Congenital Heart Disease Flashcards
CHD accounts for what % of congenital anomalies?
30%
What are the big 8 that account for most of cardiac anomalies?
VSD, ASD, PDA, pulmonary and aortic stenosis, coarctaton of aorta, transposition of great arteries, tetralogy of fallot
What is the aetiology of CHD?
Genetic predisposition and environmental hazards
What environmental hazards contribute to CHD?
Drugs (alcohol, cocaine, amphetamines, ectasy, phenytoin, lithium)
Infection - ToRCH
Maternal DM, SLE
When is the teratogenic window for cardiac anomalies?
18-60 days post conception
What does maternal DM put the child at increased risk of?
Hypertrophic cardiomyopathy which resolves after birth
What does maternal SLE put the child at risk of?
Ab attach bundle of his –> heart block
What is trisomy 13 assoc with?
VSD and ASD
What is trisomy 18 assoc with?
VSD and PDA
What is trisomy 21 assoc with?
ASVD
What is turner’s syndrome assoc with?
Coarctation of aorta and biscupid aortic valve
What is Noonan syndrome assoc with?
Pulmonary stenosis
What are the symptoms of DiGeorge syndrome?
CATCH 22
Cardiac abnormalities, anomalous face, thymus aplasia, cleft palate, hypocalcaemia due to hypoparathyroidism
22nd chromosome (22q11)
What are the key areas of history you want to cover in suspected heart problems in a child?
Feeding, wt, development Cyanosis Dyspnoea/tacypnoea Exercise tolerance Chest pain - often mistaken for GO reflux, costochondritis Syncope Palpitations Joint problems - rheumatic fever
What is rheumatic fever?
Inflammatory sequela involving heart, joints, skin, CNS after untreated GAS infection
What should you check for in examination of a child you suspect to have cardiac problems?
Weight, ehight Dysmorphic features Cyanosis Clubbing Tachy/dyspnoea Pulses/apex - esp femoral Heart sounds Murmurs
What investigations may be useful in identifying a cardiac condition?
BP O2 sats, ABG ECG CXR Echo Catheter to measure intracardial pressures/sats Angiography MRI/A Exercise testing
What % of murmurs are innocent?
70-80%
What are consistent features of innocent murmurs?
Systolic murmur/continuous in venous hum No signs of cardiac dx Soft murmur (grade 1/2 of 6) Vibratory/musical Localised Varies w. position/exercise/respiration
What is the most common innocent murmur?
Still
What is a typical presentation of still murmur?
Age 2-7
Soft, systolic vibratory, twangy murmur at apex & L sternal border
Increases when supine/exercising
What is a typical presentation of pulmonary outflow murmur?
Age 8-10
Soft systolic, vibtatory murmur at upper L sternal border, well localised, not radiating to back and worse in supine position & with exercise
Often in children with narrow chest
What is a typical presentation of carotid/brachiocephalic arterial bruit?
Age 2-10y
1-2/6 systolic, harsh
Supraclavicular, radiating to neck
Worse with exercise, decreased on turning head/extending neck
What is a typical presentation of someone with venous hum?
Age 3-8y
Soft, continuous murmur, sometimes diastolic accentuation
Supraclavicular
ONLY in upright position
Why are innocent murmurs more common in children?
As heart closer to chest wall
Heart smaller so low more turbulent and walls thinner
What are the three types of VSD?
Subaortic, perimembranous, muscular
What sort of shunt do you mostly get in VSD?
L to R
What sort of murmur do you get in VSD?
Pansystolic at lower left sternal edge
Sometimes thrill
If the hole in VSD is small what signs/symptoms will you see?
Asymptomatic + early systolic murmur
In large VSD what murmur will you get?
Diastolic murmur due to relative mitral stenosis
How can VSD lead to R –> L shunt, why is this a problem and what is the name of this syndrome?
RV doesn’t fill properly, jet goes up pulmonary artery –> extra fluid in pulmonary arteries –> irritation of pulmonary arteries –> fibroblast + smooth muscle deposition in pulmonary artery –> RV hypertrophy –> RV pressure increases –> more pulmonary HTN and switch to R to L shunt
–> cyanosis
Known as Eisenmenger syndrome
What signs do you get in large VSD?
Cardiac failure: tachypnoea, tachycardia, hepatosplenomegaly
Stopping feeding as too out of breath
How do you treat VSD?
Amplatzer (closure) device Patch closure (CP bypass w. autologous pericardium)
How do ASDs tend to present?
Asymptomatic normally & spontaneously close
Can present in adulthood with AF, heart failure + pulmonary HTN
What murmur do you get in ASD?
Wide, fixed splitting of 2nd heart sound, pulmonary flow murmur
How do you treat ASD?
Occlusion device if big
What is assoc with trisomy 21?
Single AV valve, ostium primum ASD and high VSD
How does pulmonary stenosis tend to present?
Mild - asymptomatic
Mod/severe - severe, exertional dyspnoea, fatigue
What murmur do you get with pulmonary stenosis?
Ejection, systolic murmur upper left sternal border w. radiation to back
Do you treat pulmonary stenosis? If so, how?
Monitor as when heart grows stenosis narrower relative to heart
Rx with balloon valvoplasty (leads to regurg but kids tolerate this well)
How does aortic stenosis present?
Usually asymptomatic
If severe –> reduced exercise tolerance, exertional chest pain, syncope
If coronary circulation impaired –> coronary heart disease symptoms
What sort of murmur do you get in aortic stenosis?
Ejection, systolic murmur upper right sternal border, radiation to carotids
What shunts are present in the foetal circulation?
Foramen ovale - RA, LA
Ductus arteriosus - pulmonary trunk and aorta
Ductus venosus - umbilical vein and vena cava (bypassing liver)
What changes occur to the foetal circulation at birth?
Pulmonary vascular resistance falls, pulmonary BF rises, systemic vascular resistance increases
3 shunts close
In which group are PDA most common?
Prems
How do you treat PDA?
Fluid restriction, diuretics, prostaglandin inhibitors (ibrupofen, indomethacin), surgical ligation, closure umbrella device
What is coarctation of the aorta?
Kink in aorta (usually below DA in descending aorta)
How do you test for coarctation of aorta?
Absent femoral pulses/radial-femoral delay
Be aware this may not be reliable in first few weeks as DA compensates until it closes
What must you think of in 2-3wk old child, acutely unwell and in shock?
Check femoral pulses for coarctation of aorta
How do you treat coarctation of aorta?
Re-open ductus arteriosus with prostaglandin E1/2, resection with end to end anastomosis, subclavian patch repair, balloon arthroplasty
What is transposition of the great arteries?
Cyanotic heart condition in which the aorta is coming out of the LV and pulmonary trunk is coming out of the RV
What is the treatment for transposition of the great arteries?
If know about it antenatally deliver and give prostaglandins to keep shunts open Switch procedure (be aware of damaging coronary arteries --> MI)
What four things are assoc with fallot tetralogy?
Pulmonary stenosis
RV hypertrophy
High VSD
Overriding aorta
How does fallot tetralogy lead to cyanosis?
Less O2 going to lungs –> RV hypetrophy and inc. pressure in RV –> bigger R to L shunt
