Congenital Heart Conditions Flashcards
What are the most common causes of Acyanotic Congenital heart disease
ventricular septal defects (VSD) - most common, accounts for 30% atrial septal defect (ASD) patent ductus arteriosus (PDA) coarctation of the aorta aortic valve stenosis
VSDs are more common than ASDs
true
However, in adult patients ASDs are the more common new diagnosis as they generally presents later.
What are the most common causes of cyanotic Congenital heart disease
tetralogy of Fallot
transposition of the great arteries (TGA)
tricuspid atresia
When does fallot’s present?
Fallot’s generally presenting at around 1-2 months
Fallot’s is more common than TGA. However, at birth TGA is the more common lesion as patients with Fallot’s generally presenting at around 1-2 months
The presence of cyanosis in pulmonary valve stenosis depends very much on the severity and any other coexistent defects.
true
Peripheral cyanosis, for example of the feet and hands, is very common in the first 24 hours of life
true
may occur when the child is crying or unwell from any cause
Central cyanosis can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds
5g/dl
In Cyanosis in the neonatal period what test differentiates cardiac from non-cardiac causes?
nitrogen washout test
nfant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease
Initial management of suspected cyanotic congenital heart disease
supportive care
prostaglandin E1
used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect
What is acrocyanosis
refers to the peripheral cyanosis around the mouth and the extremities (hands and feet)
often seen in healthy newborns
What causes acrocyanosis?
It is caused by benign vasomotor changes that result in peripheral vasoconstriction and increased tissue oxygen extraction and is a benign condition
How can acrocynaosis be differentiated from other causes of peripheral cyanosis with significant pathology?
it occurs immediately after birth in healthy infants. It is a common finding and may persist for 24 to 48 hours.
What is the most common cause of cyanotic congenital heart disease?
Tetralogy of Fallot (TOF)
however, at birth transposition of the great arteries is the more common lesion as patients with TOF generally present at around 1-2 months
When does TOF present?
It typically presents at around 1-2 months, although may not be picked up until the baby is 6 months old
What are the features of TOF?
Ventricular septal defect (VSD)
Right ventricular hypertrophy & outflow tract obstruction
Pulmonary stenosis
Overriding aorta
TOF is a result of what malalignment?
anterior malalignment of the aorticopulmonary septum
The severity of the right ventricular outflow tract obstruction determines the degree of cyanosis and clinical severity
What are the clinical features of TOF?
cyanosis
causes a right-to-left shunt
ejection systolic murmur due to pulmonary stenosis
ECG shows right ventricular hypertrophy
a right-sided aortic arch is seen in 25% of patients
What does CXR show inTOF?
chest x-ray shows a ‘boot-shaped’ heart,
What is the management of TOF?
surgical repair is often undertaken in two parts
cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm
What causes Transposition of the great arteries?
caused by the failure of the aorticopulmonary septum to spiral during septation
Who is most at increased risk of TGA?
Children of diabetic mothers are at an increased risk of TGA.
What are the anatomic changes in TGA?
aorta leaves the right ventricle
pulmonary trunk leaves the left ventricle
What are the features of TGA?
cyanosis
tachypnoea
loud single S2
prominent right ventricular impulse
What does CXR show in TGA?
‘egg-on-side’ appearance on chest x-ray
What is the management of TGA
maintenance of the ductus arteriosus with prostaglandins
surgical correction is the definite treatment.
What is the most common cause of congenital heart disease?
Ventricular septal defect
VSDs close spontaneously in around 50% of cases.
true
What is the aetiology of VSD?
congenital VSDs are often association with chromosomal disorders: Down’s syndrome, Edward’s syndrome, Patau syndrome, cri-du-chat syndrome
congenital infections
post-myocardial infarction
VSDs may be detected in utero during which scan
the routine 20 week
Post-natal presentations of VSD include
failure to thrive
hepatomegaly
features of heart failure
tachypnoea, tachycardia, pallor
classically a pan-systolic murmur which is louder in smaller defects
What is the management of VSD?
small VSDs which are asymptomatic often close spontaneously are simply require monitoring
moderate to large VSDs usually result in a degree of heart failure in the first few months - surgical closure of the defect?
Otherwise nutritional support, medication for heart failure e.g. diuretics
What are the complications of VSD?
infective endocarditis
right heart failure
aortic regurgitation: due to a poorly supported right coronary cusp resulting in cusp prolapse
Eisenmenger’s complex: due to prolonged pulmonary hypertension from the left-to-right shunt results in right ventricular hypertrophy and increased right ventricular pressure. This eventually exceeds the left centricular pressure resulting in reversal of blood flow this is turn results in cyanosis and clubbing Eisenmenger’s complex is an indication for a heart-lung transplant
pulmonary hypertension: pregnancy is contraindicated in women with pulmonary hypertension as it carries a 30-50% risk of mortality
Patent ductus arteriosis (PDA) can only be acyanotic
False
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis
What is PDA?
connection between the pulmonary trunk and descending aorta
PDA is more common in?
premature babies, born at high altitude or maternal rubella infection in the first trimester
How does ductus arteriosus close in first breaths?
increased pulmonary flow which enhances prostaglandins clearance
Features of PDA?
left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat
Management of PDA?
indomethacin or ibupofen - inhibits prostaglandin synthesis & closes the connection in the majority of cases
if associated with another congenital heart defect amenable to surgery then prostaglandin E1 is useful to keep the duct open until after surgical repair
Coarctation of the aorta is associated with which syndrome?
Turner’s syndrome
HOWEVER it is more common in males overall
What are the features of Coarctation of the aorta?
infancy: heart failure
adult:
hypertension
radio-femoral delay, mid systolic murmur, maximal over back, apical click from the aortic valve
notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children
What is coarctation of the aorta associated with?
bicuspid aortic valve
berry aneurysms
neurofibromatosis
