**Part 6**

**Congenital Anomaly Application** 

204 Aortic Valve Surgery

Mornals K, Santos J, Gonzalez-Prendes CM, Rodriguez F, García B, Sainz H. Disfunción

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Sanz-Ayán M.P, Rodríguez-Palero S, Garzón-Márquez F.M, Sánchez-Callejas S, Pérez-

Sotaniemi K.A., Monomen H., Hokkanen T.E. Longterm cerebral outcome after open heart surgery. A five year neurophysiological follows up study. *Stroke* 17: 410-416, 1986 Tjang YS, van Hees Y, Körfer R, Grobbee DE, van der Heijden GJ. Predictors of mortality after aortic valve replacement. Eur J Cardiothorac Surg. 2007; 32(3):469-74. Zabala J.A. Complicaciones neurológicas de la cirugía cardiaca. *Rev Esp Cardiol*. 2005;

cardiovascular surgery. *Arq Neuropsiquiatr*. 1993; 51(2):179-82

with a history of stroke. *Ann Thorac Surg* 1996; 61:42-47

coronary bypass surgery. *N Engl J Med*. 1996; 335:1857-63

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Novales D. Síndrome del "hombre en Barril". A propósito de un caso.

**12** 

*Furlanetto Institute* 

*Brazil* 

**Correction of Transposition of** 

Gláucio Furlanetto and Beatriz H. S. Furlanetto

**Great Arteries with Ventricular Septal** 

**with Double Arterial Translocation with** 

**Preservation of the Pulmonary Valve** 

**Defect and Left Outflow Tract Obstruction** 

Transposition of the great arteries (TGA) was first recognized by Mathew Bailie in 1797. The term transposition of the aorta and pulmonary artery (PA) was applied by Farre in 1814. Van Praagh proposed that TGA was one variety of malposition of the great arteries secondary to aberrations in conotruncal development. The other tipes of malposition include double-outlet right ventricle, double-outlet left ventricle, and anatomically corrected malposition. According the Congenital Heart Surgery Nomenclature and Database Project (James 2000), TGA always has discordant ventriculoarterial alignment such that the aorta arises entirely or in large party from the right ventricle (RV), the PA arises entirely or in large part above the left ventricle (LV) and concordant atrioventricular alignment is nearly always present. The term simple TGA has come to be used to denote those patients without associated ventricular septal defect (VSD) or left ventricular outflow tract obstruction (LVOTO) and nearly 75% of TGA patients have the simple type. Usually in the simple form of TGA the left ventricle doesn't has subpulmonary conus, so there is fibrous continuity between pulmonary and mitral valves, and tha aorta is anterior and to the right of the PA. The VSD occur in 40-45% of TGA patients and about 30% of these defects will be very small. LVOTO occurs in 25% of patients and is rare in patients with intact ventricular septum, ocurring in 5%. Approximated 30% of patients with TGA-VSD have LVOTO. A subvalvar LVOTO can be dynamic, localized fibrous ring, diffuse tunnel-like obstruction, muscular obstruction related to malposition of the outlet septum and LVOTO result from malposition

TGA is the second more common cyanotic congenital heart disease (CHD) and represents approximately 5-7% of all CHD and has a incidence of 20-30 in 100.000 live births, with a male preponderance of approximately 2:1. In patients with TGA, VSD and LVOTO early survival reachs 70% at one year and 29% at 5 years. Neonates with TGA, VSD and severe LVOTO have diminished pulmonary blood flow and they represents 5-8% of neonatal TGA population. Clinical findings are similar to those in the infants with tetralogy of Fallot with

of the mitral apparatus on the interventricular septum.

severe pulmonar stenosis.

**1. Introduction** 
