Double Outlet Right Ventricle
Double outlet right ventricle is a rare congenital cardiac malformation that appears to result from lack of conotruncal inversion, failure of leftward conoventricular shift and persistance of a subaortic conus [1, 2]. The anomaly is classified into two categories: those hearts with and those without pulmonary stenosis [3, 4]. The obstruction to pulmonary blood flow may be either valvular, subvalular, supravalvular, or a combination of these . The great arteries in double outlet right ventricle are either side by side (aorta on the right), d-malposed (aorta anterior and to the right of the pulmonary artery), 1-malposed (aorta anterior and to the left of the pulmonary artery), or normally related . The persistence of a subaortic conus prevents the normal leftward shift of the aorta and aortic valve. Thus, the aorta remains to the right of the pulmonary artery and muscle tissue (bilateral conus) separates the mitral and tricuspid valves from the aortic valve and pulmonic valve (Figures 1 & 2). The anomaly is further classified by the position of the ventricular septal defect. The ventricular septal defect may be related to (1) the aortic valve (subaortic), (2) the pulmonic valve (subpulmonic) (TaussigBing anomaly), (3) to both semilunar valves (doubly committed) or (4) to neither semilunar valve (remote) . In those hearts with a subaortic ventricular septal defect, pulmonic stenosis is a common feature. In addition, hearts with a subpulmonic ventricular septal defect may have an associated coarctation of the aorta or mitral valve anomalies. These mitral valve anomalies include mitral stenosis, atresia, or straddling mitral valve .
KeywordsRight Ventricle Ventricular Septal Defect Pulmonary Blood Flow Great Artery Ventricular Septum
Unable to display preview. Download preview PDF.
- 5.Krongrad E, Ritter DG, Weidman WH & Dushane JW: 14. Hemodynamic and anatomic correlation of electrocardiogram in double outlet right ventricle. Circulation 56: 995, 1972.Google Scholar
- 7.Neufeld NH & Randall PA: Double outlet right ventricle. 16. In: Moss AJ, Adams FH & Emmanoulides G (eds) Heart Disease in Infants, Children and Adolescents, Baltimore, Williams & Wilkins, 1977, pp 355–366.Google Scholar
- 12.Henry WL, Maron BJ & Griffith JM: Cross-sectional echocardiography in the diagnosis of congenital heart disease: Identification of the ventricles and great arteries. Circula-tion 56: 267, 1977.Google Scholar
- 13.Henry WL, Maron BJ, Griffith JM, Redwood DR & Epstein SE: Differential diagnosis of anomalies of the great arteries by real-time two-dimensional echocardiography. Circulation 51: 283, 1975.Google Scholar
- 17.Hagler DJ, Tajik Ai, Seward JB, Edwards WD, Mair DD & Ritter DG: Atrioventricular and ventriculoarterial discordance (Corrected transposition of the great arteries): Recognition by wide-angle two-dimensional echocardiographic assessment of ventricular morphology. Mayo Clin Proc 56: 591, 1981.PubMedGoogle Scholar
- 19.Gomes MMR, Weidman WH, McGoon DC & Danielson GK: Double outlet right ventricle without pulmonic steno-sis surgical considerations and results of operation. Circulation 43/44 (Suppl I): I - 31, 1971.Google Scholar