両大血管右室起始症 double outlet right ventricle
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/04/05 21:17:47」(JST)
[Wiki en表示]
| Double outlet right ventricle |
| Classification and external resources |
| ICD-10 |
Q20.1 |
| ICD-9 |
745.11 |
| OMIM |
217095 |
| DiseasesDB |
32215 |
| MedlinePlus |
007328 |
| eMedicine |
ped/2509 ped/2508 |
| MeSH |
D004310 |
Double outlet right ventricle (DORV) is a form of congenital heart disease where both of the great arteries connect (in whole or in part) to the right ventricle (RV). In some cases it is found that this occurs on the left side of the heart rather than the right side.
Contents
- 1 Anatomy and pathophysiology
- 2 Treatment
- 3 Epidemiology
- 4 References
- 5 External links
Anatomy and pathophysiology
DORV occurs in a multiple forms, with variability of great artery position and size, as well as of ventricular septal defect (VSD) location. It can occur with or without transposition of the great arteries. The clinical manifestations are similarly variable, depending on how the anatomical defects affect the physiology of the heart, in terms of altering the normal flow of blood from the RV and left ventricle (LV) to the aorta and pulmonary artery. For example:
-
- in DORV with a subaortic VSD, blood from the LV flows through the VSD to the aorta and blood from the RV flows mainly to the pulmonary artery, yielding physiology similar to ventricular septal defect
- in DORV with a subpulmonic VSD (called Taussig-Bing syndrome), blood from the LV flows through the VSD to the pulmonary artery and blood from the RV flows mainly to the aorta, yielding physiology similar to Transposition of the Great Arteries
- but if there is pulmonic stenosis in addition, physiology resembles Tetralogy of Fallot
- in other forms of DORV, blood from both ventricles is substantially mixed in the RV, yielding physiology that resembles a large VSD
- but again, if there is pulmonic stenosis, physiology resembles Tetralogy of Fallot
Treatment
DORV is treated with surgery.
Epidemiology
DORV affects between 1% and 3% of people born with congenital heart defects.[1]
Chromosomal abnormalities were reported in about 40% of reported cases in the medical literature.[1]
References
- ^ a b Obler D, Juraszek AL, Smoot LB, Natowicz MR (August 2008). "Double outlet right ventricle: aetiologies and associations". J. Med. Genet. 45 (8): 481–97. doi:10.1136/jmg.2008.057984. PMID 18456715.
External links
- Pediatric Heart Surgery
- The Congenital Heart Surgery Video Project
- Ashley: Repair of DORV
- synd/2285 at Who Named It?
- Illustrations at rch.org.au
|
Congenital heart defects (Q20–Q24, 745–746)
|
|
Cardiac shunt/
heart septal defect |
| Aortopulmonary septal defect |
- R→L: Double outlet right ventricle
- Transposition of the great vessels
- Persistent truncus arteriosus
|
|
| Atrial septal defect |
- L→R: Sinus venosus atrial septal defect
- Lutembacher's syndrome
|
|
| Ventricular septal defect |
- L→R and R→L: Eisenmenger's syndrome
- R→L, with other conditions: Tetralogy of Fallot
|
|
| Atrioventricular septal defect |
|
|
|
Valvular heart disease/
heart chambers |
| Right |
- pulmonary valves
- tricuspid valves
- stenosis
- atresia
- Ebstein's anomaly
- Hypoplastic right heart syndrome
|
|
| Left |
- aortic valves
- stenosis
- insufficiency
- bicuspid
- mitral valves
- Hypoplastic left heart syndrome
|
|
|
| Other |
- Dextrocardia
- Levocardia
- Cor triatriatum
- Crisscross heart
- Brugada syndrome
- Coronary artery anomaly
- Anomalous aortic origin of a coronary artery
- Ventricular inversion
|
|
|
|
|
noco/cong/tumr, sysi/epon, injr
|
proc, drug (C1A/1B/1C/1D), blte
|
|
|
|
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
English Journal
- GATA5 interacts with GATA4 and GATA6 in outflow tract development.
- Laforest B, Nemer M.SourceLaboratoire de Développement et Différentiation Cardiaque, Programme de Biologie Moléculaire, Université de Montréal, Montréal QC, Canada H3C 3J7.
- Developmental biology.Dev Biol.2011 Oct 15;358(2):368-78. Epub 2011 Aug 4.
- Members of the GATA family of transcription factors are critical regulators of heart development and mutations in 2 of them, GATA4 and GATA6 are associated with outflow tract and septal defects in human. The heart expresses 3 GATA factors, GATA4, 5 and 6 in a partially overlapping pattern. Here, we
- PMID 21839733
- Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle.
- Tan ZP, Huang C, Xu ZB, Yang JF, Yang YF.SourceClinical Center for Gene Diagnosis and Therapy of State Key Laboratory of Medical Genetics Department of Cardiothoracic Surgery, the Second Xiangya Hospital, Central South University, Changsha, Hunan Province 410011, China.
- Clinical genetics.Clin Genet.2011 Sep 15. doi: 10.1111/j.1399-0004.2011.01787.x. [Epub ahead of print]
- Tan Z-P, Huang C, Xu Z-B, Yang J-F, Yang Y-F. Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle. Congenital heart defects (CHDs) occur in about 0.5-1% of all newborns and are the most common birth defects. Double outlet right ventricle (DORV) accounts for approximately 1-3% of
- PMID 21919901
Japanese Journal
- 両大血管右室起始症術後左室流出路再狭窄に対するextended septoplasty
- 9.先天性嚢胞状腺腫様奇形(CCAM)及び両大血管右室起始症(DORV)に同側肺葉内肺分画症を合併した1例(一般演題,第41回日本小児外科学会関東甲信越地方会)
- 大橋 祐介,五味 明,杉山 彰英,真田 裕,八塚 正四,土岐 彰
- 日本小児外科学会雑誌 43(6), 795-796, 2007-10-20
- NAID 110006424161
Related Links
- 両大血管右室起始症は大動脈弁、肺動脈弁のほとんどが右室から起始する疾患です。 左右心室間の短絡のため肺血流が増加し心不全と肺高血圧症を呈する場合と、肺動脈狭窄 を合併しチアノーゼを呈する場合の2つに大別されます。 ...
Related Pictures
★リンクテーブル★
[★]
- 英
- double outlet right ventricle, double-outlet right ventricle, DORV
- 同
- 両大血管右室起始
- 関
- タウシク・ビング症候群
[show details]
[★]
両大血管右室起始症, DORV
[★]
両大血管右室起始症 DORV
[★]