Tetralogy of Fallot

Question 1

what ees eet

Answer 1

rare form of congenital cardiac disease

Question 2

clinical features? (PROVe)

Answer 2

Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta
Ventricular septal defect

Question 3

when if tof usually diagnosed?

Answer 3

most commonly antenatally or on detection of a murmur in the first few months of life

Question 4

babies can present with tet spells - what are these? cause?

Answer 4

acute episodes of cyanosis

Pulmonary stenosis cause a right-to-left shunt of deoxygenated blood across the VSD > hypoxia, pain (inconsolable crying) and cyanosis

Question 5

Management of tet spells?

Answer 5

Analgesia and supplemental oxygen

Last line med: vasoconstrictive agents e.g. phenylephrine (help to increase systemic vascular resistance, reducing the right-to-left shunt and improving cyanosis)