Transposition of Great Arteries(TGA)

Dr.Aso faeq salihpediatric cardiologist

5 % of all CHD

Types

d – TGA . l – TGA .

d – TGA

• the aorta is anterior & to the R of pulmonary artery .

• Survival depends on patency of foramen ovale & Ductus Arteriosus .

• 1/2 of patients VSD .• M : F 3 : 1 .• > common CHD in infants of diabetic

women .• Before the advent of surgery mortality

> 90 % in the first year of life .

Clinical manifestations • Cyanosis & dyspnea within 1st hrs. or

days of life .• Hypoxemia is severe , HF is less

common .Signs : Cyanosis S2 single & loud . Murmurs are usually absent unless there

is associating lesions

Investigations

CXR :• Mild cardiomegaly , narrow mediastinum ,

normal or increased pul. Vascularity .• Egg shaped heart .• ECG: normal neonatal R – sided dominant

pattern .• Arterial Po2 low & not changed

following hyperoxia test with 100% O2 .• Echo & Doppler : confirm the diagnosis .• Cardiac catheterization : palliative tretament

Treatment :

• PGE2 infusion to maintain a patent Ductus Arteriosus

• Cath and Surgery :Rashkind balloon Atrial Septoplasty .Arterial switch( Jantene) : treatment

of choice , timing 1st 2 weeks , if delayed decrease LV mass .

Mustard operation .

Prognosis :• Depends on patency of Ductus Arteriosus ,

associating defects ( VSD provide a better mixing of blood ) .

• Without surgery death within 1st year of life .

l – TGARA is connected with LV & LA is connected with

RV , the great arteries also transposed the circulation is physiologically corrected .

Pulmonary stenosisPulmonary stenosis

Pulmonary stenosis

• Common as PDA,

• There are four different types of pulmonary stenosis:

• valvar pulmonary stenosis - the valve leaflets are thickened and/or narrowed-fusion of valves- leave a small eccentric hole in middle

• supravalvar pulmonary stenosis - the pulmonary artery just above the pulmonary valve is narrowed

• subvalvar (infundibular) pulmonary stenosis - the muscle under the valve area is thickened, narrowing the outflow tract from the right ventricle

• branch peripheral pulmonic stenosis - the right or left pulmonary artery is narrowed, or both may be narrowed

The following are the most common symptoms of pulmonary stenosis.

• heavy or rapid breathing • shortness of breath • Fatigue , fainting • rapid heart rate • Cyanosis in terminal stages• Severe cases: Distended jugular veins,

hepatomegaly, ascites

• Repair options include the following:1. balloon dilation or valvuloplasty

used for valvar, supravalvar, or branch types of

pulmonary stenosis

2. ValvotomyValvotomy is the surgical release of adhesions that are preventing the valve leaflets from opening properly. This type of procedure is generally not performed, as balloon dilation or valvuloplasty has become more common