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Y5 Paediatrics > Cardiology > Flashcards

Cardiology Flashcards

1
Q

Where is the obstruction in the vast majority of aortic coarctation cases?

A

Distal to L subclavian artery, often at location of ductus arteriosus (aortic isthmus)

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2
Q

Is aortic coarctation more common in boys or girls? Syndromic caveat?

A

Boys

But seen in Turner syndrome

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3
Q

What lab finding might be apparent in aortic coarctation and why?

A

Raised lactate as hypoperfuse lower limbs

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4
Q

How does aortic coarctation present on exam?

A

Disparity in pulse volume in arms vs legs, also BP differences
Absent femorals
Systolic murmur below left clavicle is typical but variable

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5
Q

When might a coarctation present and why? What implications does this have for management?

A

Day 2 plus of life after a PDA has closed - it is duct dependent
Can give prostins to keep duct open and prevent CV collapse

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6
Q

What is milrinone and what is it used for?

A

Phosphodiesterase 3 inhibitor which increases heart contractility and reduces pulmonary vascular resistance, also decreases afterload via peripheral vasodilation
Used in heart failure and LCOS as well as post cardiac surgery to maintain ventricular function

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7
Q

How are PDAs managed?

A

Medical - IV indomethacin, ibuprofen in first 14 days of life
Surgical - cardiac catheter closure or ligation via open thoracotomy

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8
Q

What is ALCAPA?

A

Anomalous Left Coronary Artery from Pulmonary Artery
L heart therefore gets oxygen poor blood and undergoes ischaemia. Low BP in pulmonary artery can also cause reversal of flow over time so blood flows from LCA into pulmonary artery - pulmonary steal and worsening ischaemia

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9
Q

How many vessels are in the umbilical cord?

A

3 - 2 UAs and 1 UV

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10
Q

Where do the umbilical arteries branch off from?

A

Internal iliacs

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11
Q

What does the ductus venosus do?

A

Shunts blood across the Fetal liver to the IVC

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12
Q

What shunts blood across the nonfunctional Fetal liver? Into what?

A

Ductus venosus to the ivc

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13
Q

How does blood flow through the Fetal heart and why?

A

From R atrium into L atrium via foramen ovale
Because pulmonary vascular pressures are very high (hypoxic pulmonary vasoconstriction) which transmits back into RV, so pressure in R heart is greater than L and blood flows from R to L

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14
Q

What does the ductus arteriosus do?

A

Allows blood to flow from the pulmonary artery into the descending aorta at the isthmus, bypassing the nonfunctional Fetal lungs

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15
Q

What are the 2 methods the Fetal circulation uses to bypass the lungs?

A

Foramen ovale

Ductus arteriosus

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16
Q

What are the 3 shunts in the Fetal circulation?

A

Ductus venosus
Ductus arteriosus
Foramen ovale

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17
Q

What 2 major circulatory changes occur when the baby is born and what happens as a result of this?

A

Placenta disconnects from circulation
Lungs become functional and pulmonary pressure falls
RA and RV pressures fall; eventually to less than L sided pressures and flow reverses, closing the foramen ovale

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18
Q

What physiologically clamps the umbilical cord?

A

Wharton’s jelly

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19
Q

Within how long of birth does the foramen ovale usually close?

A

Few mins

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20
Q

Why does the ductus arteriosus close? What keeps it open before this?

A

Due to rising oxygen levels in the circulating blood
Prior to this the placenta produces prostaglandins which helps to keep it open. When this falls after birth the DA closes

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21
Q

3 conditions that have ductus dependent systemic circulation?

A

Coarctation
Critical aortic stenosis
Hypoplastic L heart syndrome

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22
Q

Which way does the blood flow in conditions with ductus dependent systemic circulation?

A

From pulmonary artery to aorta

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23
Q

4 conditions with ductus dependent pulmonary circulation? Which way does the blood flow?

A 
Critical pulmonary stenosis
Pulmonary atresia
Tricuspid atresia
Tetralogy of Fallot
Blood flows from aorta to pulmonary artery
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24
Q

3 common L to R shunt heart defects?

A

VSD
PDA
ASD

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25
Q

2 common R to L shunt heart defects?

A

TOF

TOGA

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26
Q

What is the major presenting symptom of L to R shunts and stenotic heart disease/coarctation?

A

Breathlessness

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27
Q

What is the major presenting sign of R to L heart disease shunts?

A

Blueness

28
Q

What is the most common mixing heart disease that causes breathlessness and blueness?

A

Complete AVSD

29
Q

What are the components of ToF?

A

Overriding aorta
Which overrides a large VSD
Pulmonary infundibular stenosis (R ventricular outflow tract obstruction)
Which causes RVH

30
Q

4 diseases/syndromes associated with ToF?

A

Down’s syndrome
Di George syndrome
Fetal alcohol/valproate/phenytoin
PKU

31
Q

How does ToF present?

A

Cyanosed and clubbed kids

Kids may squat or present with apnoeic tet spells

32
Q

When do tet spells often occur?

A

Whilst/after feeding
Crying
Defecating
Tachycardia or hypotension

33
Q

What is the common underlying cause of ToF?

