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
What are the features of a VSD?
Failure to thrive Sweating Tachypnoea Heart murmur
26
How is a VSD managed medically?
Weight gain Diuretics + ACEi Digoxin
27
How is a VSD manage surgically?
Surgically repaired
28
What is an ASD?
Atrial septal defect
29
What is the sign of an ASD?
Soft systolic mrmur due to high flow across pulmonary valve
30
What is a PDA?
Patent ductus arteriosus Failure of ductus arteriosus to close by 1 month after suspected delivery
31
What is hypoplastic left heart syndrome?
Underdevelopment of the left side of the heart
32
What often presents alongside hypoplastic left heart?
Aortic coarctation
33
What does a baby with hypoplastic left heart require for the heart to be functional?
ASD
34
How is hypoplastic left heart managed?
Urgen atrial balloon septostomy - to make ASD Repaired using Norwood procedure
35
What is tricuspid atresia?
When tricuspid fails to form properly
36
What happens in tricuspid atresia?
R->L atrial shunt No RV inlet Blood flow to lungs via VSD/PDA
37
What is pulmonary atresia?
When pulmonary valve fails to form properly
38
What happens in pulmonary atresia?
No RV outlet R->L atrial shuny Blood flow to lungs via PDA
39
What is Ebstein's anomaly?
Tricuspid valve set lower causing a larger RA and a smaller RV
40
What is Ebstein's anomaly associated with?
R->L shunt via ASD | Wolff-Parkinson-White syndrome
41
What is the presentation of Ebstein's anomaly?
Heart failure Gallop rhythm Cyanosis
42
What is Eisenmenger syndrome?
When blood flows R->L across lesion bypassing the lungs
43
What lesions can lead to Eisenmenger syndrome?
ASD VSD PDA
44
What is the pathophysiology of Eisenmenger syndrome?
L->R shunt down pressure gradient Over time leads to pulmonary hypertension + RV hypertrophy Shunt reverses