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RACP Written 2018 > Cardiology - Congenital Heart Disease > Flashcards

Cardiology - Congenital Heart Disease Flashcards

1
Q

Differential for Acyanotic Congenital Heart Disease

A 
Ventricular septal defect (most common)
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
Aortic valve stenosis
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2
Q

Which septal defects are most common?

A

Ventricular septal defect is MOST COMMON
BUT
Atrial septal defects are more commonly NEWLY diagnosed in adults

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3
Q

Differential of Cyanotic Congenital Heart Disease

A

Tetralogy of Fallot (more common than TGA BUT TGA presents earlier)
Transposition of the great arteries (TGA)
Tricuspid atresia
Pulmonary vein sTenosis

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4
Q

Is atrial septal defects more common in males or females?

A

Females

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5
Q

What conditions is ostium primum atrial septal defect associated with?

A

Downs
Cleft mitral valve
VSD
Subaortic stenosis

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6
Q

Downs Syndrome is associated with which atrial septal defect?

A

Ostium primum

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7
Q

Early life presentations of atrial septal defects

A

Early life:

  • usually asymptomatic
  • increased tendency to LRTIs
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8
Q

Late life presentations of atrial septal defects

A

Left to right shunting increases when HTN or CAD reduces LV compliance

Atrial arrhythmia

Pulmonary hypertension

Right heart failure

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9
Q

Which familial syndrome is associated with Ostium secundum atrial septal defect? Which chromosome is involved?

A

Familial ostium secundum

Autosomal dominant linked to Ch5

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10
Q

Atrial Septal Defect - auscultation findings

A

Split S1 with loud T1
Widely split FIXED S2

Systolic:
- midsystolic pulmonary outflow murmur

Diastolic:
- mid diastolic rumble at 4th ICS LSB (due to increased flow across tricuspid)

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11
Q

ECG findings in Ostium Secundum Atrial Septal Defect

A

R axis deviation

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12
Q

ECG findings in Ostium Primum Atrial Septal Defect

A

L axis deviation

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13
Q

When to close an Atrial Septal Defect?

A
  • Symptoms
  • Haemodynamically significant shunt (RV enlargement and Qp:Qs ratio >1.5 (pulm:systemic blood flow)
  • Arrythmias (BEST predictor of preclosure is AGE)
  • Paradoxical embolisaiton
  • Platypnoea/Orthopnoea Syndrome = dyspnoea and hypoxia on upright due to increased R to L shunting when upright
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14
Q

When NOT to close an Atrial Septal Defect?

A
  • Irreversible pulm arterial hypertension (ie Eisenmenger physiology)

BECAUSE: if it is closed with severe pulmonary hypertension there is possibility for haemodyanmic collapse AND can convert to primary pulmonary hypertension which has a worse prognosis

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15
Q

How do Ventricular Septal Defects present?

A

Murmur: loud holosystolic at LLSB

Heart failure with dilated LV

Endocarditis

Cyanosis if pulmonary hypertension

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16
Q

Complications of Ventricular Septal Defects?

A

1) Ao regurgitation (due to poorly supported RCA cusp)
2) Infective endocarditis
3) Eisenmengers
4) Right heart failure
5) Pulmonary hypertension

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17
Q

When to close a Ventricular Septal Defect?

A
  • Haemodynamically significant shunt with:
    1) symptoms
    2) LV enlargement
    3) Qp:Qs >2
  • Pulmonary hypertension with net L to R shunt
  • Other: infective endocarditis / aortic regurg / RVOTO
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18
Q

What conditions predispose to a Patent Ductus Arteriosus?

A
  • maternal rubella

- neonatal prematurity

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19
Q

What is differential cyanosis?

A

Cyanosis + clubbing of toes

  • when severe pulmonary vascular disease results in reversal of flow through the ductus (–> unoxygenated blood shunted to descending aorta)
    (ie: patent ductus arteriosus)
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20
Q

Clinical features of patent ductus arteriosus

A
  • Continuous murmur beneath left clavicle
  • -> assoc with thrill
  • -> machine quality
  • wide pulse pressure with ‘bounding’ pulses
  • usually develop EISENMENGER’S
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21
Q

Complications of Patent Ductus Arteriosus

A
  • Heart failure
  • Infective endocarditis (10% lifetime risk)
  • Severe pulmonary vascular obstruction can cause aneurysmal dilatation, calcification and rupture of ductus
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22
Q

When to close a Patent Ductus Arteriosus

A
  • left chamber enlargement
  • pulmonary hypertension with L to R shunt
  • previous IE
  • audible murmur

* DO NOT CLOSE if EISENMENGER or if ASYMPTOMATIC+INAUDIBLE*

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23
Q

DDx for continuous murmur

A
  • PDA
  • Coronary fistula
  • Ruptured sinus of valsalva aneurysm
  • Aortopulmonary window
24
Q

How does EISENMENGER’S SYNDROME develop?

A

Starts with L to R shunt but

  • -> over time develops increased pulmonary blood flow and increased peripheral vascular resistance
  • -> turns into reverse shunt R to L
25
Q

What defects are associated with EISENMENGER’S SYNDROME?

A

VSD
ASD
PDA

26
Q

Clinical Features of EISENMENGER’S SYNDROME?

A
  • Original murmur may disappear
  • cyanosis
  • clubbing
  • RV failure
  • haemoptysis
27
Q

Clinical complications of EISENMENGER’S SYNDROME?

