Paediatric Cardio Conditions Flashcards
What are the causes of cyanotic congenital heart disease?
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- Truncus arteriosus
- Transposition of the Great Arteries
- Tricuspid / Pulmonary Atresia
- Tetralogy of Fallot
- Total anomolous pulmonary venous return
What are the two classes of acyanotic congenital heart disease?
Obstructive and L-R shunt
What are the causes of obstructive heart disease?
- Hypoplastic L heart syndrome
- AS
- Coarctation of the aorta
- Interruption of the aortic arch
As a general rule, which group of congenital heart defects are ALWAYS duct dependent? How is this treated in the early stages?
Obstructive congenital heart defects (hypoplastic L heart syndrome, AS, interruption of the aortic arch and aortic coarctation). They are treated by PGE1 in the short term, to keep the duct open
As a general rule, which group of congenital heart defects are USUALLY duct dependent? Is it harmful to treat with PGE1, while still in the diagnostic process?
R–>L shunts (1T2T3T4T5T) but this time you depend on the duct being open for blood to flow into the PULMONARY circulation
How and when do obstructive congenital heart defects present?
A shocked neonate. Often on D2 of life when PDA closes. Present as a dusky / grey coloured neonate with weak / absent pulses and a low BP, with a metabolic acidosis.
What are the defects of tetralogy of fallot?
- PULMONARY STENOSIS 2. RVH 3. VSD 4. Overriding aortic arch
When does tetralogy of fallot tend to present and how?
6-12 months of life. “Tet spells” or “hypoxic spells” - cyanosis or LOC / going floppy on feeding, crying or exertion
What is the surgical correction of transposition of the great arteries?
balloon atrial septotomy
What is the surgical correction of hypoplastic L heart syndrome?
Norwood operation
Which two congenital heart defects are associated with DiGeorge syndrome? Which chromosomal abnormality is this and what are the other associated features?
TOF and transpos are both associated with microdeletion of the long arm of chromosome 22. The other associated features are ‘CATCH 22’ Cardiac (trasnpos, TOF) Abnormal (long) facies Thymic hypoplasia Cleft palate Hypocalcaemia / hypoparathyroidism
With which CHD is Turner’s syndrome associated?
Aortic coarctation
Compare the timing of onset of transposition of the great arteries and TOF?
Transpos always presents as cyanosis in the newborn period. TOF presents 6-12 months of life as cyanosis.
What is the most common cause of ACQUIRED heart disease in paediatrics?
Kawasaki disease
What are the diagnostic features of Kawasaki disease?
Fever persisting 5 days or more (usually high, >39 degrees)
AND
4 of the following 5 features
Important note: you can also diagnose Kawasakis with only 3 of the 5 features if you get a positive echo.
- bilateral nonpurulent conjunctivitis
* ie. redness without exudate
- red fissured lips, strawberry tongue, erythema of oropharynx (without tonsillitis or evidence of URTI)
- changes of the peripheral extremities
- acute phase: erythema, edema of hands and feet, groin peeling. May manifest as refusal to weight bear.
- subacute phase: peeling from tips of fingers and toes
- polymorphous rash
- usually begins in the nappy area (where there may be desquamation early in the disease) and spreads to involve the trunk, extremities and face.
- Rash may be maculopapular, annular or scarlatiniform.
- cervical lymphadenopathy > 1.5 cm in diameter
What is the treatment of Kawasaki disease
- Intravenous immunoglobulin (2 g/kg over 10 hours; preferably within the first 10 days of the illness.)
- Aspirin 3 - 5 mg/kg once a day for at least 6 to 8 weeks
What should be organised before a patient treated for Kawasaki disease is discharged?
Follow-up echocardiogram in 6-8 weeks
Kawasaki disease is primarily a clincial diagnosis, but what Ix would you perform (and what might you expect to see)?
Bedside tests ECG - check for ischaemia / infarction Urine dipstick - negative Laboratory Tests FBE - neutrophilia, anaemia, thrombocytosis ESR/CRP - markedly raised LFTs - ALT raised Imaging Echocardiogram
What are the clinical features of a Patent ductus arteriosus?
- Patients with a small PDA are usually asymptomatic
-
Murmur
- Continuous murmur audible at the upper left sternal border or left infraclavicular area – called a machinery murmur
- May disappear during diastole and be mistaken for a systolic murmur, especially if the duct is large and there is associated pulmonary hypertension.
- Radiates along the pulmonary arteries, and often well heard over the left side of the back
- With a large duct, the large left-to-right shunt causes left heart dilation
- Can cause symptoms such as failure to thrive, dyspnea and recurrent chest infections.
- Bounding pulses
- Low diastolic BP
- Apex may be displaced and forceful, and an apical mid-diastolic murmur may be heard due to increased flow through the mitral valve
- A thrill may be palpable
What is the management of a patent ductus arteriosus in preterm infants?
IV indomethacin
IV ibuprofen can be used in infants of larger weight
What is the management of PDA in term infants?
Percutaneous catheter closure
What is Eisenmenger syndrome?
Intracardiac communication with severe pulmonary vascular disease which is inoperable
What is the most common congenital heart defect?
Ventricular septal defect
What are the clinic presentation of VSDs?
- Small VSDs with little shunt are often asymptomatic but have a loud murmur
- Loud, harsh, high pitched pansystolic murmur usually heard best at the lower left sternal border
- There may be a thrill
- Large VSDs - pulmonary overcirculation and heart failure
- Increase flow across the mitral valve causing a mid-diastolic murmur
- Parasternal heave
- Difficulty feeding due to tachypnea
- Hepatomegaly
- Splitting of S2 and intensity of P2 – depending on the pulmonary artery pressure
Eisenmenger syndrome
- At rest patients may be asymptomatic, but experience exertional dyspnea, cyanosis, chest pain, syncope and hemoptysis with exercise
What are the four structural defects of Tertalogy of Fallot?
Ventricular septal defect
Pulmonary stenosis
Overriding aorta
Right ventricular hypertrophy
At what age would you expect to see cyanosis in children with tertaology of fallot?
6 to 12 months
How do you treat a hypercynanotic spell or tet spell?
Treatment of hypercyanotic spells
Basics
ABC, oxygen, sedation and pain relief (morphine)
Place and person
Hospital, ICU
Investigate and confirm diagnosis
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Non-invasive management
Keep the child calm – stress will exacerbate
Definitive management
IV propranolol
- Works as a peripheral vasoconstrictor and by relieving the subpulmonary muscular obstruction that is the cause of reduced pulmonary blood flow
IV volume administration
Bicarbonate to correct acidosis
Muslce paralysis and artificial venticlation in order to reduce metabolic oxygen demand
At what age does transposition of the great arteries usually become apparent?
- Presentation is usually on day 2 of life when ductal closure leads to a marked reduction in mixing of the desaturated and saturated blood.
Physical signs
- Cyanosis is always present
- Quiet tachypnea
- The second heart sound is often loud and single
- Usually no murmur, but may be a systolic murmur from increased flow or stenosis within the left ventricular outflow tract
- Metabolic acidosis may develop because of tissue hypoxia.
What are the signs of heart failure in a child?
Recurrent chest infections
FTT
Difficulty feeding
Hepatomegaly
Tachycardia
Respiratory distress
Profuse sweating when feeding
What is Eisenmenger’s Syndrome?
Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease.
