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FRCR 2A (Crack The FRCR 2A) > 1. Cardiac (congenital) > Flashcards

1. