Paeds Cardiology Flashcards

1
Q

what is the difference between acyanotic and cyanotic heart disease?

A

acyanotic heart disease: the blood contains enough oxygen but is pumped abnormally around the body

cyanotic: there is not enough oxygen present in the blood

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2
Q

name the type of acyanotic heart diseases?

A

ASD
VSD
aortic stenosis
pulmonary stenosis
mitral stenosis
PDA (patent ductus arteriosus)
coarction of aorta

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3
Q

name the type of cyanotic heart diseases

A

the five ‘t’s’

  • tetralogy of fallot
  • transposition of the great arteries
  • total anomalous pulmonary venous return (TAPVR)
  • truncus arteriosus
  • tricuspid valve abnormalities
    (- univentricular heart)
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4
Q

what is patent ductus arteriosus?

A

when the ductus arteriosus (shunt from aorta to pulmonary artery) doesnt close

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5
Q

what is the pathophysiology of a patent ductus arteriosus?

A
  • blood flows into pulmonary vessesl due to increased pressure in teh aorta
  • increases the pressure in the pulmonary vessels = pulmonary HTN
  • this leads to right heart strain and right ventricular hypertrophy
  • increased blood in pulmonary vessels and returnign to left side of heart = left ventricular hypertrophy
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6
Q

how does a patient with patent ductus arteriosus present?

A
  • continuous crescendo-decrescendo ‘machinery’ murmur
  • SOB
  • difficulty feeding
  • poor weight gain
  • lower resp tract infections
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7
Q

how is patent ductus arteriosus diagnosed?

A

confirmed by echo (+/- doppler flow studies)

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8
Q

management of patent ductus arteriosus?

A

monitored until 1yo with echos (as can close spontaneously up to this age)
after this: transcatheter or surgical closure needed

**those who are symptomatic or have heart failure needed earlier

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9
Q

what are the types of atrial septal defects?

A

ostium secondum
patent foramen ovale
ostium primum

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10
Q

what are teh complications of atrial septal defects?

A

stroke in pts with DVTs
atrial fibrillation or atrial flutter
pulmonary HTN and right sided heart failure
eisenmenger syndrome

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11
Q

presentation of a pt with atrial septal defect?

A

mid systolic, crescendo-decrescendo murmur (loudest at upper left sternal border)
SOB
difficulty feeding
poor weight gain
lower resp tract infections

may present in adulthood with dyspnoea, heart failure or stroke

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12
Q

Mx of atrial septal defect?

A

small - watching and waiting may be appropriate

transvenous catheter closure (via femoral vein) or open heart
anticoags (aspirin, warfarin, NOACs) needs to reduce clots/strokes in adult

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13
Q

what syndromes are ventricular septal defects associated with?

A

downs syndrome and turners syndrome

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14
Q

typical clinical features of ventricualr septal defects?

A
  • poor feeding
  • dyspnoea
  • tachypnoea
  • failure to thrive
  • pan systolic murmur (heard at left lower sternal border in 3rd and 4th intercostal space, possible systolic thrill)
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15
Q

Tx of ventricular septal defect?

A

watched overtime if have no symptoms/pulmonary HTN/heart failure - close spontaneously

surgically -> transvenous catheter closure via femoral vein or open heart surgery
need ABX prophylaxis as high risk of developing infective endocarditis

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16
Q

symptoms of aortic stenosis

A
  • fatigue
  • SOB
  • dizziness
  • fainting
    **all worse on exertion
  • if severe can present as heart fialure witihin months of birth
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17
Q

key signs of aortic stenosis

A
  • ejection systolic murmur (heard loudest in aortic area) + crescendo-decrescendo charcter and radiates to carotids

may be present:
- ejection click before murmur
- palpable thrill during systole
- slow rising pulse and narrow pulse pressure

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18
Q

Ix for aortic stenosis?

A

echo = gold standard

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19
Q

Mx for aortic stenosis?

A

monitoring + guidance to endocarditis + family screening

  • percutaneous balloon aortic valvoplasty
  • surgical aortic valvotomy
  • valve replacement
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20
Q

complications of aortic stenosis?

A
  • left ventricular outflow tract obstruction
  • heart failure
  • ventricular arrhythmia
  • bacterial endocarditis
  • sudden death, often on exertion
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21
Q

symptoms of pulmonary stenosis?

A
  • mostly asymptomatic and picked up with findings of murmur
  • fatigue on exertion
  • SOB
  • dizziness
  • fainting
22
Q

signs of pulmonary stenosis?

A
  • ejection systolic murmur (loudest at pulmonary areA)
  • palpable thrill in pulmonary area
  • right ventricualr heave due to right ventricular hypertrophy
  • raised JVP
23
Q

Ix for pulmonary stenosis

A

gold standard = echo

24
Q

Tx of pulmonary stenosis?

A

watch and waiting if no symptoms

if symptomatic:
- balloon valvuloplasty via venous catheter = gold standard
- open heart surgery

25
Q

what is transposition of the great arteries?

A

attachments of aorta and pulmonary trunk are swapped = two seperate circulations that dont mix = baby cyanosed

26
Q

what is the presentation of transposition of the great arteries ?

