Reading Time:
Pathology
The most common cyanotic congenital heart disease consisting of:
1) Ventricular septal defect
2) Pulmonary stenosis
3) Overriding aorta
4) Right ventricular hypertrophy
Aetiology
Associated with DiGeorge Syndrome
Signs
Cyanosis in the first few days of life as the ductus arteriosus closes,
Ejection systolic murmur associated with pulmonary stenosis, squatting
improves cyanosis, clubbing, polycythaemia, hypoxia, right ventricular heave
and harsh S2 heart sound.
Symptoms
Failure to thrive
Investigations
ECG: Right axis deviation, right ventricular hypertrophy and right bundle branch
block
Chest X-Ray: Boot-shaped heart and decreased pulmonary vasculature
Echo: To assess for structural abnormality and confirm diagnosis
Treatment
Hypercyanotic spells: Place in knee-chest position, administer oxygen,
morphine and propanolol
Surgical: Blalock-Taussig shunt to increased pulmonary blood flow, when
infants are symptomatic with cyanosis.
Prognosis
Patients generally remain asymptomatic post surgical correction and have a
normal life expectancy
Arrhythmias, pulmonary regurgitation, heart failure and infective endocarditis.
Key Facts
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key Images
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.
Key References
Lorem ipsum dolor sit amet, sapien platea morbi dolor lacus nunc, nunc ullamcorper. Felis aliquet egestas vitae, nibh ante quis quis dolor sed mauris.