Congenital Heart Disease Flashcards

1
Q

Who gets atrioventricular septal defects?

A

40% of Down’s children

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2
Q

What is considered ‘complete AVSD’?

A

A single leaky 5 leaflet valve which joins the atrium to the ventricle

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3
Q

What are the clinical features of AVSD?

A

Pulmonary HTN

Cyanosis at birth/ HF in 2-3 wks

No murmur

Superior axis on ECG

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4
Q

What investigations do you do for AVSD?

A

Antenatal US- apparent

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5
Q

What is the management for AVSD?

A

Medical treatment for HF (Furosemide/ ACEi)

Surgical repair at 3-6 months

Lifelong follow up –catching AV regurge or LVOT obstruction

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6
Q

What is the most common form of complex congenital heart disease?

A

Tricuspid atresia

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7
Q

What are the clinical features of tricuspid atresia?

A

Absence of tricuspid valve

Mixing of systemic and pulmonary return in LA

Cyanosis (duct dependent)

May be well at birth

ESM at L sternal edge

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8
Q

What investigations would you do for tricuspid atresia?

A

CXR: cardiomegaly, prominent RA border (Ebstein’s abnormality)

ECG: LAD

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9
Q

What is the management of tricuspid atresia?

A
  1. Maintain pulmonary blood supply via BT shunt insertion or Pulmonary artery banding
  2. Glenn operation (SVC to pulmonary artery)
  3. Fontan operation (IVC to pulmonary artery)

[Complete correction not possible]

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10
Q

Which CHD is the most common?

A

VSD (80%)

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11
Q

What is the size of a VSD based on?

A

Larger or smaller than an aortic valve

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12
Q

What are the clinical features of a small VSD?

A

Asymptomatic

Loud Pan systolic murmur at LSE

Quiet pulmonary second sound

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13
Q

What are the features of a large VSD?

A
HF/ SOB
poor growth
chest infections
tachypnoea
active precordium
soft pansystolic murmur
apical mid diastolic murmur loud pulmonary second sound
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14
Q

What investigations would you do for VSD?

A

CXR
ECG
Echo

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15
Q

What findings would you get if a patient has a VSD?

A

Small: CXR/ECG: normal
Echo: anatomical defect

Large:
CXR: cardiomegaly, large pulm arteries, increased vassc markings, pulmonary oedema

ECG: Biventricular hypertrophy, demonstrates the anatomy of the defect and Pulm HTN

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16
Q

What is the management of a (A) small and (B) large VSD?

A

Small: Closes spontaneously
Good dental hygiene to avoid IE

Large: Diuretics (captopril) for HF, nutritional support, surgery at 4-6 mnths

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17
Q

What are the types of ASD?

A

Secundum (common 80%)
Central defect involving FO

Primum (less common)

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18
Q

What are the clinical features of ASD?

A

Asymptomatic

Recurrent chest infections

Arrhythmias

ESM, fixed widely split S2

Partial = apical pansystolic murmur

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19
Q

What are the investigation findings in ASD?

A

CXR: Cardiomegaly, Enlarged pulm arteries, increased pulm vasc markings

ECG: Superior QRS

Echo: Structural abnormality, definitive

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20
Q

How common is PDA?

A

1 in 5000

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21
Q

Where is the join in PDA?

A

Pulmonary artery to descending aorta

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22
Q

What are the clinical findings of PDA?

A

Continuous murmur below left clavicle

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23
Q

What are the investigations in PDA?

A

CXR/ECG normal or LVH and pulmonary HTN

Echo: diagnostic

24
Q

What is the management of a PDA?

A

Closure
Preterm: indomethacin/ ibuprofen for closure
Surgical ligation

Older child: Coil or occlusive device via cardiac catheter at 1 year

Diuretics if excess pulm blood flow

(If transposition of great arteries or other cyanotic disease: prostaglandins until corrective surgery because PDA needs to stay open)

25
Q

What is the most common cyanotic CHD?

A

Tetralogy of Fallot

26
Q

What are the 4 diagnostic criteria in Tetralogy of Fallot?

A
  1. Subpulmonary stenosis causing right ventricular outflow tract obstruction
  2. A large ventricular septal defect
  3. Dextroposition of the aorta so that it overrides the ventricular septum
  4. Right ventricular hypertrophy (as a result of RVOT obstruction)
27
Q

What are some signs of ToF?

A

Severe cyanosis, tet spells, squatting on exercise

Clubbing, ESM on LSE

28
Q

What investigations are used in ToF?

