Tetralogy of Fallot

Image: "Tetralogy of Fallot" by Mariana Ruiz (LadyofHats) is in the public domain. Link to the source.

Definition

Tetralogy of fallot is a congenital heart defect with 4 features:  an overriding aorta, pulmonary stenosis, large ventricular septal defect and right ventricular hypertrophy.

Aetiology

Combination of genetic and environmental factors.

Pathophysiology

As a result of the numerous heart abnormalities, the right ventricle pressure increased, resulting in less oxygenated blood being pumped to the lungs. This results in decreased perfusion to the body and cyanosis, characterised by the skin and mucous membranes taking on a bluish skin colour.

Risk factors

  • A history of congenital heart defects in the family.

  • Utilisation of specific medications during pregnancy.

  • Some genetic disorders.

Sign and symptoms

  • Cyanosis. 

  • Shortness of breath (SOB).

  • Fatigue.

  • Heart murmur.

  • Failure to thrive.

Investigations

  • Foetal screening ECHO. 

  • Physical exam: severe cyanosis at birth.

  • Antenatal uss - performed in suspected mothers.

  • CXR ( boot-shaped heart ).

  • ECG: RBBB (after surgery).

Management

  • Emergency surgery.

  • If surgery can’t be performed immediately, symptom relieve e.g oxygen therapy, painkiller and beta-blockers.

 
 
 

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