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Paediatrics RACP Exam Study > Paediatric Cardiology 3 > Flashcards

Paediatric Cardiology 3 Flashcards

1
Q

What syndromes are associated with coarctation of the aorta?

A

Turner syndrome

Shone complex

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2
Q

P wave criteria for atrial hypertrophy:

A

RAH >3mm high

LAH >0.1s long (2.5 squares) or >0.08 in infants, bifid P wave

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3
Q

What is the normal BP difference between arms + legs?

What about in coarctation?

A

Legs - 10-20mmHg higher due to arterial pressure waves and stretch reflex.
Neonates - same in arms and legs
Coarctation - arms > legs

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4
Q

What % of people with coarctation have bicuspid AV?

A

70%

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5
Q

ECG signs of LVH:

A
  • LAD for the patient’s age
  • QRS voltages in favour of the LV (in the presence of a normal QRS duration): R in I, II, III, aVL, AVF, V5 or V6 > ULN for age; S in V1 or V2 > ULN for age
  • Abnormal R/S ratio in favour of the LV; R/S ratio in V1 and V2 < LLN for age
  • Q in V5 and V6, 5 mm or more, plus tall symmetric T waves in the same leads (“LV diastolic overload”)
  • In the presence of LVH, a wide QRS-T angle with the T axis outside the normal range indicates “strain” pattern; this is shown by inverted T waves in I or AVF.
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6
Q

What does PHACE stand for in PHACE syndrome?

A 
P - posterior brain fossa malformations
H - haemangiomas
A - arterial anomalies
C - cardiac, coarctation of the aorta
E - eye and endocrine
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7
Q

What would be complications expected after RVOT obstruction repair?

A

RBBB if RV opened

Pulmonary regurgitation e.g. with valvotomy

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8
Q

What findings do you expect with pulmonary regurgitation?

A
  • Majority is well tolerated and therefore asymptomatic
  • Decrescendo, low pitched diastolic murmur at the ULSE/mid LSE upper and midleft sternal border
  • CXR - Enlarged MPA and possibly RV enlargement
  • ECG - normal or mild RVH
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9
Q

Describe the murmur of mitral regurgitation:

A

Mild - high-pitched, apical holosystolic murmur

Moderate to severe - low-pitched, apical mid-diastolic rumble

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10
Q

What syndromes and congenital anomalies are associated with MV prolapse?

A
  • Fragile X
  • Marfan
  • Ehlers-Danlos
  • Straight back syndrome
  • Pseudoxanthoma elasticum
  • Osteogenesis imperfecta
  • Scoliosis

Being female!
May be inherited - autosomal dominant

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11
Q

When are tet spells most commonly seen in Tetralogy of Fallot (and what do you do about them)?

A

First 2 years of life, after crying and on waking
Reduction in pulmonary blood flow
Knees-to-chest, oxygen, morphine

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12
Q

Name cardiac lesions associated with Tetralogy of Fallot and approximate percentages:

A 
R sided arch - 20%
Coronary artery anomalies - 5-10%
Absent pulmonary valve
Absent branch PA (L>R)
PDA
ASD
AVSD esp if Trisomy 21
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13
Q

Describe the presentation and clinical findings in Tetralogy of Fallot in infancy:

A

Infancy/young toddler:

  • Heart failure - Dyspnoea, diaphoresis, poor feeding, may have cyanotic “tet spells”
  • Cyanosis usually in the first year of life unless severe RVOT which may present early in life.

Examination

  • normal pulse
  • substernal RV impulse
  • systolic thrill LSE 3rd and 4th spaces
  • harsh ejection systolic murmur LSE (mixed RVOT/VSD), radiates to lungs, can get quieter with tet spell
  • Single S2
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14
Q

What are the ECG findings in Tetralogy of Fallot?

A
  • Right axis deviation
  • RVH - may have dominant R wave in chest leads, positive T wave in V1
  • Tall peaked P wave for RA enlargement
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15
Q

Complications of late repair/diagnosis of Tetralogy of Fallot:

A
  • Cerebral thromboses due to extreme polycythemia and dehydration (<2 years)
  • Brain abscess
  • Endocarditis
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16
Q

Normal value for PA pressure?

A

15mmHg

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17
Q

Normal value for atrial pressures?

A

<10mmHg

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18
Q

Formula for Qp/Qs:

A

(Aortic sats - mixed venous sats) / (pulmonary vein sats -pulmonary artery sats)

19
Q

When do you fix VSD?

A
  • Heart failure

- Qp>Qs is 2 or higher

20
Q

Formula for pulmonary vascular resistance:

What are normal values?