A

Antenatal

Murmur at 2months of life

CXR: normal but small heart, uptilted apex (boot shaped heart), pulm art bay, decreased pulm vasc markings

ECG: Normal at birth, RVH/ RAD

Echo

29
Q

What is the management of ToF?

A

Medical- prostaglandins to open DA

Surgical at 6 months- closure of VSD and relieving RVOT

Maybe: shunting to increase pulmonary flow (balloon or tube)

Prolonged Tet spells: Sedation/ pain relief, IV propranolol, IV volume administration, bicarbonate, muscle paralysis and artificial ventilation

30
Q

What is transposition of the great arteries? (TGA)

A

Aorta from RV and PA from LV

Only compatible with life if opening between two circuits (e.g. PDA)

31
Q

What are the signs of TGA?

A

Cyanosis

Loud single second heart sound

No murmur but potentially systolic

32
Q

What are the investigation findings of TGA?

A

CXR: Egg on string mediastinum, increase pulm vasc

ECG: normal

Echo: confirmatory

33
Q

What is the management of TGA?

A

Maintain PDA with prostaglandins

Balloon atrial septostomy

Arterial switch

34
Q

What is pulmonary stenosis?

A

Partial fusion of Pulmonary valve leaflets

35
Q

What are the clinical features of Pulmonary Stenosis?

A

Asymptomatic

Cyanotis

ESM at ULSE

Ejection click at ULSE

RV Heave

36
Q

What are the investigation findings of Pulmonary Stenosis?

A

CXR: normal or post stenotic dilatation of the pulmonary artery

ECG: RVH

Echocardiogram

37
Q

What is the management of Pulmonary Stenosis?

A

Transcatheter balloon dilation if pressure grad across pulmonary valve >64mm Hg

38
Q

What is Aortic Stenosis?

A

Stenosis, valve 1-3 leaflets

39
Q

What are the clinical features of Aortic Stenosis?

A

Reduced ET, chest pain, syncope

ESM, slow rising pulse, carotid thrill, delayed and soft aortic second sound, apical ejection click

40
Q

What are the investigation findings of Aortic Stenosis?

A

CXR: normal or prominent LV

ECG: LVH

Echo

41
Q

What is the management of Aortic Stenosis?

A

Monitoring

Balloon valvulotomy

AV replacement

42
Q

What is Coarctation of the Aorta?

A

Adult type: Narrowing of aorta, not duct dependent

Other: ductus arteriosus, duct tissue encircling aorta

43
Q

What are the clinical features of Coarctation of the Aorta (adult)?

A

Gradual

Asymptomatic

Radio femoral delay

ESM at ULSE

Collaterals heard at back

HTN

44
Q

What are the investigation findings of Coarctation of the Aorta (adult)?

A

CXR: Rib nothcing, 3 sign

ECG: LVH

Echo

45
Q

What is the management of Coarctation of the Aorta?

A

Stent if severe

Surgical repair

46
Q

What are the clinical features of Coarctation of the Aorta (adult)?

A

Other: Acute circulatory collapse at day 2 when DA closes, sick baby, HF, absent femorals, metabolic acidosis

47
Q

What are the investigation findings of Coarctation of the Aorta (adult)?

A

Other:

CXR: Cardiomegaly (HF)

ECG: normal

48
Q

What is Eisenmenger syndrome and how does it present?

A

L to R shunt not treated and progresses onto thick resistive pulmonary arteries which eventually undergoes shunt reversal in teen years (cyanotic)

49
Q

What is the management objective of Eisenmenger syndrome?

A

Early intervention

50
Q

What is Interruption of the aortic arch?

A

Rare, R to L shunt, VSD present

Presents with shock as a neonate

51
Q

What is the management of an interruption of the aortic arch?

A

Complete correction with VSD closure and repair of aortic arch within first few days

Associated with DiGeorge’s

52
Q

What is hypoplastic left heart syndrome?

A

An underdeveloped left heart

53
Q

How does hypoplastic left heart syndrome present?

A

Sick baby at birth

Weakness/ absence of peripheral pulses

Acidosis

Cardiovascular collapse

54
Q

What is the management of hypoplastic left heart syndrome?

A

Surgery (Norwood procedure)

55
Q

How can we classify congenital heart disease?

A

Acyanotic: L to R (ASD, VSD, PDA), outflow obstruction (AS/PS, COA, hypoplastic L heart, Eisenmonger’s syndrome)

Cyanotic (TA, TGA (R to L), AVSD, ToF (R to L), Eisenmenger)