Paeds Cardio Flashcards
What is tetralogy of fallot and its 4 features ?
- key issue: interior dislocation of the septum which partially obstructs RVOT and PA
1. ventricular septal defect: leading to equal ventricular pressures
2. pulmonary stenosis
3. overriding aorta
4. RV hypertrophy
What is the pathophysiology of a VSD and overriding aorta in tetralogy of fallot?
- VSD allows blood to flow between ventricles
- overriding aorta: aortic valve is above VSD allowing deoxygenated blood into aorta
What is stenosis of the pulmonary valve in tetralogy of fallot?
- greater resistance against blood flow from RV
- encourages blood to flow through VSD and into aorta
- causing R to L shunt and cyanosis
What is RV hypertrophy in tetralogy of fallot?
- inc muscular strain on RV
- trying to pump blood against resistance of LV
- causes RV hypertrophy and thickening of heart muscle
How is cyanosis caused in tetralogy of fallot?
- all factors cause R to L cardiac shunt
- blood bypasses lungs and is not oxygenated
- deoxygenated enters systemic circulation
- causes cyanosis
What is the presentation of tetralogy of fallot?
- cyanosis
- clubbing
- dyspnoea on feeding, crying, exertion
- Tet spells: sudden onset dyspnoea/cyanosis
What are the signs of Tetralogy of Fallot?
- ejection systolic murmur
- heard loudest in pulmonary area: 2nd intercostal space on left sternal border
What are risk factors for Tetralogy of Fallot?
- rubella
- age of mother >40
- alcohol in pregnancy
- diabetic mother
What is seen on investigation of tetralogy of fallot?
- echocardiogram
- doppler flow studies
- boot shaped heart due to RV thickening
What is the pathophysiology of a Tet spell?
- intermitten symptomatic period where R to L shunt gets worse
- occurs when pulmonary vascular resistance inc or systemic resistance dec
- blood going from RV to aorta and bypassing pulmonary vessels
What is the presentation of a Tet spell?
- waking, crying
- irritability, cyanosis, SOB
- Severe: reduced consciousness and seizures
How is a Tet spell treated?
- older children squat
- younger: knees to chest
- increases systemic resistance
- supplementary oxygen
- β blockers
- IV fluids
How is tetralogy of fallot managed?
- VSD patched
- PV widened or replaced
- neonates: prostaglandin infusion to maintain ductus arteriosus
What is a ventricular septal defect?
- most common form of congenital heart disease
- malformation of ventricular septum
- if LV pressure > RV then blood flows LV to RV
- inc blood flow through lungs
Why are patients acyanotic in VSD?
- blood flows L to R and flows around the lungs again
- therefore blood is oxygenated
- L to R shunt causes R side overload
- R heart failure and inc flow into pulmonary vessels
What is Eisenmenger syndrome?
- inc blood in RV > inc pulmonary > pulmonary hypertension
- causes R to L shunt due to inc pressure in R side
- patient becomes cyanotic as blood is bypassing lungs