congenital heart defects Flashcards
what condition its VSD most associated with?
fetal alcohol syndrome
what condition is PDA most associated with?
congenital rubella
what condition is ASD associated with?
ASD (ostium primum type) is associated with Down’s syndrome.
what condition is transposition of the great vessels associated with?
maternal diabetes
what condition is turners syndrome most associated with?
coarctation of the aorta -causes hypertension in upper extremities and weak pulses in the lower extremities.
what happens in eisenmengers syndrome?
Left to right shunts (ASD, VSD, and PDA) will cause increased flow in the pulmonary circulation leading to pathologic remodelling of vasculature and pulmonary hypertension.
RVH occurs to compensate and the shunt is reversed (now right to left). This will present as late cyanosis, polycythaemia and clubbing.
how is polycythaemia treated?
venesection
how long does the PDA take to close in term babies?
72hrs
what are the acyanotic heart conditions?
- ventricular septal defect
- atrial septal defect
- coarctation of the aorta
- patent ductus arteriosus
- all cause left to right shunting
what are the cyanotic heart defects?
- tetralogy of Fallot
- transposition of the great arteries
3, truncus arteriosus
what type of murmur is present with atrial septal defect?
ejection systolic murmur, fixed splitting of S2- because of prolonged emptying of the right ventricle which delays pulmonic closure
what type of murmur is present with ventricular septal defect?
pan-systolic murmurloudest at theleft lower sternal border
palpable thrill - larger VSD can cause parasternal heave and displaced apex beat
what type of murmur is present with a patent ductus arteriosus?
continuous crescendo-decrescendo“machinery” murmur
what is the effect of pulmonary hypertension on heart sounds?
Loud P2:loud second heart sound due to forceful shutting of the pulmonary valve
what type of management is used for VSD when Eisenmenger’s is present?
closure not recommended and can use pulmonary vasodilators like sildenafil
which type of ASD is the most common?
ostium secundum
which type of ASD is Holt-Orman syndrome associated with?
ostium secundum ASD - characterised by skeletal abnormalities of the hands and arms and heart problems
what ECG changes would be present in someone with ostium secundum ASD?
- RBBB
- right axis deviation
what ECG changes would be present with ostium primum?
- RBBB
- LAD
- prolonged PR interval
how can ASD cause stroke in someone with DVT?
ormally when patients have a DVT and this becomes an embolus, the clot travels to the right side of the heart, enters the lungs and becomes a pulmonary embolism. In patients with an ASD the clot is able to travel from the right atrium to the left atrium across the ASD. This means the clot can travel to the left ventricle, aorta and up to the brain, causing a large stroke.
how can patent ductus arteriosus be treated?
IV indomethacin for 3-8 days- blocks the prostaglandins which are keeping the duct open
what is the most common cyanotic defect?
tetralogy of Fallot
which congenital heart defect is at particular increased risk of endocarditis?
- VSD- give prophylactic antibiotics when operating
what are the 4 defects of tetralogy of Fallot?
- pulmonary stenosis
- right ventricular hypertrophy
- overriding aorta
- ventricular septal defect
pulmonary stenosis and ventricular hypertrophy increases the pressure in the right side and encourages blood through the VSD and into the overriding aorta
more deoxygenated blood going to the body causes cyanosis
which defect will show boot shaped heart in CXR?
tetralogy of Fallot
what are the 2 types of management for tetralogy of Fallot?
in neonates, aprostaglandin infusioncan be used to maintain theductus arteriosus. This allows blood to flow from the aorta back to the pulmonary arteries.
Total surgical repairbyopen heart surgeryis the definitive treatment, however mortality from surgery is around 5%.
what is a TET spell?
babies who have tetralogy of Fallot will suddenly develop deep blue skin, nails and lips after crying or feeding, or when agitated
how can a tet spell be managed?
- older children can squat and can do knee to chest for babies- this increases vascular resistance and may encourage blood to go to pulmonary arteries
- supplementary oxygen
- beta blockers- relaxes right ventricle and can increase blood flow to pulmonary vessels
- IV fluids- increases preload and volume of blood to pulmonary vessels
- morphine- to decrease Respiratory drive and have more effective breathing
- sodium bicarb- to buffer acidosis
- phenyl epinephrine- increases systemic vascular resistance
what happens in transposition of the great arteries?
- the aorta and the pulmonary artery are switched
what are the 3 options for treating transposition of the great arteries?
Aprostaglandininfusion can be used to maintain theductus arteriosus. This allow blood from the aorta to flow to the pulmonary arteries for oxygenation.
Balloon septostomyinvolves inserting a catheter into theforamen ovalevia the umbilicus, and inflating a balloon to create a largeatrial septal defect. This allows blood returning from the lungs (on the left side) to flow to the right side of the heart and out through the aorta to the body.
Open heart surgeryis the definitive management. Acardiopulmonary bypassmachine is used to perform an “arterial switch” procedure within a few days of birth. If present, a VSD or ASD can be corrected at the same time.
what is truncus arteriosus?
the aorta and pulmonary arteries fail to separate- oxygenated and deoxygenated blood mix
what is ebsteins anomaly?
the tricuspid valve doesnt form propery - leading to regurg and right atria enlargememnt - can lead to palpatations and arrhythmias
what is a risk factor for ebstiens anomaly?
lithium exposure
is ebsteins anomally cyanotic or acyanotic?
cyanotic- causes right to left shunting
