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MeSH Review:

Double Outlet Right Ventricle

Hamblet, N.S. et al., Gruber, P.J. et al., Artrip, J.H. et al., Russo, P. et al., Rocchini, A.P. et al., et al.

Pu, Ishiwata, Juraszek, Ma, Izumo,

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Disease relevance of Double Outlet Right Ventricle

The cardiac defects include atrial and ventricular septal defects, overriding aorta, double-outlet right ventricle, persistent truncus arteriosus and right-sided aortic arches [1].
Four of the patients had a Rastelli procedure for D-transposition of the great arteries with a ventricular septal defect and pulmonary stenosis; one patient had repair of double outlet right ventricle with a ventricular septal defect and pulmonary stenosis [2].
Cardiac malformations (pulmonary trunk stenosis, ventricular septal defect, and double outlet right ventricle) were induced by the administration of two doses of retinoic acid (RA) to Wistar rats on d 13 of pregnancy [3].
Six patients, 4 of whom had complete atrioventricular (AV) canal and tetralogy of Fallot (TOF) and 2 of whom had double-outlet right ventricle with subaortic ventricular septal defect (VSD) and right ventricular outflow tract obstruction, were treated [4].
A case of total thoracic ectopia cordis with double-outlet right ventricle and ventricular septal defect is presented [5].

High impact information on Double Outlet Right Ventricle

5. RXR alpha -/- embryos display complex defects, including ventricular septal, atrioventricular cushion, and conotruncal ridge defects, with double outlet right ventricle, aorticopulmonary window, and persistent truncus arteriosus [6].
In particular, rescued FOG-1-/- mice die at embryonic day (E) 14.5 with cardiac defects that include double outlet right ventricle and a common atrioventricular valve [7].
CFC1 mutations in patients with transposition of the great arteries and double-outlet right ventricle [8].
In experiment 1, among 45 embryos treated with retinoic acid 70 mg/kg at day 8.5 of gestation, 35 (78%) had TGA and 3 (6.7%) had a double-outlet right ventricle with a subpulmonary ventricular septal defect [9].
The surgical management of patients with biventricular hearts and complex double-outlet right ventricle with noncommitted ventricular septal defect, hypoplastic ventricles, common atrioventricular orifice, straddling tricuspid valve, and straddling mitral valves is controversial [10].

Chemical compound and disease context of Double Outlet Right Ventricle

BACKGROUND: Optimal management of double-outlet right ventricle with subpulmonary ventricular septal defect remains controversial [11].
A 24-year-old man known to have a double outlet right ventricle, status post-modified Fontan procedure at age 10, taking coumadin after having a stroke one year prior, presented with a massive pulmonary embolism and hemodynamic instability [12].

Biological context of Double Outlet Right Ventricle

BACKGROUND: The STS-EACTS International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the presence of right ventricular outflow tract obstruction (RVOTO) [13].

Anatomical context of Double Outlet Right Ventricle

Two defects, ventricular septal defect and double outlet right ventricle, were significantly correlated with the location of neural crest removed [14].
This report describes an infant with double-outlet right ventricle who underwent pulmonary artery banding as palliation for excessive left to right shunting through a ventricular septal defect [15].
These embryos had common atrioventricular canal (CAVC), double outlet right ventricle (DORV), hypoplastic ventricular myocardium, and normal coronary vasculature [16].
Additional double outlet right ventricle, hypoplastic left ventricle, and ventricular septal defect, as well as multiple extracardiac anomalies, were found by prenatal echocardiographic investigation and confirmed by necropsy examination [17].
METHODS: Since 1989, 12 of 13 patients with aortic arch obstruction and transposition of the great arteries or double-outlet right ventricle with subpulmonary ventricular septal defect have undergone complete single-stage repair [18].

Gene context of Double Outlet Right Ventricle

Mice containing null mutations in Dvl2 present with 50% lethality in both inbred 129S6 and in a hybrid 129S6-NIH Black Swiss background because of severe cardiovascular outflow tract defects, including double outlet right ventricle, transposition of the great arteries and persistent truncus arteriosis [19].
In Cx40-/- mice resulting from crossing of Cx40+/- mice, the most common cardiac malformations were double-outlet right ventricle (DORV), TOF, and endocardial cushion defects [20].
The jmj homozygous mouse embryos showed heart malformations, including ventricular septal defect, noncompaction of the ventricular wall, double-outlet right ventricle, and dilated atria [21].
The conduction system in double outlet right ventricle with subpulmonic ventricular septal defect and related hearts (the Taussig-Bing group) [22].
Primary cardiac diagnoses included TGA with intact ventricular septum (TGA/IVS, n = 79, 63%), TGA with ventricular septal defect (TGA/VSD, n = 37, 30%), and Taussig Bing Anomaly (TBA, n = 9, 7%) [23].

