W8 - Cardiovascular disease in Childhood Flashcards
what are the 2 types of congenital cardiac heart disease
acyanotic
cyanotic
what type of congenital cardiac disisease is most common
acyanotic (85%)
what are some examples of acyanotic congenital heart defects that cause increased pulmonary flow
ASD, VSD, AVSD, PDA
what are some examples of acyanotic congenital heart defects that cause obstruction
Pulmonary stenosis, Aortic stenosis, Coarctation
what are some examples of cyanotic congenital heart defects
Tetralogy of Fallot, Tricuspid Atresia, Transposition of GA, TAPVD, Truncus, HLHS
what are some symptoms of congenital heart disease
Symptoms of heart failure
Cyanosis, SaO2 <96%
Abnormal pulses
Hyperactive precordium
Abnormal heart sounds
Murmur ≥3/6
harsh diastolic, pansystolic, or continuous
ejection click
how does neonatal heart disease tend to present
Death
Cyanosis
Heart failure
Abnormal Neonatal Examination
- Murmur and/or Pulses
what are some signs and symptoms of heart failure
Poor feeding
Tachypnoea, respiratory distress
Sweating, clammy skin, poor perfusion
Pallor, cyanosis
Tachycardia, hyperactive praecordium
Gallop rhythm
Oedema, hepatomegaly
what is the most common acyanotic lesion
Ventricular septal defect
what are the next most common acyanotic lesions (all 10%)
Patent Arterial Duct
Pulmonary stenosis
Coarctation of the aorta
Atrial Septal Defect
Aortic Stenosis
what conditions have the pathophysiology of a volume overload
ASD
VSD
AVSD
Patent Ductus Arteriosus
what conditions have the pathophysiology of a pressure overload
Aortic stenosis
Pulmonary stenosis
Coarctation
describe foetal circulation
go through
will be in OSCE and EoY
what is the management of ventricular septal defect
nothing - can close on its own if not too big and child is asymptomatic
may need surgery if too big or symptomatic
what is the pathophysiology of ventricular septal defect
↑ blood flow through LV
LV enlargement
↑ pulm blood flow
High pulmonary blood flow causes pulmonary hypertension
what is the presentation of atrial septal defect
Usually asymptomatic, though may have symptoms of high pulmonary blood flow
what is the management of atrial septal defect
surgery or devise closure
what is the pathophysiology of atrial septal defect
Left to right Shunt
↑ blood flow across tricuspid valve -> MDM
↑ blood flow across pulmonary valve -> ESM and wide, fixed splitting S2
↑ flow through RV -> RV enlargement
in what condition is Complete Atrio-Ventricular Septal Defect ( AVSD) most common
downs syndrome
when does AVSD need to be repaired by
before 6 months of age
what is the pathophysiology of complete AVSD
Large left to right shunt -> early development of heart failure
RV pressure never falls so murmur is ejection systolic across pulmonary valve
what is aortic coarctation
Narrowing of aorta causing reduced flow to systemic circulation
what is the characteristic featuer of coarctation of the aorta
ABSENT FEMORAL PULSES
what are some symptoms and signs of coarctation of the aorta
Poor feeding & weight gain, SOB
Hypertensive in arm
ABSENT FEMORAL PULSES
what type of murmur is heard on coarctation of the aorta
Ejection systolic murmur radiates to back
what is the treatment for coarctation of the aorta
PGE1 infusion to reopen ducts, anti-failure Rx, inotropes, diuretics.
