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