References

Cardiac malformations, adrenal agenesis, neural crest defects and exencephaly in mice lacking Cited2, a new Tfap2 co-activator. Bamforth, S.D., Bragança, J., Eloranta, J.J., Murdoch, J.N., Marques, F.I., Kranc, K.R., Farza, H., Henderson, D.J., Hurst, H.C., Bhattacharya, S. Nat. Genet. (2001) [Pubmed]
Subaortic obstruction after the use of an intracardiac baffle to tunnel the left ventricle to the aorta. Rocchini, A.P., Rosenthal, A., Castaneda, A.R., Keane, J.F., Jeresaty, R. Circulation (1976) [Pubmed]
Inotropic effect of increasing concentration of Ca2+ in the fetal rat heart with retinoic acid-induced malformations. Ostádalová, I., Pexieder, T., Ostádal, B., Kolár, F. Pediatr. Res. (1995) [Pubmed]
Complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: a report of successful surgical treatment. He, G.W., Mee, R.B. Ann. Thorac. Surg. (1986) [Pubmed]
Complete thoracic ectopia cordis with double-outlet right ventricle: neonatal repair. Watterson, K.G., Wilkinson, J.L., Kliman, L., Mee, R.B. Ann. Thorac. Surg. (1992) [Pubmed]
RXR alpha deficiency confers genetic susceptibility for aortic sac, conotruncal, atrioventricular cushion, and ventricular muscle defects in mice. Gruber, P.J., Kubalak, S.W., Pexieder, T., Sucov, H.M., Evans, R.M., Chien, K.R. J. Clin. Invest. (1996) [Pubmed]
Endothelial lineage-mediated loss of the GATA cofactor Friend of GATA 1 impairs cardiac development. Katz, S.G., Williams, A., Yang, J., Fujiwara, Y., Tsang, A.P., Epstein, J.A., Orkin, S.H. Proc. Natl. Acad. Sci. U.S.A. (2003) [Pubmed]
CFC1 mutations in patients with transposition of the great arteries and double-outlet right ventricle. Goldmuntz, E., Bamford, R., Karkera, J.D., dela Cruz, J., Roessler, E., Muenke, M. Am. J. Hum. Genet. (2002) [Pubmed]
Morphological observations on the pathogenetic process of transposition of the great arteries induced by retinoic acid in mice. Yasui, H., Nakazawa, M., Morishima, M., Miyagawa-Tomita, S., Momma, K. Circulation (1995) [Pubmed]
Modified Fontan procedure for biventricular hearts with complex forms of double-outlet right ventricle. Russo, P., Danielson, G.K., Puga, F.J., McGoon, D.C., Humes, R. Circulation (1988) [Pubmed]
Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Takeuchi, K., McGowan, F.X., Moran, A.M., Zurakowski, D., Mayer, J.E., Jonas, R.A., del Nido, P.J. Ann. Thorac. Surg. (2001) [Pubmed]
A pulmonary embolism treated with the Angiojet technique in a patient with double outlet right ventricle. Brenes, J.C., Ferreira, A., Galindo, A. The Journal of invasive cardiology. (2004) [Pubmed]
Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Artrip, J.H., Sauer, H., Campbell, D.N., Mitchell, M.B., Haun, C., Almodovar, M.C., Hraska, V., Lacour-Gayet, F. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. (2006) [Pubmed]
Effects of the size of lesions of the cardiac neural crest at various embryonic ages on incidence and type of cardiac defects. Besson, W.T., Kirby, M.L., Van Mierop, L.H., Teabeaut, J.R. Circulation (1986) [Pubmed]
Pseudoaneurysm of the pulmonary artery after the banding procedure: two-dimensional echocardiographic description. Foale, R.A., King, M.E., Gordon, D., Marshall, J.E., Weyman, A.E. J. Am. Coll. Cardiol. (1984) [Pubmed]
GATA4 is a dosage-sensitive regulator of cardiac morphogenesis. Pu, W.T., Ishiwata, T., Juraszek, A.L., Ma, Q., Izumo, S. Dev. Biol. (2004) [Pubmed]
Congenital absence of aortic and pulmonary valve in a fetus with severe heart failure. Marek, J., Skovránek, J., Povýsilová, V. Heart (1996) [Pubmed]
Single-stage arterial switch with aortic arch enlargement for transposition complexes with aortic arch obstruction. Tchervenkov, C.I., Tahta, S.A., Cecere, R., Béland, M.J. Ann. Thorac. Surg. (1997) [Pubmed]
Dishevelled 2 is essential for cardiac outflow tract development, somite segmentation and neural tube closure. Hamblet, N.S., Lijam, N., Ruiz-Lozano, P., Wang, J., Yang, Y., Luo, Z., Mei, L., Chien, K.R., Sussman, D.J., Wynshaw-Boris, A. Development (2002) [Pubmed]
High incidence of cardiac malformations in connexin40-deficient mice. Gu, H., Smith, F.C., Taffet, S.M., Delmar, M. Circ. Res. (2003) [Pubmed]
Jumonji, a nuclear protein that is necessary for normal heart development. Lee, Y., Song, A.J., Baker, R., Micales, B., Conway, S.J., Lyons, G.E. Circ. Res. (2000) [Pubmed]
The conduction system in double outlet right ventricle with subpulmonic ventricular septal defect and related hearts (the Taussig-Bing group). Bharati, S., Lev, M. Circulation (1976) [Pubmed]
Current expectations for newborns undergoing the arterial switch operation. Dibardino, D.J., Allison, A.E., Vaughn, W.K., McKenzie, E.D., Fraser, C.D. Ann. Surg. (2004) [Pubmed]

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