Needs urgent surgical resection & end to end anastomosis
what is the pathophysiology of coarctation of the aorta
Neonatal collapse – circulatory collapse, resp distress, severe acidemia
↑ proximal pressure -> may affect heart and brain
↓ distal pressure -> poor renal flow compounds hypertension
which gender is more likely to have Aortic valve stenosis
M:F = 3:1
what symptoms do you present with in Aortic valve stenosis
normally asymptomatic
Can present in cardiac failure +/-collapse, weak pulses, pale cool skin – severe/critical AS in newborn
Older child
angina, syncope, exertional dyspnoea/chest pain
Mild stenosis worsens with time in sig number of pts
what heart sounds can you hear in Aortic valve stenosis
Harsh ejection systolic murmur
Ejection click with valvular AS
Systolic thrill suprasternal notch or over carotid arteries
what is the treatment of Aortic valve stenosis
Balloon valvuloplasty or surgery
what kind of murmur can be heard in Pulmonary valve stenosis
Ejection systolic murmur radiating to axilla
what signs and symptoms do people with Pulmonary valve stenosis present with
Often asymptomatic
Severity can improve or worsen
May present in heart failure/cyanosis
what is the treatment for Pulmonary valve stenosis
Balloon valvuloplasty or surgery
how would tetrology of fallot present
Cyanotic
failing to thrive
Soft ejection or pan systolic murmur
low O2 sats
what are the 4 feature of tetralogy of fallot
Pulmonary Stenosis
Overriding aorta
Ventricular Septal Defect
Right Ventricular Hypertrophy
what would you hear on heart sounds for Tetralogy of Fallot
Harsh ejection systolic murmur and right ventricular heave
what would you see on Chest XR for tetrallogy of fallot
Boot shaped heart, decreased pulm markings
what are Tetralogy of Fallot “Hypercyanotic spells”
Muscle spasm below pulmonary valve leads to marked reduction in pulmonary blood flow
Cry/Rapid & deep breathing, irritability -> pallor -> cyanosed+++ -> arrest
Spells occurs after crying, feeding – peaks 2-4months
what is the treatment for “Hypercyanotic spells”
Educate
Knees to chest or over your shoulder
Supplemental O2 + Morphine 0.2 mg/kg SC/IM to suppress the resp centre and abolish hyperpnea
Acidosis treatment with sodium bicarbonate
oral Propranolol pending surgery
what are the 3 types of syndromes with cardiac disease
Aneuploidies
Single gene
Non-genetic syndromes
what type of syndrome is downs syndrome
aneuploides
what are the feature of downs syndrome
Mental retardation / Learning difficulties
Developmental Delay
Floppiness
GI anomalies
Conductive deafness
Duodenal atresia, Hirschsprung,
Leukaemia,
what are the types of cardiac abnormality associated with downs syndrome
VSD, AVSD, ASD
Mainly AVSD
with is patau’s syndrome
trisomy 13
how does pataus syndrome present
extra fingers and toes
cleft lip
what is edwards syndrome
trisomy 18
what is the mortality of edwards syndrome
90-95% mortality by 1 year
what cardiac defects are associated with edwards syndrome
VSD
ASD
polyvalvar anomalies
what cardiac conditions are associated with turners syndrome
Valvar aortic stenosis 15%
Coarctation of the Aorta 5-10%
how does tuners syndrome present
Lymphoedema in infants
Short stature in children
Primary amenorrhoea
Webbed neck, low hairline, low ears
Dystrophic nails
Renal (horseshoe, arterial and ureteral abnormalities)
what cardiac defect is associated with noonans (single gene defect)
Pumonary valve stenosis
what cardiac defect is associated with williams (single gene defect)
Supra valvar aortic stenosis
what cardiac defects are associated with marfans
AR dilatation, dissection
what cardiac defects are associated with di georges
VSD, ASD, Fallot’s Tetralogy
what are the features of noons syndrome
Pulmonary stenosis, Hypertrophic cardiomyopathy
what are the features of williams syndrome
Pulmonary artery branch stenosis
Supra valvar aortic stenosis
what are the features of marfans syndrome
MV prolapse
Aortic root problems
what are the features of di georges syndrome
Fallot’s tetralogy,
Truncus arteriosus,
Arch abnormalities
what is charge association
Coloboma
Heart defect
Atresia (Choanal)
Retardation (mental)
Genital
Ear
autosomal dominant
what is Vacteral association
Vertebral defects,
Anal atresia,
Cardiac abnormalities
Tracheo –
Esophageal fistula
Renal
Limb abnormalities
VSD, PDA, Tetralogy TGA all reported
what heart defects are associated with foetal alcohol syndrome
VSD or ASD
what are some features of foetal alcohol syndrome
Microcephaly, small maxilla, up-turned nose smooth philtrum and upper lip, small eyes, prominent epicanthic folds
how do you treat a ‘blue baby’
O2 sats
CXR
ABG
Hyperoxia test
ECG
urgent ECHO
what 2 conditions can cause a heart disease
Kawasaki disease
Rheumatic Heart disease
Which of the following is the approximate percentage of paediatric chest pain that is cardiac in origin?
80%
50%
20%
1%
<0.01%
1
Which of the following factors increases the likelihood that chest pain is cardiac in origin?
A. Family history of acute MI at 55 years of age
B. Past history of repaired Tetralogy of Fallot
C. Syncopal episode while swimming
D. Past medical history of asthma
E. ‘Crushing’ chest pain exacerbated by exercise
B
C
E
whats this
whats this
what are some red flags for cardiac disease
Exertional chest pain
Exertional syncope
palpitations
FHx - familial hypercholesterolemia
genetic disorders (Marfan’s)
Kawasaki disease
