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Paediatrics > Congenital Heart Disease > Flashcards

Congenital Heart Disease Flashcards

1
Q

What is the most common cyanotic congenital heart disease?

A

Tetralogy of fallot

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2
Q

What makes up tetralogy of fallot?

A

VSD
Pulmonary stenosis
Right ventricular hypertrophy
Overriding aorta

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3
Q

What are risk factors for tetralogy of fallot?

A

Male
Maternal teratogen use
CHARGE syndrome
Digeorge syndrome

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4
Q

What is CHARGE syndrome?

A

CHD7 mutation

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5
Q

What are the features of CHARGE syndrome?

A 
Colobloma
Heart defects
Atresia choneae 
Retardation of growth/development
Genitourinary abnormalities
Ear anomalies
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6
Q

What is DiGeorge syndrome?

A

22q11 deletion

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7
Q

What are the features of DiGeorge syndrome?

A

Learning problems
Speech/hearing problems
Cleft lip/palate
Hypoparathyroidism

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8
Q

What is the pathophysiology of TOF?

A

VSD - right to left shunt
PS - narrowing of right ventricular outflow
RVH - response to high pressures due to PS
Overriding aorta - dilated + displaced over IVS

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9
Q

What is the key feature of TOF?

A

Tets spells

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10
Q

What is a tet spell?

A

Periods of hypoxia

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11
Q

What happens in a tet spell?

A

Increase in right to left shunt - due to hypernoea
Increasing cyanosis
Irritability

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12
Q

How is a tet spell managed?

A

Bring knees to chest - increase systemic resistance
Morphine
Oxygen

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13
Q

How is suspected TOF investigated?

A

ECHO

CXR = boot sign

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14
Q

How is TOF managed?

A

Prostins

Surgery

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15
Q

What is transposition of the great arteries?

A

Swapping over of aorta and pulmonary artery

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16
Q

What are the types of TGA?

A

dTGA

ccTGA

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17
Q

What is dTGA?

A

Dextro-transposition - aorta is anterior + to right of PA

Aorta connected to RV
PA connected to LV

Needs an VSD/ASD/PDA

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18
Q

WHat is ccTGA?

A

Congenitally corrected - aorta is anterior + to left of PA

Ventricles swapped

  • LA -> RV -> aorta
  • RA -> LV -> PA
19
Q

What are the features of TGA?

A

Cyanosis

Right ventricular heave

20
Q

What are the investigations for suspected TGA?

A

ECHO

CXR = egg on string

21
Q

How is TGA managed?

A

Prostins - keep PDA open
Urgent atrial balloon septostomy
Surgery

22
Q

What is VSD?

A

Ventricular septal defect - hole in ventricular septum

23
Q

What is the pathophysiology of a VSD?

A

Left -> right shunt
Small = no change to pulmonary flow

Moderate = left atrial/ventricular dilatation due to increase blood flow from lungs + pulmonary hypertension

Large = heart failure + pulmonary hypertension

24
Q

What is Eisenmenger’s syndrome?

A

Condition where pressure in RV exceeds pressure in LV due to increase in pulmonary resistance

Causes shunt reversal and cyanosis

25
Q

What are the features of a VSD?

A

Failure to thrive
Sweating
Tachypnoea
Heart murmur

26
Q

How is a VSD managed medically?

A

Weight gain
Diuretics + ACEi
Digoxin

27
Q

How is a VSD manage surgically?

A

Surgically repaired

28
Q

What is an ASD?

A

Atrial septal defect

29
Q

What is the sign of an ASD?

A

Soft systolic mrmur due to high flow across pulmonary valve

30
Q

What is a PDA?

A

Patent ductus arteriosus

Failure of ductus arteriosus to close by 1 month after suspected delivery

31
Q

What is hypoplastic left heart syndrome?

A

Underdevelopment of the left side of the heart

32
Q

What often presents alongside hypoplastic left heart?

A

Aortic coarctation

33
Q

What does a baby with hypoplastic left heart require for the heart to be functional?

A

ASD

34
Q

How is hypoplastic left heart managed?

A

Urgen atrial balloon septostomy - to make ASD

Repaired using Norwood procedure

35
Q

What is tricuspid atresia?

A

When tricuspid fails to form properly

36
Q

What happens in tricuspid atresia?

A

R->L atrial shunt
No RV inlet
Blood flow to lungs via VSD/PDA

37
Q

What is pulmonary atresia?

A

When pulmonary valve fails to form properly

38
Q

What happens in pulmonary atresia?

A

No RV outlet
R->L atrial shuny
Blood flow to lungs via PDA

39
Q

What is Ebstein’s anomaly?

A

Tricuspid valve set lower causing a larger RA and a smaller RV

40
Q

What is Ebstein’s anomaly associated with?

A

R->L shunt via ASD

Wolff-Parkinson-White syndrome

41
Q

What is the presentation of Ebstein’s anomaly?

A

Heart failure
Gallop rhythm
Cyanosis

42
Q

What is Eisenmenger syndrome?

A

When blood flows R->L across lesion bypassing the lungs

43
Q

What lesions can lead to Eisenmenger syndrome?

A

ASD
VSD
PDA

44
Q

What is the pathophysiology of Eisenmenger syndrome?

A

L->R shunt down pressure gradient
Over time leads to pulmonary hypertension + RV hypertrophy

Shunt reverses

Paediatrics (40 decks)

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