Congenital Heart Disease Flashcards
Bicuspid aortic valves are often undetected at birth. TRUE/FALSE?
TRUE
children are normally asymptomatic
Why do patients with bicuspid aortic valves eventually need valve replacement?
Develop aortic stenosis
May also develop aortic regurgitation predisposing to infective endocarditis +/- aortic dilatation/dissection
What accelerates complications in patients with bicuspod aortic valves?
Intense exercise
=> beware in athletes
Describe the two types of Atrial Septal Defect (ASD) and when they tend to present
Ostium SECUNDUM (80%)
- high in septum
- asymptomatic until adulthood when L to R shunt develops
- presents with SOB/HF age 40-60
Ostium PRIMUM (20%)
- low in septum near AV
- associated with Down’s Syndrome
- symptomatic in childhood
Symptoms of Atrial Septal Defect (ASD)
chest pain
palpitations
SOB
arrhythmias
Signs of ASD on examination
Increased JVP
Wide fixed split S2
Pulmonary systolic murmur
Pulm/Tricuspid Regurg.
ECG findings in ASD
RBBB with left axis deviation (PRIMUM)
RBBB with right axis deviation (SECUNDUM)
CXR findings in ASD
Small aortic knuckle
atrial enlargement
Complications of ASD
Reversal of L to R shunt (Eisenmenger’s syndrome)
the L to R shunt causes chronic pulmonary HTN and high R heart pressures. These can then exceed the L and cause a R to L shunt
Symptoms of Eisenmengers syndrome
Cyanosis
R to L shunt causes deoxygenated blood to enter systemic circulation
Paradoxical emboli
- venous clots enter arterial circulation via ASD
Management of ASD
May close spontaneously
PRIMUM - need closed in childhood if symptomatic
SECUNDUM - closed if symptomatic or signs of RV overload
transcatheter closure
Symptoms of VSD
severe HF in infancy
may be asymptomatic and found later in life
Signs of VSD on examination
harsh pansystolic murmur at left sternal edge
systolic thrill
Left parasternal heave
Complications of VSD
Aortic regurgitation
Infective endocarditis
Pulmonary hypertension
Eisenemenger’s syndrome
Heart failure
ECG findings in VSD
Normal
Left axis deviation
LVH
CXR findings in VSD
normal if VSD is small
cardiomegaly and marked pulmonary plethora if large VSD
Treatment of VSD
Many close spontaneously
Surgery if symptomatic and medical tx not helping
if shunt >3:1
infective endocarditis
What condition’s hallmark is a narrowing of the descending aorta just distal to the origin of the left subclavian artery?
Coarctation
Is coarctation of the aorta more common in males or females?
Males
Name 2 conditions that aortic coarctation is associated with
Bicuspid aortic valve
Turners syndrome
Signs of coarctation of aorta on examination
radiofemoral delay
weak femoral pulse
raised BP
scapular bruit/systolic murmur
Cold feet
Complications of aortic coarctation
Heart failure (increased afterload)
infective endocarditis
intracerebral haemorrhage
CXR finding in aortic coarctation
rib notching
(blood diverted via intercostal arteries which dilate and erode local ribs)
Treatment of coarctation
Surgery:
Balloon dialtation +/- stenting
What causes Tetralogy of Fallot?
Abnormality in separation of truncus arteriosus into aorta and pulmonary arteries early in gestation
4 features of Tetralogy of Fallot
VSD
Pulmonary stenosis
Overriding aorta
RVH
How do children normally present with TOF?
Agitated and restless during hypoxic spells
They often “squat” to increase peripheral vascular resistance and reduce the R to L shunt
Symptoms of TOF in an adult who has had surgical repair
SOBOE
palpitations
clubbing
RV failure
syncope
death
ECG findings in Tetralogy of Fallot
RVH and RBBB
CXR findings in Tetralogy of Fallot
may be normal
HALLMARK = boot shaped
What investigation can be used to assess the degree of stenosis in Tetralogy of Fallot?
ECHO
Treatment of Tetralogy of Fallot
Surgery at <1 year old
Closure of VSD and correction of pulmonary stenosis
Without surgery, what is the mortality of TOF?
95% by age 20 without intervention
