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RACP Written 2018 > Cardiology - Congenital Heart Disease > Flashcards

Cardiology - Congenital Heart Disease Flashcards

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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

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

How does EISENMENGER’S SYNDROME develop?

Study These Flashcards
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
What defects are associated with EISENMENGER'S SYNDROME?
VSD ASD PDA
26
Clinical Features of EISENMENGER'S SYNDROME?
- Original murmur may disappear - cyanosis - clubbing - RV failure - haemoptysis
27
Clinical complications of EISENMENGER'S SYNDROME?
- 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
Treatment of EISENMENGER'S
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
When to close a Patent Foramen Ovale?
- platypnoea/orthodeoxia syndrome - decompression illness - migraine - stroke
30
What increases the risk of stroke in Patent Foramen Ovale?
Atrial septal aneurysm PLUS PFO
31
Gender imbalance in coarctation of aorta?
Male predominant
32
Associated congenital disease with COARCTATION OF AORTA
- biscuspid aortic valve (in >50%) - VSD - PDA - Double inlet LV - Subaortic stenosis (Shone Syndrome) - Hypoplastic left heart syndrome
33
Shone Syndrome features?
- supravalvular mitral membrane - parachute mitral valve - subaortic stenosis - coarctation of the aorta
34
Intracerebral complication of COARCTATION OF AORTA?
10% have circle of willis berry aneurysm
35
Clinical features of coarctation of aorta
Headache Epistaxis Angina Exercise claudication Hypertension of the UPPER limbs - absent/diminished femoral pulse - radiofemoral delay - enlarged and pulsatile intercostal vessels
36
Murmur of coarctation of aorta?
- 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
What is the '3' sign?
Notching of the 3rd to 9th ribs in COARCTATION OF AORTA due to inferior rib erosion by dilated collateral vessels
38
Complications of COARCTATION OF AORTA?
- cerebral aneursym rupture - aortic dissection and rupture - premature coronary atherosclerosis - aortic valve failure - LV failure - infective endocarditis at coarctated site
39
When to treat COARCTATION OF AORTA?
- Hypertension AND peak-peak gradient >20mmHg | - severe anatomic coarctation with significant collaterals
40
Treatments available for COARCTATION OF AORTA?
- percutaneous (efficacious and safe) - stents BETTER than balloon angioplasty - covered stents are SAFER
41
Do you get hypertension post treatment for coarctation of aorta?
30- 75% of patients develop hypertension
42
What is the BEST PREDICTOR for LONGTERM SURVIVAL post repair of coarctatino of aorta?
Age
43
Name the three types of pulmonary stenosis and what their associated conditions are?
VALVULAR: (often isolated) assoc with Noonan's syndrome SUPRAVALVULAR: associated with Williams Syndrome SUBVALVULAR: assoc with VSD
44
What condition is SUPRAVALVULAR pulmonary stenosis associated with?
Williams syndrome
45
What condition is VALVULAR pulmonary stenosis associated with?
Noonan's syndrome
46
What condition is SUBVALVULAR pulmonary stenosis associated with?
VSD
47
Heart sounds in pulmonary stenosis?
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
Non-murmur findings in pulmonary stenosis?
- 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
Treatment of pulmonary stenosis?
Balloon valvuloplasty with excellent outcomes
50
Features of TETRALOGY OF FALLOT
1) VSD 2) RVOTO (subpulmonary stenosis or pulmonary atresia) 3) Overriding aorta 4) RVH
51
Genetic assocations with TETRALOGY of FALLOT
- 15% have 22q11 chromosome microdeletion | - Assoc Down's Syndrome
52
Long term complications of tetralogy of fallor repair?
- Pulmonary regurgitation - RV outflow obstruction or branch pulmonary artery stenosis - LV dysfunction - Arrhythmias: atrial (25%), ventricular (15%)
53
Predictors of sudden cardiac death in ventricular arrythmias post tetralogy of fallot repair?
- LVEDP >12mmHg - NSVT - QRS >180ms
54
EBSTEIN ANOMALY? What is it?
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
Why does Ebstein Anomaly occur?
Due to failure of delamination Can be caused by in utero lithium exposure
56
What cardiac condition is associated with in utero lithium exposure?
Ebstein anomaly
57
Associated conditions with Ebstein Anomaly?
- ASD or PFO (in 80%) - 20% have accessory pathways with tachyarrhythmias (ie WPW) - Tricuspid regurgitation

RACP Written 2018 (167 decks)

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