A

usually on antenatal US scans
if not detected then baby with present with cyanosis within a few days of birth
if baby has a shunt (e.g. PDA, ASD, VSD) then blood can move between circulations so usually detected a few wks after birth (with resp distress, tachy, poor feeding, poor weight, sweating)

27
Q

Mx of transposition of the great arteries when baby has a ASD or PDA?

A

ASD - balloon septostomy involves inserting catheter into foramen ovale via umbilicus + inflating balloon to create large ASD

PDA - prostaglandin infusion given to maintain ductus arteriosus

28
Q

Mx of transposition of great arteries when no shunt present?

A

open heart surgery = definitive management

29
Q

what is tetralogy of fallot?

A

consists of four pathologies:
- VSD
- overriding aorta
- pulmonary valve stenosis
- right ventricular hypertrophy

30
Q

name some risk factors for tetralogy of fallot?

A
  • rubella infection
  • increased age of mother (>40yo)
  • alcohol consumption in pregnancy
  • diabetic mother
31
Q

Ix to diagnose tetralogy of fallot?

A

echo = gold standard
+ doppler flow studies

xray can show boot shaped heart due to right ventricular thickening

32
Q

how do babies with tetralogy of fallot usually present?

A

most picked up on antenatal scans
ejection systolic murmur cuased by pulmonary stenosis picked up on newborn baby check
if severe, present with heart fialure <1yo
if milder, present with heart failure when older children

33
Q

signs and symptoms of tetralogy of fallot?

A
  • cyanosis
  • clubbing
  • poor feeding
  • poor weight gain
  • ejection systolic murmur (loudest in pulmonarry area)
  • “tet spells”
34
Q

what are tet spells in tetralogy of fallot?

A

intermittent symptomatic periods (cyanotic episode) where right to left shunt becomes temporarily worsened e.g. precipitated by waking, physical exertion or crying
= SOB, irritabilty, cyanotic, reduced consciousness, seizures and potential death

35
Q

Tx for tet spells in tetralogy of fallot?

A

older children: squat
younger children: bring knees to chest

  • supplementary oxygen
  • beta blockers
  • IV fluids
  • morphine
  • sodium bicarbonate
  • phenylephrine infusion
36
Q

Mx of tetralogy of fallot

A

in neonates, need a prostaglandin infusion to maintain ductus arteriosus allowing blood to flow from aorta back to pulmonary arteries
definitive = total surgical repair by open heart surg

37
Q

what congenital heart defects can result in eisenmenger syndrome?

A

atrial septal defect
ventricular defect
patent ductus arteriosus

38
Q

what is eisenmenger syndrome?

A

when there is a left to right shunt present
but over time blood flowing to R side of heart cauases increased pressure in pulmonary vessels = pulmonary HTN
when pulmonary pressure exceeds systemic pressure bloods flows R to L (right to left shunt) = cyanosis + polycythaemia

39
Q

what are the signs of pulmonary HTN?

A
  • right ventricular heave
  • loud P2: loud second heart beat
  • raised JVP
  • peripheral oedema
40
Q

what are the main causes of death in eisenmenger syndrome?

A
  • heart failure
  • infection
  • thromboembolism
  • heamorrhage
41
Q

Mx of eisenmenger syndrome?

A

heart-lung transplant as not reversible once syndrome caused
+
- o2
- sildenafil for pulmonary HTN
- treat arrhythmias
- treat polycythaemia with venesection
- prevention and treatment of thrombosis with anticoags
- prevent infective endocarditis with prophylactic abx

42
Q

what Ix need to be done when a child has a murmur?

A
  • ECG
  • CXR
  • Echo
43
Q

what congenital heart defects would cause pan systolic murmurs?

A
  • mitral regurg (heard fifth intercostal space, mid clav line)
  • tricuspid regurg (heard fifth intercostal space, left sternal border)
  • VSD (heard left lower sternal border)
44
Q

what congenital heart defect woudl cuase an ejection -systolic murmur?

A
  • aortic stenosis (heard 2nd intercsotal space, R sternal border_
  • pulmonary stenosis (heard 2nd intercostal space, L sternal border)
  • hypertrophic obstructive cardiomyopathy (heard 4th intercostal space, L sternal bordeR)
45
Q

what is coarctation of the aorta?

A

narrowing of the aorta usually around the ductus arteriosus

46
Q

pathophysiology of coarctation of the aorta

A

reduces the pressure of the blood flowing to the arteries that are distal to narrowing, increased pressure proximal to narrowing, such as heart and first 3 branches of aorta

47
Q

presentation of coarctation of the aorta

A
  • weak femoral pulses
  • high BP in arteries supplying limb before narrowing
    low BP in arteries supplying limbs after narrowing
  • systolic murmur below left clavicle and left scapula
  • tachypnoea
  • poor feeding
  • grey and floppy baby
48
Q

Tx in coarctation of the aorta?

A

prostaglandin E given initially to keep ductus arteriosus open whilst waiting for surgery (balloon dilatation and stent implantation - transcatheter)

49
Q

initial Mx of a cyanosed neonate?

A
  • stabilise airway, breathing and circulation
  • start prostaglandin infusion to keep ductus arteriosus open as neonates will be duct dependent to allow
50
Q

syndrome associated with aortic stenosis?

A

turners