History

The transposition of the great arteries was first

described by Mathew Baillie in 1797, in the second edition of the book "The Morbid Anatomy of Some of the Most Important Parts of the Human Body". However, the term transposition was only applied in 1814, by Farre, meaning that aorta and pulmonary trunk were placed (positio) across (trans) the ventricular septum.

Epidemiology
The hearts with complete TGA represent 57% of all

congenital heart diseases, Corresponding to an incidence of 20 to 30 per 100 000 live births. There is a male predominance with a male/female sex ratio that varies, in the literature, from 1.5:1 to 3.2:1 In 10% of the cases, this cardiac lesion is associated with other non cardiac malformations

Nomenclature
Basic anatomic abnormality in transposition:
the aorta arising from a morphologically right

ventricle, and the pulmonary artery arising from a morphologically left ventricle

 The common clinical type, that is,

With situs solitus of the atria, Concordant AV (right atrium to right ventricle and left

atrium to left ventricle) and

Discordant ventriculoarterial alignments, Is widely termed complete TGA

Complete TGA
TGA with (S,D,D)
situs solitus (S) of the atria and viscera, usual (D) looping of the ventricles,

and an anterior and rightward (D) aorta

Congenitally corrected
Corrected transposition of the great arteries
I.E., Physiologically corrected In which systemic venous blood flows to the

pulmonary artery and

Pulmonary venous blood to the aorta

Mortality/Morbidity
The mortality rate in untreated patients is

approximately 1. 30% in the first week, 2. 50% in the first month, 3. And 90% by the end of the first year. With improved diagnostic, medical, and surgical techniques, The overall short-term and midterm survival rate exceeds 90%.

Morphogenesis and Etiology

The detailed developmental aspects remain largely

unknown Distal infundibulum (conus) considered by some a major factor The normal conus is subpulmonary, left sided, and anterior, and it prevents fibrous continuity between the pulmonary and tricuspid valve rings In TGA, the infundibulum is usually subaortic, right sided, and anterior, and it prevents fibrous continuity between the aortic and tricuspid valve rings

 The morphogenesis of TGA can be hypothesized to

result from the abnormal growth and development of the subaortic infundibulum

Cardiac Segments
The atria are formed normally, with normal internal

anatomy Almost always, there is a patent foramen ovale and only rarely (5%) a true secundum atrial septal defect (ASD) The sinus and AV nodes are in their usual locations.

 The right ventricle is normally positioned and

becomes progressively hypertrophied The inflow and sinus portions are essentially normal in architecture, but the central fibrous body is abbreviated With intact ventricular septum, the entire septum is usually a relatively straight structure

 There is a subaortic conus separating the aortic valve

from the tricuspid valve whereas the pulmonary valve is in fibrous continuity with the mitral valve In most patients the aortic root is directly anterior or anterior and to the right of the pulmonary trunk

 The two aortic sinuses of Valsalva adjacent to the

aorticopulmonary septum that face the pulmonary artery contain the ostia of the coronary arteries in >99% of cases In addition, multiple ostia may be present in a given sinus, the epicardial course may be unusual, or there may be complete absence of one of the branches

 The course of the sinus node artery, which typically

arises as the first branch from the right coronary artery close to its aortic origin, may be of surgical importance

Coexisting Anomalies
Half of the hearts with TGA have no other anomaly

except a persistent patent foramen ovale or a PDA VSD is common and present in about 40% to 45% a combination of VSD and significant LVOTO occurs in about 10% Structural anomalies of the mitral valve have been observed at autopsy in about 20% Tricuspid valve anomalies were present in about 4% of surgical patients

 Coarctation of the aorta, arch hypoplasia, or rarely,

interrupted aortic arch, coexist in about 5%

Ventricular Septal Defect

VSD is the most frequent coexisting anomaly. The approximate distribution of vsd location includes 1. Perimembranous (conoventricular, 33%), 2.AV canal (inlet septum, 5%), 3. Muscular (27%), 4. Malalignment (30%), and 5. Conal septal hypoplasia type (5%)

VSD
The typical membranous defect lies adjacent to the

membranous septum and tricuspid annulus at the

anteroseptal tricuspid valve commissure

Most muscular defects are in the midseptum

Physiology
The dominant physiologic abnormalities in TGA are a

deficiency of oxygen supply to the tissues and An excessive right and left ventricular workload The systemic and pulmonary circulations function in parallel In tga with intact ventricular septum, only a relatively small proportion of blood is exchanged

Intercirculatory Mixing

 The extent of intercirculatory mixing in TGA depends

on the number, size, and position of the anatomic communications and on the total blood flow through the pulmonary circuit

Sites of shunting
ASD

the interatrial shunt is from right atrium to left atrium during ventricular diastole AND reverse in systole PDA Bidirectional shunting takes place VSD Depends on size and location of VSD

Bronchopulmonary Collateral Circulation

A significant role for the bronchopulmonary collateral

circulation in TGA Visualized by angiography in >30% of infants with TGA younger than 2 years May play a role in the accelerated and more widespread pulmonary vascular disease Persistence of a significant bronchopulmonary collateral circulation after surgical repair

Lung circulation
Maldistribution pattern of pulmonary blood flow in

TGA patients Blood flow distributed to the right lung than normal Abnormal rightward inclination of the main pulmonary artery Some degree of hypoplasia of the left pulmonary arterial vessels

Pulmonary blood flow

Arterial Blood Gases and Metabolic Responses

The pulmonary venous blood reflects chemoreceptor-

stimulated hyperventilation. In room air, pO2 levels may be increased to as high as 110 mm Hg and the pCO2 levels reduced to 15 to 25 mm Hg. In contrast, systemic arterial pO2 levels are rarely higher than 35 mm Hg, and the pCO2 is usually normal or slightly elevated (<45 mm Hg)

