Paediatric Cardiology Flashcards
Congenital heart diseases aetiology
Name 3 environmental causes giving at least 2 eg of each
Teratogenic insult during which period post-conception has a high risk of cardiac developmental abnormality?
Genetic causes Environmental - Drugs - alcohol, illicit drug use, phenytoin, lithium - Infections - TORCH - Maternal - DM, SLE Teratogenic insult at 18-60 days
Chromosomal abnormalities associated with congenital heart diseases:
Trisomy 13 Patau is associated with… [2]
Trisomy 18 Edwards [2]
Trisomy 21 Down [1]
Trisomy 13 - VSD, ASD
Trisomy 18 - VSD, PDA
Trisomy 21 - AVSD
Other genetic syndromes causing congenital heart disease: (for each syndrome, state the associated heart defect) 22q11 deletion syndrome Turner syndrome Noonan syndrome Williams syndrome
22q11 deletion syndrome - general congenital heart disease
Turner syndrome - co-arctation of aorta
Noonan syndrome - pulmonary stenosis
Williams syndrome - supravalvular aortic stenosis
Which cardiac defects present with cyanosis? [5]
- Transposition of the great vessels
- Pulm Atresia
- Truncus Arteriosus
- TAPVD
- Tetralogy of Fallot
Innocent murmurs are pediatric murmurs unrelated to any pathology - 4 examples
1) Still’s (LV outflow) murmur
2) Pulm Outflow Murmur
3) Carotid/brachiocephalic bruit
4) Venous Hum
What features would suggest an innocent murmur? [6]
What would you hear in pulmonary area vs aortic area?
1) Soft, localized and grade 1 or 2/6
2) Vibratory, musical, soft
3) No signs of cardiac disease
4) Vary on position, exertion or respiration
5) Localised with no radiation
6) All ejection murmurs, no diastolic component
Soft blowing in pulmonary area
Short buzzing in aortic area
Still's murmur is aka [1] Age at presentation Quality [3] Position [2] Factors that increase audibility of murmur [2]
LV outflow murmur 2-7 years presentation Soft systolic, vibratory and musical Apex, left sternal border Increases in supine position, with exercise
Pulmonary outflow murmur Age at presentation Quality [3] Position [2] Factors that increase audibility of murmur [2]
8-10 years
Soft systolic, vibratory
Upper left sternal border, well localized
Increases in supine position, with exercise
Carotid/brachiocephalic arterial bruits Age at presentation Quality [3] Grading [2] Factors that increase audibility of murmur [2]
2-10 years
Harsh systolic
1 or 2/6
Position: supraclavicular, radiates to neck
Only heard in upright position, disappears on lying down or turning head
Venous hum Age at presentation Quality [4] Position [1] Factors that increase audibility of murmur [2]
3-8 years Soft, indistinct, continuous Sometimes with diastolic accentuation Supraclavicular only heard in upright position, disappears on lying down or turning head
What are 3 the types of VSD?
Mx: what’s first line?
What 2 indications are for second line treatment?
Subaortic
Intramembranous
Muscular
Amplatzer device
Patch closure indicated in big holes or position difficult to assess via catheter
VSD presentation
Quality [2]
Location [1]
In small VSD, describe the murmur you would expect [1]
In large VSD, describe the murmur you would expect [3]
Eventual outcome of large VSD [2]
Pansystolic murmur, ~ thrill
Lower left sternal edge
In small VSDs, early systolic murmur
In large VSDs, diastolic rumble due to relative mitral stenosis + signs of cardiac failure
Eisenmengers syndrome: Eventually progressing to biventricular hypertrophy and pulmonary hypertension
Pediatric signs of cardiac failure [3]
Tachypnea (pulm congestion) and tachycardia
Hepatomegaly
Early satiety or increased work of feeding
In a VSD what is the pathological shunt [1]
What is special about the pathogenesis or course of VSD? [3 main steps]
Hole in inter ventricular septum causes L to R shunt
Eisenmenger’s syndrome causes pulmonary hypertension and eventual reversal of shunt into cyanotic R > L shunt
Pulmonary artery becomes fibroses
Pulmonary vascular resistance increases - pulmonary hypertension
RV pressure > LV pressure
Cyanotic
ASD
Few clinical symptoms in early childhood [2]
Prognosis/outcome [1]
Presentation in adulthood [3]
Describe the murmur and what kind is it? [2]
Mx: [2]
Good chance of spontaneous closure
