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Discordant Atrioventricular (AV) Connection with Discordant VA Connection

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In this condition, the morphological right atrium is usually situated in the normal position but is connected to the morphological left ventricle, which in turn gives rise to the pulmonary artery. The morphological left atrium is connected to the morphological right ventricle, which gives rise to the aorta. Thus, the systemic venous return passes to the pulmonary artery and the pulmonary venous return to the aorta, although the ventricular anatomy is 'inverted'. Therefore, the circulation is anatomically 'corrected'. If these are isolated findings, this condition is compatible with a long and uncomplicated life. However, usually there is associated pulmonary stenosis, a VSD, complete heart block or Ebstein's malformation. The timing and symptoms at presentation will vary according to the severity of the associated lesion. Severe Ebstein's malformation can be very difficult to treat successfully in this condition, as the abnormal atrioventricular valve lies in the systemic circulation.On the fetal echocardiogram, the venous drainage will usually be normally positioned, but the heart will lie rather centrally in the fetal thorax. On the four chamber view, the posterior AV valve will be inserted more apically in the septum than the anterior AV valve, the reverse from normal. The moderator band will be seen in the posterior left sided ventricle. The pulmonary artery will arise from the morphological left ventricle, the aorta from the right. The great arteries will not cross over each other at their origin but arise in parallel orientation. The aorta will lie anterior or side-by-side with the pulmonary artery but positioned to the left of it. Displacement of the AV valve only affects the tricuspid valve but in this setting this means that the systemic (posterior, left-sided) AV valve is affected. If it is severe, it will compromise aortic outflow. Title: In: Paediatric Cardiology Author: Losekoot TG, Becker AE Journal: Anderson RH, McCartney FJ, Shinebourne EA, Tynan M (Eds). Churchill Livingstone: Edinburgh, p867-889 Year: 1987 Title: Prospective diagnosis of 1,006 consecutive cases of congenital heart disease in the fetus Author: Allan LD, Sharland GK, Milburn A, Lockhart SM, Groves AMM, Anderson RH, Cook AC, Fagg NLK Journal: J Am Coll Cardiol 23:1452-8 Year: 1994 Title: In: Color Atlas of Fetal Cardiology Author: Allan LD, Sharland G, Cook A Journal: Mosby-Wolfe: London, p59-69 Year: 1994 Title: In: Heart Disease in Infants, Children and Adolescents Author: Ruttenberg HD Journal: Adams FH, Emmanouilides GC, Riemenschneider TA (Eds) Williams and Wilkins: Baltimore, p424-442 Year: 1989 Title: Corrected transposition of the great arteries with isolated aortic coarctation: In utero echocardiographic diagnosis. Author: Santoro G; Masiello P; Baldi C; Farina R; Fittipaldi O; Di Benedetto G Journal: Pediatr Cardiol Sep-Oct;18(5):396-8 Year: 1997 Title: Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance. Author: Sano T; Riesenfeld T; Karl TR; Wilkinson JL Journal: Circulation Nov 1;92(9 Suppl):II272-8 Year: 1995 Title: Echocardiographic considerations in demonstrating complex anatomy of criss-cross atrioventricular valves and discordant atrioventricular and ventriculoarterial relations. Author: Morelli PJ; Kimball TR; Witt SA; Meyer RA Journal: J Am Soc Echocardiogr Sep-Oct;9(5):727-9 Year: 1996 Title: Corrected transposition of the great arteries with isolated aortic coarctation: In utero echocardiographic diagnosis. Author: Santoro G; Masiello P; Baldi C; Farina R; Fittipaldi O; Di Benedetto G Journal: Pediatr Cardiol Sep-Oct;18(5):396-8 Year: 1997 Title: Combined Rastelli and atrial switch procedure: anatomic and physiologic correction of discordant atrioventricular connection associated with ventricular septal defect and left Author: Delius RE; Stark J Journal: Eur J Cardiothorac Surg ;10(7):551-5 Year: 1996

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