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Questions and Answers
What determines whether an infant with Tetralogy of Fallot is cyanotic or acyanotic?
Which of the following statements about Pulmonary artery anatomy in Tetralogy of Fallot is true?
Which maternal condition is associated with an increased risk of Congenital Heart Disease (CHD)?
How does significant pulmonary obstruction affect an infant with Tetralogy of Fallot?
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What role does the ventricular septal defect (VSD) play in the physiology of Tetralogy of Fallot?
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Study Notes
Tetralogy of Fallot
- Maternal exposure to certain substances increases the risk of Tetralogy of Fallot (TOF)
- TOF pathophysiology and management are dictated by 3 anatomical factors:
- Degree of right ventricular outflow tract obstruction: Determines if the infant is cyanotic or acyanotic
- Pulmonary artery anatomy: Factors influencing blood flow and physiology
- Murmur: The typical murmur may disappear during a spell, requiring intervention
Diagnosis and Investigations
- Examination of cyanotic infants is critical for diagnosis
- Severe TOF may present with tachypnoea and cyanosis
- Transthoracic 2-dimensional and Doppler echocardiography is the preferred diagnostic tool
Management
- Immediate surgical intervention is necessary for severely cyanotic infants
- Treatment options for infants with TOF and severe pulmonary obstruction include:
- Surgical shunt
- Transcatheter stent/balloon
- Complete repair
- ECMO can be used to reverse cyanosis and acidosis before surgery
- Complete surgical repair is the primary long-term management goal
Follow-up
- Regular assessments by a paediatric cardiologist are required
- Neurodevelopmental follow-up is routine
- Adult patients with repaired TOF should be followed by a cardiologist with CHD expertise
- Pre-conception counseling and management during pregnancy is crucial
- Cardiac MRI and echocardiograms evaluate RV function and pulmonary stenosis progression
- Multi-modality imaging approaches tailored to patient needs are often used
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Description
This quiz covers essential aspects of Tetralogy of Fallot (TOF), including its pathophysiology, diagnosis, and management strategies. It emphasizes the anatomical factors influencing TOF, critical diagnostic investigations, and treatment options for affected infants. Test your understanding of this congenital heart defect and its clinical implications.
