Dextro-Transposition of the Great Arteries

d-TGA

is the most common cyanotic congenital heart lesion in neonates systemic and pulmonary circulations in parallel more frequently in males of normal birth weight

d-TGA

ventriculoarterial discordance aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle

Lesiones asociadas

coronary artery anomalies coarctation of the aorta hypoplastic or interrupted arch pulmonic stenosis Interventricular septal defect right ventricular outflow tract obstruction mitral or tricuspid valve abnormalities hypoplasia of the right ventricle left ventricular outflow tract obstruction

Fisiologia Fetal

The low resistance of the umbilicalplacental system receives the right ventricular output left ventricle faces the high pulmonary vascular resistance and placental vascular resistance

Fisiologia Postnatal

oxygenated blood will be ejected into the pulmonary circulation without reaching the systemic circulation. de-oxygenated blood will not reach the pulmonary circulation for oxygenation. hypoxemia incompatible with life unless intracardiac (PFO/ASD or VSD) or extracardiac (patent ductus arteriosusbronchial circulation) communications are patent

Presentacion Clinica

cyanosis, systemic hypoperfusion, cardiogenic shock, metabolic acidosis, oliguria and multiorgan dysfunction symptoms appear after PDA closure or even sooner if the patent foramen ovale is very restrictive prominent cardiac impulse Murmurs are not usually heard pulses are normal until cardiogenic shock appears Sat arterial O2 5070% and arterial PO2

Manejo Inmediato

ABC along with a brief echocardiogram for diagnosis, assessment of ventricular function, and evaluation of patency of DA and FO PGE1 0.01 0.1 mcg/kg/min inotropic support, fluid and bicarbonate If insufficient mixing: emergent ballon atrial septostomy head ultrasound, renal ultrasound, and genetic tests may be done prior to surgical intervention

Tratamiento Quirurgico

arterial switch operation via a median sternotomy with cardiopulmonary bypass and moderate to severe hypothermia transection of both great vessels, translocation of the pulmonary arteries anterior to the aorta and suturing the ascending aorta to the arterial root attached to the left ventricle and the distal main pulmonary artery to the arterial root attached to the right ventricle coronary arteries need to be harvested

ASO

Rastelli

creation of interventricular tunnel funneling the left ventricular blood into the anterior aorta; a conduit is used to established right ventricle to pulmonary artery continuity

R.E.V. (Rparation a lEtage Ventriculaire)

enlargement of the ventricular septal defect to create a more direct communication between the left ventricle and the aorta and a direct anastomosis between the right ventricle and the pulmonary

Aortic Translocation procedure (Nikaidoh procedure)

the aortic root is moved into the pulmonary position, closer to the left ventricle, avoiding the creation of an interventricular tunnel, the right ventricular outflow tract can be repaired with or without a conduit

Complicaciones Postoperatorio

low cardiac output syndrome mitral regurgitation coronary insufficiency and/or ischemia supravalvar aortic stenosis pulmonary stenosis Bleeding arrhythmias

Manejo Cardiovascular

chest open/close TEE immediately available inotropic support: low dose dopamine (3 5g/kg/min), low dose epinephrine (0.05 1 g/kg/min), milrinone (0.75 g/kg/min) and sodium nitroprusside (13 g/kg/min) Avoid high inotropic support: ECMO until the ventricular function recovers!!!! rule out residual lesions, suboptimal myocardial preservation, and/or excessive tissue stretching/damage during the

Manejo Cardiovascular

check: echocardiogram, CVP, left atrial pressure, lactate levels, mixed venous saturations, and NIRS, physical exam ECG Labs Chest Rx

Manejo Respiratorio

extubation when ahemodynamically stable without residual lesions Extubation failure alerts of diaphragmatic paralysis Prior to chest closure endotracheal tube suctioned and a pacemaker must be at the bedside with wires connected chest closed: tidal volumes of 1012 cc/kg, avoid over-distention and collapse, PEEP 56 cm/H2O, and FIO2 to maintain saturations of 98100%

Fluid, Electrolytes and Nutritional Management

Parenteral nutrition is begun 24 hours after repair with Dx 2025%, protein intake 3 g/day, and fat intake 33.5 g/kg/day Enteral nutrition is commenced once the infant reaches hemodynamic stability.

Hematologic Management

Bleeding is after ASO, more commonly when the procedure involves a long CPB time and cross clamp time transfusion of platelets, fresh frozen plasma, or cryoprecipitate If bleeding above 710 ml/kg/h: chest reexploration at the bed side blood clots may compress some coronary branches causing ischemia and cardiovascular collapse

Gastrointestinal Management

Liver and pancreatic function should be monitored PO intake after 24 h of extubation, attention to changes in the quality and quantity of the chest and peritoneal drainage after enteral feeds are started (chylothorax and chyloperitoneum)

Renal Management

Furosemide and thiazides are the most commonly used diuretics Furosemide is started within 612 h after repair (dose 0.10.4 mg/kg/h) to achieve negative fluid balance within 2448 h after repair; this will facilitate chest closure and the extubation

Neurologic Management

head ultrasounds may be performed post repair EEG 1224 h post repair to detect seizure activity

Infectious Disease Management

vancomycin and third generation cephalosporins until the chest is closed Following closure first generation cephalosporin is used until the chest tubes are removed Central lines must be discontinued as soon as possible

Long Term Outcome

satisfactory in most patients results are influenced by the surgical learning curve of each institution re-operation free survival rate 82.2% at 10 years and 75.7% at 15 years neurologic abnormalities Late complications:supravalvar aortic stenosis, supravalvar pulmonary stenosis, neoaortic root dilation and neoaortic regurgitation, sinus node dysfunction,