Y4 - Paediatric Cardiology Flashcards
What sort of congenital heart defects can cause decompensation and heart failure?
All of them
In which situation will you get cyanosis?
Only if there is a right to left shunt
What investigations should you do for suspected congenital heart disease?
FBC, CXR, PaO2, ECG, echo, cardiac catheter/advanced imaging techniques
How should you manage heart failure in babies with congenital heart disease?
Sit up right, oxygen, calories via NG tube, diuretics (e.g. furosemide)
Duct dependent conditions req. prostaglandins to keep the duct patent (alprostadil)
Transfer to neonatal cardiac centre
What are the symptoms of a small VSD?
Usually mild
What are the symptoms of large VSDs?
Tachypnoea, hypoxia, difficulty feeding, irritability, tachycardia, growth faltering
When do kids with VSD tend to present and why?
Tend to present between 3-6 weeks
Lung vasculature in neonate is very stiff and so pressure in lungs is very high, but as child grows older, gradually the vasculature relaxes and the pressure falls (lowest at 6 weeks)
Between 3-6w reaches critical level and floods the lungs –> pulmonary oedema and hypoxia
What are the signs of VSD?
Loud harsh pansystolic murmur at L sternal edge + thrill
What is the course of a VSD?
Small ones & muscular ones tend to close on their own
Larger ones more likely to need surgery
What is a VSD?
A hole connecting the ventricles
True or false:
In VSD, smaller holes give louder murmurs.
True
What is a major complication of VSD?
Pulmonary hypertension as pulmonary vessels become stiff
How do you manage VSD?
Diuretics and vasodilators
Optimise feeding to improve weight
Open heart surgery if large and req.
What is an ASD?
Failure of closure of the septum between the atria
What does the atrial septum do?
Separates the right and left atria
Describe the development of the atrial septum
- Septum primum grows downward & leaves gap at bottom (ostium primum)
- Septum primum fuses with endocardial cushion closing gap between R & L atria completely
- Hole appears in upper part of septum primum (ostium secundum)
- Septum secundum grows down parallel & to the right of septum primum which covers the ostium secundum but leaves an opening (foramen ovale)
(This all creates a one way valve allowing blood to go from the R to L atrium)
What is the function of the foramen ovale in the foetal circulation?
It allows the already oxygenated blood (from the placenta) to bypass the R ventricle and lungs and go into the L atrium
What happens to the septum secundum & primum at birth?
They snap closed and fuse to close the foramen ovale
What are most ASDs due to defects in?
Ostium secundum (secudum septum doesn’t form properly)
What conditions are ASDs related to?
Down’s syndrome
Foetal alcohol syndrome
What is the pathophysiology of an ASD?
Higher pressure in L side of heart means blood is shunted from L to R
True or false:
ASD is a cyanotic heart defect
False
Acyanotic
What sort of murmur do you get in ASD?
Ejection systolic murmur in pulmonary area
Extra blood in R atrium & therefore R ventricle so more blood and therefore turbulence as the blood is going through the pulmonary artery
What other sign may indicate ASD?
Wide and fixed second heart sound
P2 is widened as there is more blood in the RV and so more blood passing through the pulmonary artery on systole so valve takes longer to close than the aortic
Fixed as consistently more blood being pumped (unlikely physiological splitting of HS on inspiration due to increased intrathoracic pressure)
What causes physiological splitting of the second heart sound?
Inspiration increases intrathoracic pressure, which pulls more blood into the right side of the heart which means there is more blood pumping through the pulmonary artery and thus it closes later than the aortic valve
How do most people with ASD present?
Most asymptomatic
How would you diagnose as ASD?
Using echo
How do you Rx ASD?
If very small may close on its own
Ample T device (access through femoral)
Larger ones may req. open heart surgery
What condition is ASVD associated with?
Down’s (40-50% of Down’s babies will have one)
What parts of the heart does an ASVD involve?
Atrial and ventricle septa
AV valves
True or false:
ASVD is an acyanotic condition
True
What does an ASVD present like?
Like a large VSD
If very big may get symptoms of congestive heart failure & pulmonary congestion
Poor feeding, tachycardia, tachypnoea, excessive sweating etc.
A defect in which embryonic structure is responsible for ASVD?
Endocardial cushion
How old are children when they present with ASVD & why?
3-6w
Same reason as VSD
What signs will you see of ASVD on ECG?
L axis deviation and RV hypertrophy
What is used to confirm the diagnosis of ASVD?
Echo
What is the management of AVSD?
Vasodilators & diuretics
Try and wait until 4-12m old until surgery
What does the ductus arteriosus do during development?
Connect aortic arch to the pulmonary artery
Foetuses do not rely on the lungs & req. oxygenated blood from the placenta (most of this blood flows from foramen ovale and that which doesn’t goes to pulmonary artery (where most of blood shunted to aorta via DA))
When does the ductus arteriosus usually close?
After birth
What does the ductus arteriosus become after birth?
Ligamentum arteriosum
What is patent ductus arteriosus?
When the ductus arteriosus stays open after birth
What keeps the ductus arteriosus patent during development?
Prostaglandin E2 (vasodilator) Made by placenta and DA
After birth what causes the DA to close?
Fall in levels of prostaglandin E2
Lungs also produce bradykinin which constricts smooth muscle walls of DA
When does the DA usually close?
Between 48-72h
Why is a PDA a problem?
L to R shunt from aorta into pulmonary artery as pressure in L side of heart higher than R
How do most people with PDA present?
Usually asymptomatic Continuous murmur (harsh)
What complications may occur later in life from a PDA?
Pulmonary hypertension due to increased pulmonary volume
In which group of individuals is PDA most problematic?
Premature babies
How do manage PDA?
If premature or persistent PDA (>6m)
Indomethacin/ibruprofen
Surgery - e.g. T. coil closure/simple device closure
What must you do before administering ibruprofen to treat an PDA?
Monitor for IVH and check kidney function
What is coarctation of the aorta?
Narrowing of the ventricles
What are the two forms of coarctation of the aorta?
Infant/preductal coarctation - coarctation comes after aortic arch & before ductus arteriosus
Adult - no PDA, no mixing of blood, blood flow increases to head and upper extremities (increases risk of berry aneurysms), dilation of aortic valve & aortic dissection, lower BP in extremities (claudication)
What is the typical presentation of coarctation of the aorta?
Cyanosis of lower limbs Collapse, pale/grey Poor sats and circulation Absent femorals If critical won't feel any pulse
Without intervention what can occur in aortic coarctation?
Death in the neonatal period
What condition is coarctation of the aorta strongly associated with?
Turner’s syndrome (45 X)
How is aortic coarctation managed?
Prostaglandins to keep PDA open
Operation within 48h at specialist centre (e.g. balloon dilatation)
What is transposition of the great arteries?
Aorta coming from RV
Pulmonary artery coming from LV
Creating two closed circuits where the systemic and pulmonary circuits don’t link
Blood on right side ever gets oxygenated and is pumped to the body
Blood on left side never gets deoxygenated
What is the presentation of transposition of the great arteries?
Cyanosis
Dyspnoea
Distressed
Oxygen doesn’t help
What are the great arteries?
Pulmonary artery and aorta
What is the ductus venosus?
Duct containing the umbilicus to the inferior vena cava
What is lifesaving in TGA?
Giving prostaglandins early until surgery can occur at a cardiac centre
What defects may be lifesaving in TGA?
PDA
VSD
ASD
True or false:
TGA is a cyanotic condition
True
What surgery is used for TGA?
Switch procedure
What are the conditions in tetralogy of fallot?
- Stenosis of the right ventricular outflow tract (narrowing of valve/area below valve)
- Right ventricular hypertrophy
- VSD
- Overriding aorta
In ToF what does stenosis of the RV outflow tract lead to?
Difficulty for the blood to get into the pulmonary artery which leads to RVH in order to push blood against the stenosed pulmonary valve
What finding will you see on CXR in ToF and why?
Boot shaped heart (due to RVH)
What is different about VSD in ToF and VSD in patients with isolated VSD?
In ToF, pulmonary stenosis leads to really high pressures in RV so there is a R to L shunt
How do people with ToF present?
Cyanosis
Cyanotic spells (cry/feeding leads to increased cyanosis & can lose consciousness/have seizures)
FTT
Feeding difficulties
What kind of murmur do you get in ToF?
Ejection systolic murmur
P2 very quiet
What component of ToF causes cyanosis?
RV outflow tract obstruction as it increases pressure in RV leading to the R to L shunt
How do you manage ToF?
Oxygen, IV fluids, beta-blockers (e.g. propranolol)
Corrective surgery at 6m with shunt in interm
What is the prognosis of ToF based on?
Degree and extent of RV outflow tract obstruction
What are the 6 grades for loudness systolic murmurs?
- Just audible
- Quiet, but easily audible
- Loud but no thrill
- Loud with thrill
- Audible even if stethoscope only makes partial contact w. skin
- Audible without stethoscope
What does inspiration augment?
Systemic venous return negative pressure draws blood from abdomen into thorax) & so pulmonary stenosis and tricuspid regurg murmurs
What does expiration augment?
Pulmonary venous return therefore VSD, mitral incompetence, aortic stenosis