A

Failure of subpulmonic conus to expand

34
Q

What factor influences the severity/age of presentation of ToF?

A

The degree of RVOTO (stenosis)

35
Q

What is the underlying cause of tet spells?

A

Sudden decrease in SVR which increases the R to L shunting across the VSD, acutely decreasing PaO2 and increasing PaCO2
This causes acidosis and increase in breathing rate, which increases negative thoracic pressures and increase venous return to R heart, worsening the R to L shunt
Which carries on the cycle

36
Q

What is responsible for the classic XR finding in ToF?

A

Boot shaped heart - the prominent R ventricle and decreased pulmonary vascularity

37
Q

How do you manage a tet spell?

A

High flow O2
Calm down, reduce pain
Encourage squatting if older
Consider opioids

38
Q

Why do kids with ToF squat?

A

To increase systemic vascular resistance and reduce the R to L shunt
Also acutely drops venous return

39
Q

Broadly how do you manage ToF?

A

Duct dependent so give prostin to maintain patency and surgically correct within 1st year of life

40
Q

Why is TOGA bad and what is required to maintain blood flow to body?

A

Creates 2 separate parallel circulations

So needs PDA or VSD to keep L to R shunt and maintain oxygenation

41
Q

CXR sign of TOGA?

A

Egg on a string appearance of heart

42
Q

What is persistent truncus arteriosus?

A

Failure of truncus to divide into pulmonary artery and aorta

Leads to mixed blood in circulations causing cyanosis at birth

43
Q

What is Ebsteins anomaly?

A

Medial displacement of the septal and posterior leaflets of the tricuspid valve into R ventricle, creating severe tricuspid regurgitation and functional RV hypoplasia

44
Q

What maternal medication is a RF for Ebsteins anomaly?

A

Lithium

45
Q

What rhythmic disturbance is associated with Ebsteins anomaly?

A

WPW syndrome

46
Q

What are TAPVD and PAPVD?

A

One or more pulmonary veins don’t return to L atrium
In TAPVD, a single pulmonary vein drains straight to the SVC leading to mixing
Needs a R to L shunt to maintain systemic circulation

47
Q

What is Eisenmengers syndrome?

A

Seen in long term L to R shunt defects that would usually only cause breathlessness (ASD, VSD, PDA)
Over time pulmonary hypertension due to this and causes RVH, which eventually reverses the defect into a R to L shunt and causes cyanosis

48
Q

What is heterotaxy/isomerism?

A

Improper positioning of visceral organs, including situs invertus

49
Q

What is R atrial isomerism?

A

Asplenic syndrome - as if body was trying to be symmetrically R sided so have bilateral SVC, APVD, TOGA, no Coronary sinus, pulmonary stenosis, endocardial cushion defects e.g. AVSD
Plus no spleen, midline liver, trilobar lungs, R sided stomach with malrotation

50
Q

What is L atrial isomerism?

A

Polysplenic syndrome - as if body was trying to make itself symmetrically L sided so get interrupted ivc, azygous continuation, pulm/aortic stenosis, AVSD
Plus multiple but non functional splenules, bilobated lungs, hepatic drainage into L atrium and gut malrotation

51
Q

What is the Norwood procedure?

A

Treatment for Hypoplastic L heart syndrome made of 3 steps (staged palliation) as alternative to heart transplant

52
Q

What are the 3 steps of the Norwood procedure?

A

Stage 1 where R ventricle is turned into main ventricle for body and lungs
Stage 2 - bidirectional Glenn operation (6m after stage 1) to allow half of blood to flow around lungs
Stage 3 - Fontan (18-36m post Glenn) to connect IVC to pulmonary artery

53
Q

What is the surgery for Hypoplastic L heart syndrome called?

A

Norwood procedure with bidirectional Glenn and Fontan

54
Q

What 2 heart defects is Di George particularly associated with?

A

TOF

Truncus arteriosus

55
Q

What cardiac defect is mother with DM associated with?

A

Transposition of great arteries

56
Q

What does a PDA murmur sound like?

A

Continuous syst and diast machine like murmur

57
Q

What infection is associated with PDA?

A

Rubella

58
Q

What are the characteristics of an innocent cardiac murmur?

A

Very positional
Musical quality
Worsens with e.g. Infection or tachycardia

59
Q

What is the most common type of ASD? Other types?

A

Secundum most common

Also primum, sinus venosus and PFO

60
Q

What heart defect is associated with trisomy 21?

A

Endocardial cushion defect - AVSD plus common valve in middle

61
Q

What is a tet spell an indication for?

A

Surgery ASAP

62
Q

When is surgery for TOF usually done?

A

Wait til at least 5m of age (4-5kg plus) if poss

63
Q

What heart defect is the most common cause of neonatal cyanosis?

A

Transposition of great arteries

64
Q

What other defect accompanies a truncus arteriosus?

A

Large VSD

65
Q

What does tricuspid lead to and what does it require for viability?

A

Leads to Hypoplastic RV

Needs both ASD and VSD for viability

66
Q

What is the spot diagnosis for cyanotic heart defect presenting with heart taking up while thorax on CXR?

A

Ebsteins anomaly

Y5 Paediatrics (13 decks)

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