Fetal Circulation
Both TGA physiology and anatomy appear compatible

with normal fetal survival Right side of the heart ejects blood directly into the ascending aorta Provides blood of slightly lower glucose concentration and pco2 to the coronary and cerebral circulations

Transitional Circulation
The pulmonary vascular resistance falls with

expansion of the lungs The systemic vascular resistance increases because of removal of the low-resistance placental circulation PFO is patent with bidirectional shunting The ductus arteriosus is often widely patent after birth The ductus soon constricts with resulting increased hypoxemia The presence of a high proportion of fetal hemoglobin

Pulmonary Vascular Disease

TGA has an apparent accelerated rate of development

and an increased frequency of this complication Marked increases in pulmonary vascular muscularity and intimal hyperplasia

Clinical manifestations
The parallel circulation just described results in a

significant hypoxemic status that is observed clinically by central cyanosis. The bluish discoloration of the skin and mucous membranes if no obstructive lesions are present, and there is a large ventricular septal defect that allows for satisfactory mixing between the two circulations, cyanosis may go undetected

Clinical features
Tachypnoea,
tachycardia, diaphoresis,

poor weight gain,

a gallop rhythm, and eventually hepatomegaly

PHYSIOLOGIC-CLINICAL CLASSIFICATION
TGA (IVS or small VSD) with increased PBF and small

ICS TGA (VSD large) with increased PBF and large ICS TGA (VSD and LVOTO), with restricted PBF TGA (VSD and PVOD), with restricted PBF

Physical apperance
Increased birth weight
Chest deformities are common: left precordial buldge

and increased AP diameter Early intence cyanosis Reversed differential cyanosis

Arterial pulse
Bounding pulses
Warm extremities Large PDA there is no bounding pulse

Precordial movements
Prominent RV impulse
Palpable second heart sound Right arch causing right sternoclavicular impulse

Systolic thrills

Auscultation
First heart sound is normal
Pulmonary ejection sound indicates dilated PA Systolic murmur are absent at birth, except in cases of

subpulmonic stenosis Pulmonary stenosis murmur best heard mid left sternal edge VSD murmur is absent at birth, increases later due to fall in pulmonary resistance

Auscultation
PDA murmur is usually systolic
Diastolic murmur: mid diastolic due to mitral and

tricuspid Early diastolic murmur: PR , Graham steell Third heart sound

Electrocardiographic Features
Sinus rhythm
usual ECG findings are right-axis deviation with right or combined ventricular hypertrophy

Tall peaked right atrial P waves

Left precordial leads : small r , q waves absent

Xray
Increased pulmonary blood flow
Right lung more pefused Thymic shadow is usually absent

Vascular pedicle is narrow

Right sided arch is uncommonly seen Egg on side apperarance

Echocardiographic Features
echocardiography has become the diagnostic method

of choice in the patient with TGA Subcostal imaging provides a flexible acoustic window that allows wide angulation to optimize simultaneous visualization of the great arteries and their respective ventricular connections

 Additional imaging from the apex (four-chamber

view) is useful in establishing the identity of the posterior vessel as the pulmonary artery Suprasternal and high parasternal views enable the aorta to be traced arising from the right ventricle to the arch and its branches

 Spatial orientation of the great arteries and the origin

and proximal segments of the coronary arteries can be viewed on parasternal and short-axis scans at the base of the heart

 Echocardiography can diagnose most of the important

associated anomalies with a high degree of accuracy, including size, number, and location of VSDs, anatomic type of LVOTOs, form and function of tricuspid and mitral valve anomalies, and outlet septum malalignment defects

 Imaging can be used to guide catheter placement and

movements during balloon atrial septostomy to assess the anatomic adequacy of the septostomy Routine obstetric fetal echocardiography may lead only to the prenatal diagnosis of TGA institution of prostaglandin E1 (PGE1) therapy, and prompt transfer to a specialized cardiac facility

Cardiac Catheterization
Cardiac catheterization with balloon atrial septostomy
particularly for the neonate with poor intercirculatory

mixing angiography, balloon atrial septostomy, and meaningful physiologic information can be obtained safely only if optimum techniques are expertly and judiciously employed using percutaneous femoral vein entry, umbilical vein catheterization, or (rarely) direct femoral vein cutdown

Angiocardiography
selective cardiac chamber and great vessel

angiographic injections should be performed

diagnoses of more complex forms of TGA

pulmonary artery pressure; pulmonary blood flow (and vascular resistance); coronary artery anatomy; morphologic details of pulmonary or subpulmonary obstruction; VSD number, site, and size; great vessel alignments in relation to the VSD and outlet septum; and type and severity of aortic arch abnormalities

Balloon Atrial Septostomy

initial management of the severely hypoxic neonate

with TGA Survival for the first month of life improved from 20% before septostomy was introduced to as high as 95% afterward

 The catheter should be advanced across the foramen

ovale into the left atrium or a pulmonary vein and the position of the tip established balloon is inflated with diluted angiographic contrast medium to 12 to 15 mm diameter and then rapidly withdrawn across the atrial septum

 the septum primum flap of the fossa ovalis is ruptured

The catheter should be advanced immediately and the

balloon pushed cephalad out of the inferior vena caval orifice into the right atrium This same procedure should be repeated several times with increasing balloon volumes

Medical Care
Initial treatment consists of maintaining ductal

patency with continuous IV prostaglandin E1 infusion. This is particularly important in patients with severe left ventricular outflow tract stenosis or atresia.