Presentation in childhood: Recurrent chest infections/wheeze, arrhythmias
Presentation in adulthood: atrial fib, HF, pulmonary htn
Wide fixed splitting of 2nd heart sound, pulmonary outflow murmur
ASD closure: catheter procedure, surgery
AVSD - two other names
Associated with which genetic abnormality
Whats the problem? [3]
Mx [2] and its rationale
aka endocardial cushion defect or AV canal defect
Associated with trisomy 21
Singular AV valve with ostium primium/low ASD and high VSD
Medical mx with diuretics and ACEi to reduce sx of CHF allowing child to grow older before undergoing operative repair
Pulmonary stenosis Mild stenosis presentation vs mod-severe stenosis [2] Describe its murmur [3] Mx and rationale [2] What are the cons of this management
Mod-severe pulmonary stenosis - exertion dyspnea and fatigue
Ejection Systolic Murmur on upper L SB + radiates to back
Mx: balloon valvoplasty as temporary holding measure until past puberty as child will outgrow as growing
Cons: pulmonary regurg
Aortic stenosis
Presentation in mild-mod [1]
Presentation in severe stenosis [3] - explain why
Describe its murmur [3] and a pathognomic examination finding
Tx [1]
Severe aortic stenosis - reduced ex tol, exertional CP, syncope due to coronary supply impairment
Ejection Systolic Murmur on upper L SB + radiates to carotids
Suprasternal thrill pathognomic findings
Same treatment as pulmonary stenosis and rationale - balloon valvoplasty
PDA
Risk factor [1]
Treatment: describe medical mx [2], describe 2 surgical options
Common in pre-term infants
Fluid restriction/diuretics
PG inhibitors - indomethacin, ibuprofen
Surgical ligation or closure with umbrella device
Coarctation of aorta Examination finding [1] 2 other investigations Surgical mx [3] What other option for management is there?
Femoral Pulses will be reduced or absent and may be femoral-radial delay
USS and MRI can be done
Surgical resection with end-to-end anastomosis
Re-open PDA with PGE1, E2
Subclavian patch repair
OR balloon valvoplasty to buy time before major surgery
Cyanotic heart conditions [2]
Tetralogy of fallot
Transposition of great vessels
Transposition of great vessels
Whats the main problem?
Mx: medical [1] and surgical [2]
Deoxygenated and oxygenated blood don’t mix, 2 separate circuits
Medical: large infusion of PG to keep DA patent
Switch procedure - switch both vessels above coronary artery level then transplant coronary arteries
Tetralogy of fallot
Whats the main problem
4 characteristics of the tetralogy
What can be seen on CXR?
Pulmonary outflow tract obstruction
Pulm Stenosis Overriding Aorta VSD RVH Boot shaped heart
Describe what a tet spell is [3]
Tet spells: sudden development of signs of cyanosis [1] caused by a rapid drop in the amount of oxygen in the blood [1]
Squatting increases peripheral vascular resistance (PVR) and thus decreases the magnitude of the right-to-left shunt across the ventricular septal defect (VSD). [1]
What are the signs of the tetralogy of Fallot? [5]
Pulmonary Stenosis Murmur Cyanosis Clubbing RV heave Heart failure symptoms Tet spells
TOF IX [4]
CXR - Boot shaped heart
Echo - Degree of Stenosis
CT/MRI - Helps you plan surgery
FBC - Polycythaemia
What treatments can be given for tetralogy of fallot?
Acute treatment of hyper cyanotic spell [6]
Acute treatment:
- O2
- Artificial ventilation to reduce metabolic demand, muscle paralysis
- B: IV propranolol (reduce strain on RV, peripheral vasoconstrictor) and IV fluids
- Bicarbonate to correct acidosis
- Sedation, pain relief - morphine
When is surgery indicated for TOF?
Surgery [2]
At 6 months:
Surgery to close the VSD and correct the pulmonary stenosis
Blalock Taussig shunt to increase pulmonary blood flow
Ebsteins anomaly
Ax
Pathophysiology
Ax: maternal lithium in pregnancy
Px:
- displacement of posterior tricuspid leaflets anteriorly
- causing tricuspid stenosis and tricuspid regurgitation and right atrial enlargement
Sy/Si: pan systolic murmur (tricuspid regurgitation) and diastolic murmur (tricuspid stenosis)
Ebsteins anomaly
Ix [3]
Mx
Associations [2]
Ix: CXR (cardiomegaly with prominent right atrium), ECG, echo
Mx: cone reconstruction
Associations:
- tricuspid incompetence (pan-systolic murmur, giant V waves in JVP)
- Wolff-Parkinson White syndrome