A
  • erythrocytosis with hyperviscosity
  • abnormal haemostasis
  • gallstones (due to haem pigment)
  • gout (due to hyperuricaemia)
  • stroke or cerebral abscess
  • (late) heart failure
  • sudden cardiac death
28
Q

Treatment of EISENMENGER’S

A

1) GENERAL:
- avoid intravascular depletion / pregnancy / vasodilators / altitude
- endocarditis prophylaxis
- bubble filters of IV lines

2) PHLEBOTOMY for hyperviscosity (NOT for Hct)
- aim pre-surgery HcT >0.65
- BEWARE of iron deficiency causing microcytosis and worsening hyperviscosity

3) PULMONARY VASODILATORS
- bosasentan and sildenafil

4) HEART&LUNG Transplant
- if syncope or refractor RHF

29
Q

When to close a Patent Foramen Ovale?

A
  • platypnoea/orthodeoxia syndrome
  • decompression illness
  • migraine
  • stroke
30
Q

What increases the risk of stroke in Patent Foramen Ovale?

A

Atrial septal aneurysm PLUS PFO

31
Q

Gender imbalance in coarctation of aorta?

A

Male predominant

32
Q

Associated congenital disease with COARCTATION OF AORTA

A
  • biscuspid aortic valve (in >50%)
  • VSD
  • PDA
  • Double inlet LV
  • Subaortic stenosis (Shone Syndrome)
  • Hypoplastic left heart syndrome
33
Q

Shone Syndrome features?

A
  • supravalvular mitral membrane
  • parachute mitral valve
  • subaortic stenosis
  • coarctation of the aorta
34
Q

Intracerebral complication of COARCTATION OF AORTA?

A

10% have circle of willis berry aneurysm

35
Q

Clinical features of coarctation of aorta

A

Headache
Epistaxis
Angina
Exercise claudication

Hypertension of the UPPER limbs

  • absent/diminished femoral pulse
  • radiofemoral delay
  • enlarged and pulsatile intercostal vessels
36
Q

Murmur of coarctation of aorta?

A
  • midsystolic over left interscapular space
  • murmur can become continuous if the lumen narrows ++++
  • if bicuspid aortic valve as well then may have ejection click
  • S4 often
37
Q

What is the ‘3’ sign?

A

Notching of the 3rd to 9th ribs in COARCTATION OF AORTA due to inferior rib erosion by dilated collateral vessels

38
Q

Complications of COARCTATION OF AORTA?

A
  • cerebral aneursym rupture
  • aortic dissection and rupture
  • premature coronary atherosclerosis
  • aortic valve failure
  • LV failure
  • infective endocarditis at coarctated site
39
Q

When to treat COARCTATION OF AORTA?

A
  • Hypertension AND peak-peak gradient >20mmHg

- severe anatomic coarctation with significant collaterals

40
Q

Treatments available for COARCTATION OF AORTA?

A
  • percutaneous (efficacious and safe)
  • stents BETTER than balloon angioplasty
  • covered stents are SAFER
41
Q

Do you get hypertension post treatment for coarctation of aorta?

A

30- 75% of patients develop hypertension

42
Q

What is the BEST PREDICTOR for LONGTERM SURVIVAL post repair of coarctatino of aorta?

A

Age

43
Q

Name the three types of pulmonary stenosis and what their associated conditions are?

A

VALVULAR: (often isolated) assoc with Noonan’s syndrome

SUPRAVALVULAR: associated with Williams Syndrome

SUBVALVULAR: assoc with VSD

44
Q

What condition is SUPRAVALVULAR pulmonary stenosis associated with?

A

Williams syndrome

45
Q

What condition is VALVULAR pulmonary stenosis associated with?

A

Noonan’s syndrome

46
Q

What condition is SUBVALVULAR pulmonary stenosis associated with?

A

VSD

47
Q

Heart sounds in pulmonary stenosis?

A

S4

Harsh systolic cresc-decresc + thrill in LUSB

May get TR holosystolic murmur if severe

If SUPRAVALVULAR: systolic or continuous best heard over the narrowing

48
Q

Non-murmur findings in pulmonary stenosis?

A
  • prominent ‘a’ waves of JVP
  • Presystolic pulsation of liver due to vigorous atrial contraction
  • cyanosis if there is a R to L shunt via PFO or ASD
49
Q

Treatment of pulmonary stenosis?

A

Balloon valvuloplasty with excellent outcomes

50
Q

Features of TETRALOGY OF FALLOT

A

1) VSD
2) RVOTO (subpulmonary stenosis or pulmonary atresia)
3) Overriding aorta
4) RVH

51
Q

Genetic assocations with TETRALOGY of FALLOT

A
  • 15% have 22q11 chromosome microdeletion

- Assoc Down’s Syndrome

52
Q

Long term complications of tetralogy of fallor repair?

A
  • Pulmonary regurgitation
  • RV outflow obstruction or branch pulmonary artery stenosis
  • LV dysfunction
  • Arrhythmias: atrial (25%), ventricular (15%)
53
Q

Predictors of sudden cardiac death in ventricular arrythmias post tetralogy of fallot repair?

A
  • LVEDP >12mmHg
  • NSVT
  • QRS >180ms
54
Q

EBSTEIN ANOMALY? What is it?

A

Downward displacement of the tricuspid valve into the RV due to anomalous attachment of tricuspid leaflets

The Ebstein tricuspid valve is DYSPLASTIC
–> leads to tricuspid regurgitation

55
Q

Why does Ebstein Anomaly occur?

A

Due to failure of delamination

Can be caused by in utero lithium exposure

56
Q

What cardiac condition is associated with in utero lithium exposure?

A

Ebstein anomaly

57
Q

Associated conditions with Ebstein Anomaly?

A
  • ASD or PFO (in 80%)
  • 20% have accessory pathways with tachyarrhythmias (ie WPW)
  • Tricuspid regurgitation

RACP Written 2018 (167 decks)

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