Congenital Cardiac Defects Flashcards
What is a huge difference between adult and paediatric cardiac failure?
Cardiac failure in children does not have the sign of oedema
At what age can ankle oedema occur in cardiac failure in a child?
3 years onwards
What are the signs and symptoms of cardiac failure in a child?
- SOB
- Poor feeding
- Tachycardia
- Hepatomegaly (only in RHS heart failure)
- Poor pulses
- Acidosis
- Sweating
What is a VSD?
Ventricular Septal Defect
What are the 3 heart problem categories in children?
- Congenital (born with it)
- Acquired
- Inherited
What is the incidence of congenital heart disease per 1000 live births?
8 per 1000 live births (0.8%)
What percentage of babies with CHD are diagnosed within 2/52 of age?
30-40%
What percentage of babies with CHD are diagnosed within 4/52 of age?
60%
What are the acyanotic causes of CHD?
- Septal defects (VSD or ASD)
- Aortic stenosis
- Pulmonary stenosis
- Coarctation of the aorta
- PDA
- Mitral stenosis
- Tricuspid stenosis
What sort of murmur is produced by a VSD?
Pansystolic murmur (holosystolic)
Describe the murmur and pulses in a patient with PDA.
Continuous murmur with bounding pulses
What would we find on examination of a baby/child with coarctation of the aorta?
- Weak/absent femoral pulses
- Systemic hypertension in the upper limbs
- Aorta can be interrupted in severe forms - blue, mottled, pulseless, cold legs
What is coarctation of the aorta?
A narrowing in the descending aorta, this causes weakened or interrupted blood flow to the lower extremities
What is a common complication of coarctation of the aorta?
Necrotising enterocolitis due to the reduced blood supply to the gut
What percentage of CHD patients have cyanotic CHD?
30%
Name the most common 3 causes of cyanotic CHD.
- Fallot’s tetralogy
- Transposition of Great Arteries (TGA)
- Complete Atrio-Ventricular Septal Defects (CAVSD)
What is transposition of the great arteries (TGA)?
This is where the pulmonary artery and the aorta fail to switch to the correct ventricle on birth (e.g. the pulmonary arteries come from the left ventricle and supply the lungs with blood and the aorta comes from the right ventricle and supplies the rest of the body with blood). This means blood will not be oxygenated in the lungs, hence the cyanotic presentation.
What is Fallot’s Tetralogy (TOF) - It’s 4 components?
- Large VSD - L to R shunt
- Overriding aorta - enlarged aortic valve and seems to rise from the left and right ventricles
- Pulmonary artery stenosis - narrowing
- Right ventricular hypertrophy
What is the murmur heard in TOF?
Loud ejection systolic murmur
What are children who have TOF prone to?
Hypercyanotic episodes
This means they have a constant baseline of cyanosis, however this can worsen at random times where the become profoundly cyanosed and terribly blue.
What heart defect can commonly occur in trisomy 21?
AVSD
What do we usually see on an ECG of someone with an AVSD?
Left-axis deviation
What percentage of children with Trisomy 21 (Down’s) have CHD?
40%
What percentage of children with trisomy 13 (Patau’s) have CHD?
80%
What percentage of children with trisomy 18 (Edward’s) have CHD?
80-100%
How is Di-George syndrome inherited?
Autosomal-dominant
What are the features of an autosomal dominant inheritance?
- Males and females affected equally
- Transmitted by both sexes
- Incomplete penetrance - might have the gene but no symptoms
- Variable expression - affects individuals differently
- New mutations
- Risk to offspring is 50%
List 3 examples of autosomal dominant inheritance conditions.
- Neurofibromatosis
- Marfan’s syndrome
- Familial adenomatous polyposis
What is tested for on the neonatal heel prick test?
- MCAD
- HCU
- Sickle cell
- Isovalaric acidaemia
- CF
- Hypothyroidism
- Phenylketonuria (PKU)
What are common causes of acquired heart disease in children (even though acquired heart disease is uncommon)?
- Viral pericarditis
- Viral myocarditis (commonly caused by Coxsackie B)
- Kawasaki disease
What are the signs and symptoms a child with acquired heart disease might present with?
- Fever
- Lethargic
- Heart failure symptoms (hepatomegaly, tachycardia, tachypnoea)
- Unwell appearance
What are the common signs/symptoms of Kawasaki disease?
- Swinging fevers - unresponsive to paracetamol
- Lymphadenopathy
- Red eyes - no pus
- Rash (macular)
- Red, swollen tongue
- Swollen feet and hands with peeling skin from the digits
- Platelet rise in week 2 (up to a million)
What is a severe complication of Kawasaki disease? How do we prophylactically treat this?
- Coronary artery aneurysm
This can be avoided by early administration of aspirin and IV gammaglobulin
Why are we more commonly missing CHD antenatally?
Due to maternal obesity which makes the USS scanning more difficult and harder to get a clear picture therefore CHD is being missed
Why are we missing diagnosis of CHD in neonatal patients?
Mum and baby are being discharged earlier than they used to, as they used to be kept for monitoring until day 3/4 on the postnatal ward where signs were becoming clearer.
What oxygen saturations can children with TGA tolerate?
60-70%