A

PVR in units x m^2 = (mean PA pressure - left atrial pressure) / (pulmonary blood flow in L/min/m^2)

Normal: 1-2
Mild: 2-4
Moderate: 4-6
Severe: >8

Previously correlated with biopsies done for PVOD

21
Q

Which cardiac conditions are associated with neurofibromatosis?

A
  • Pulmonary Stenosis
22
Q

ECG findings in antidromic SVT?

A
  • Rate usually 200 – 300 bpm.
  • Wide QRS
  • anterograde conduction via accessory pathway, retrograde conduction via AV node.
  • ~5% of patients with WPW
    DO NOT GIVE DIGOXIN
23
Q

ECG findings in orthodromic SVT?

A
  • Rate 200-300bpm
  • P in QRS or retrograde
  • Narrow QRS unless BBB/aberrant conduction
  • anterograde conduction via AV node, retrograde conduction via accessory pathway
  • QRS Alternans – phasic variation in QRS amplitude associated with AVNRT and AVRT, normal QRS amplitude
  • TWI, ST depression
24
Q

How does iloprost work?

A
  • Synthetic analogue of prostacyclin PGI2.
25
Q

What is the abnormality in the cardiac action potential which results in QT prolongation in LQT1, LQT2 and LQT5?

A
  • Prolonged potassium efflux
  • QT 1-3 is 97% of all cases
  • All but QT3 are potassium channel issues
26
Q

Which cardiac condition is most commonly associated with neurofibromatosis?

A

PS

27
Q

Describe the presentation of untreated ToF in childhood:

A

Tet squat/dyspnoea with exertion, dusky blue skin, gray sclerae with engorged blood vessels, and marked clubbing of fingers and toes.

Examination

  • normal pulse
  • substernal RV impulse
  • systolic thrill LSE 3rd and 4th spaces
  • harsh ejection systolic murmur LSE (mixed RVOT/VSD), radiates to lungs, can get quieter with tet spell
  • Single S2
28
Q

What are the causes of complete heart block in children?

A
  • Most common is surgery for congenital heart disease - usually well, post-op
  • drugs - digitalis/digoxin
  • infective - viral myocarditis, Lyme, EBV
  • post-infective - ARF
  • neonatal lupus
  • sarcoidosis
29
Q

What type of immune reaction is acute rheumatic fever?

A

Type II

30
Q

How does simvastatin work?

A

Upregulation of low density lipoprotein (LDL) receptor activity

31
Q

‘Egg on a string’ heart on CXR suggests:

A

TGA

32
Q

‘Snowman sign’ on CXR suggests:

A

Supracardiac/Type 1 TAPVR

33
Q

What are the first 3 normal aortic branches?

A

R brachiocephalic, L common carotid, L subclavian

34
Q

Which type of AVRT gives you WIDE complex tachycardia?

A

Antidromic atrio-ventricular re-entrant tachycardia

35
Q

Which neonatal congenital heart lesion presents with LVH?

A

Tricuspid atresia (although other signs must be in keeping!!)

36
Q

Which congenital heart lesions present with LAD?

A
  • Tricuspid atresia

- Endocardial cushion defects (both complete atrioventricular [AV] canal and primum ASD) b

37
Q

What congenital anomalies are associated with WPW?

A
  • TGA and Ebstein’s
38
Q

Describe examination and investigation findings of a large VSD:

A

Exam - Prominent left precordium is common, palpable parasternal lift, displaced apex+apical thrust, thrill. May be quieter murmur. Louder P2 due to pulmonary hypertension. Mitral rumble - mid-diastolic, low-pitched rumble at the apex with increased blood flow across the MV
Ix - ECG shows biVH, notched/peaked P wave. CXR frank cardiomegaly + failure with oedema or effusion. Qp : Qs ratio of ≥2 : 1.

39
Q

Describe typical clinical presentation of large VSD:

A

Dyspnoea, feeding difficulties, poor growth, profuse diaphoresis, recurrent pulmonary infections, and cardiac failure in early infancy (2-6 weeks). May get cyanosis with crying/infections or shunt reversal.

40
Q

What is the most common cardiac anomaly in Costello syndrome?

A

Valvular PS

41
Q

What is the most common cardiac anomaly in Alagille?

A

ToF

42
Q

What are the triggers for the different types of long QT?

A
  • 1: 5-15yo male, swimming/emotion
  • 2: Female, post partum, loud noise/surprise e.g. alarms
  • 3: Young men who die in their sleep, emotion
43
Q

How do you calculate CO when you are given sats and VO2?

A

Ca-Cv in ml/L so you should get O2 in mL/L
Multiply same by sats to get difference

CO = VO2/(Ca-Cv)

44
Q

What’s actually absent in PCD/Kartagener?

A

Absent dynein arms
Associated with dextrocardia
Look for Nasal Nitric Oxide - LOW in PCD

Paediatrics RACP Exam Study (30 decks)

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