all (3)