1/2: Cardio - Acaynotic Heart Defects Flashcards
How is congenital heart disease in paediatrics divided
Into cyanotic or acyanotic congenital heart defects
What are 4 broad categories of causes of congenital heart disease
- Idiopathic
- Genetic
- Maternal illness - infection or underlying condition
- Maternal exposure to toxins in-utero
What 2 heart conditions is Turner’s Syndrome associated with
- Bicuspid aortic valve
- Coarctation aorta
What 4 heart conditions is DiGeorge’s Syndrome associated with
- TOF
- PDA
- VSD
- CA
What heart condition is William’s syndrome associated with
AS
What 2 heart conditions is rubella associated with
PS
PDA
What does congenital HD increase the risk of in children
Infective Endocarditis
What are the 4 acyanotic congenital heart defects
- ASD
- VSD
- Coarctation of the aorta
- Patent ductus arteriosus
What are the three cyanotic congenital heart defects
- TOF
- Tricuspid atresia
- TGA
What is a way to remember 4 acyanotic CHD
4D’s
What is a way to remember 3 cyanotic CHD
3T’s
Describe circulation in a foetus
- Oxygenated blood passes from mother into umbilical vein via placenta.
- Umbilical vein enters IVC by ductus venous
- Enters right atrium
- Blood right atrium passes to left atrium via foramen oval
- Some passes to right ventricle and out pulmonary.a
- Ligamentum arteriosum in pulmonary.a shunts blood to aorta
- Umbilical vein originates fro, internal iliac
Explain changes in circulation at birth
- Clamping of the umbilical cord increases systemic vascular resistance
- As baby breathes air, alveoli expand and surrounding vessels dilate due to oxygenation. This reduces pulmonary pressure. Reduction in pulmonary pressure increases blood flow to the lungs and hence decreases flow through ductus arteriosus which closes at 10-15h following birth
- Increase systemic vascular resistance increases pressure in right atrium > left atrium causes closure of foramen ovale within 3 minutes
Is peripheral cyanosis a normal or abnormal finding in first 24h
Normal
Is central cyanosis normal or abnormal finding in first 24h
Abnormal
What does central cyanosis indicate
deoxygenated Hb concentration >5
What is used to distinguish respiratory from cardiac causes of neonatal cyanosis
Nitrogen wash out test
What is the nitrogen wash out test
- Baby is given 100% oxygen for 10-minutes then an ABG given to measure PaO2
- If respiratory cause, oxygen should mean deficit resolves. In cardiac cause, additional oxygen will not help
What does a PaO2 of <15kPa indicate on nitrogen wash out test
Cyanotic congenital heart disease is the cause of cyanosis
When is paediatric heart failure most common
3m life
How is does paediatric HF present
- Poor Feeding
- SOB
- Sweating
- FTT
- Sacral oedema (due to lying down)
What type of shunt is present in acyanotic heart disease
Left-to-Right Shunt
What are 5 causes of acyanotic heart disease
ASD VSD Patent Ductus arteriosus Coarctation of aorta Aortic stenosis
Explain general pathophysiology of acyanotic heart conditions
- Due to increased pressure in left side (systemic system) compared to right side blood is shunted left to right
- This means already oxygenated blood passes through pulmonary circulation again
- This can increase pressure in pulmonary circulation causing pulmonary HTN
What can acyanotic heart conditions eventually lead to
Eisenmenger syndrome
What is Eisenmenger syndrome
- Left-to-right shunt increases pulmonary pressure
- Leads to remodelling of pulmonary vasculature which results in permanent pulmonary HTN
- To compensate, right ventricle undergoes hypertrophy
- This increases right-sided pressure reversing shunt from right-to-left causing cyanotic HD
What are the two types of atrial septal defect
- Ostium primum
2. Ostium secondum
What is the most common type of ASD
Ostrium Secondum (70%)
What is the most common congenital HD found in adulthood
ASD
Which gender is ASD more common
Female (3:1)
What syndrome is osteum secondum ASD associated with
Holt-Oram Syndrome
What is holt-oral syndrome called
Hand-Heart Syndrome
What is heart-hand syndrome
Individual presents with ASD and tri-phalangeal thumb
How does ASD present clinically
Asymptomatic in majority.
May present with complications in 3rd-4th decade of life due to pulmonary HTN
How are ASD often detected
On incidental exam
How does osteum primum present
If large defect may present with heart failure
What is a palpable sign of ASD and why does this happen
Right ventricular heave - due to increased blood flow through right-side heart
What murmur is present in ASD and why does it happen
Ejection Systolic - due to increased/more blood flow through narrow (same-size) pulmonary valve causing turbulence
What HS is present in ASD and why does it happen
Split S2 - Due to more blood flow in left side of the heart it takes longer for pulmonary valve to close
Why is ostium primum more serious
As it involves endocardial tissue which is used to form mitral and tricuspid valves. Therefore is also associated with valve regurgitation.
Why is ostium secundum less serious than primum
Does not involve endocardial cushions used for valves
What is first-line investigation in suspected ASD
ECG
What will ECG show in a small shunt
Normal
What will ECG show in larger shunt
- P pulmonale
- Right axis deviation
What may a CXR show in ASD
Pulmonary plethora - evidence increased blood flow to lungs
What is diagnostic of ASD
ECHO
How is ostium primum managed
surgical closure before pre-school
How is ostium secundum managed
surgical closure before school age
How are ASD closed
Either via cardiac catheterisation or open heart surgery
Without repair what will happen in ostium primum
Lead to heart failure in childhood
Without repair what will happen in ostium secundum
Lead to heart failure in 3-4th decade life
What is the most common congenital HD
VSD
What % of congenital HD is VSD
25%
What are VSD caused by
- Chromosomal abberations
- Maternal toxins in-utero
- Congenital infection
What does clinical presentation of VSD depend on
Size
How will a small VSD present
Asymptomatic