congenital heart disease Flashcards
commonest causes of acyanotic congenital heart diseases
ventricular septal defects - commonest (30%)
atrial septal defects
patent ductus arteriosus
coarcation of aorta
aortic valve stenosis
VSDs vs ASDs in kids vs adults
VSDs overall are more common
in adults -> ASDs more common new diagnosis, generally present later
commonest causes of cyanotic congenital heart disease
tetralogy of fallot (commonest)
transposition of great arteries
tricuspid atresia
presentation age of TGA vs tetralogy
TGA -> at birth
tetralogy -> 1-2months
peripheral cyanosis in first 24hrs of life
v common, may occur when child is crying or unwell from any cause
how is central cyanosis clinically recognised
conc of reduced Hb in blood >5g/dl
test given to cyanotic neonates
nitrogen washout test (heperoxia test)
- used to differentiate between cardiac vs non-cardiac causes
given 100% o2 for 10mins, then ABGs taken
- pO2 <15 = cyanotic congen heart disease
intial management of suspected cyanotic congenital heart disease
suportive care
prostaglandin E1 - elprostadil
- used to maintain patent ductus arteriosus in ductal dependant congen heart dis
(acts as holding measure until a definite diagnosis is made/surgical correction done)
acrocyanosis
cyanosis around mouth + extremities - hanfs/feet
- seen in healthy newborns
- seen immediately after birth
- may persist for 25-48hrs
what age does tetralogy of fallot typically present
around 1-2months
but may not be picked up until 6months
4 characteristics of tetralogy of fallot
Pulmonary stenosis
Overriding Aorta
Ventricular Septal Defect (VSD)
Right ventricular hypertrophy
what is does the degree of clinical severity and cyanosis depend on in tetralogy of fallot
right ventricular outflow tract obstruction (pulmonary stenosis)
what are tet spells?
unrepaired tetra of fallot develop episodic hypercyanotic “tet” spells
- due to near occlusion of right ventricular outflow tract
px - tacypnoea, severe cyanosis, occasionally loss of consciousness
typically occur when upset, in pain or fever
murmur heard in tetralogy of fallot
ejection systolic
- due to pulmonary stenosis
Chest Xray + ECG of tetralogy of fallot
“boot-shaped” heart
ECG - ventricular hypertrophy, right axis deviation
management of tetralogy of fallot
- surgical repair undertaken in 2 parts
- cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm
Transposition of the Great Arteries (TGA)
cyanotic congenital heart disease
failure of aorticopulmonary septum to spiral during septation
- aorta leaves right ventricle
- pulmonary trunk leaves left ventricle
who is at increased risk of Transposition of the Great Arteries (TGA)?
kids of diabetic mums
Transposition of the Great Arteries (TGA) presentation
cyanosis
tachypnoea
loud single S2
prominent right ventricular impulse
egg on side appearance on CXR
management of Transposition of the Great Arteries (TGA)
maintenance of ductus arteriosus with prostaglandins (prostaglandin E1 - elprostadil)
definitive mx = surgical correction
ebsteins anomaly
low insertion of tricuspid valve resulting in a large atrium + small ventricle
= atrialisation of right ventricle
what can cause ebsteins anomaly and other assoc conditions
exposure to lithium in utero
Assoc
- patent foramen ovale or ASD in 80% -> right to left shunt
- wolff-parkinson white
- supraventricular tachycardia
ebsteins anomaly features
cyanosis
prominent “a” wave in distended jugular venous pulse
tricuspid regurg - pansystolic murmur, worse on inspiration
right bundle branch block -> widely split S1 + S2
may be asymptomatic until adolescence/adult
ebsteins anomaly investigation + management
ix = echo
medical = mx arrythmias + heart failure
surgical = tricuspid valve repair/replacement
patent ductus arteriosus
acyanotic - if uncorrected can result in late cyanosis of lower extemeties
connection between pulmonary trunk + ascending aorta
- usually closes with first breaths
which babies is PDA commoner
premature
born at high altitude
maternal rubella infection in 1st tri
patent ductus arteriosus features
left subclavicular thrill
continuous machinery murmur
large volume, bounding, collapsing pulse
wide pulse pressure
heaving apex beat
patent ductus arteriosus management in preterm infants
preterm infants if ventilator dependant after 1 week = pharma closure
- ibuprofen, indomethacin or paracetamol –> (inhibits prostaglandin synthesis)
indication for closure management of patent ductus arteriosus in term infants + kids
- moderate or large PDA
- prior episode of endocarditis
- small audible PDA
management of patent ductus arteriosus in term infants + kids
transcatheter PDA closure
(NOT pharma management, not effective in term infants)
coarctation of aorta
congenital narrowing of the descending aorta
- more common in males, despite assoc with Turners syndrome
coarctation of aorta associations
- turners
- bicuspid aortic valve
- berry aneurysms
- neurofibromatosis
coarctation of aorta features
infancy - heart failure
adults - hypertension
radio-femoral delay
mid-systolic murmur, maximal over back
apical click from aortic valve
notching of ribs - due to collateral vessels (NOT seen in young)`
