Tetralogy of Fallot

Pathology:                  

The most common cyanoticcongenital heart disease consisting of:

1)     Ventricularseptal defect

2)     Pulmonarystenosis

3)     Overridingaorta  

4)     Rightventricular hypertrophy

Aetiology:                    

‍Associatedwith DiGeorge Syndrome

Symptoms:                  

‍Failureto thrive

Signs:                              

‍Cyanosis in thefirst few days of life as the ductus arteriosus closes,

Ejection systolic murmur associated withpulmonary stenosis, squatting  

improves cyanosis, clubbing, polycythaemia,hypoxia, right ventricular heave

and harsh S2 heart sound.

Investigations:          

ECG: Right axis deviation, rightventricular hypertrophy and right bundle branch block

Chest X-Ray: Boot-shapedheart and decreased pulmonary vasculature

Echo: Toassess for structural abnormality and confirm diagnosis

Treatment:                  

‍Hypercyanotic spells: Place in knee-chest position, administeroxygen,

morphine and propanolol

Surgical: Blalock-Taussig shunt to increased pulmonary blood flow, when

 infants aresymptomatic with cyanosis.

Complications:          

Arrhythmias,pulmonary regurgitation, heart failure and infective endocarditis

Prognosis:                    

‍Patientsgenerally remain asymptomatic post surgical correction and have a

normal life expectancy

Figure 1.19 Tetralogy Of Fallot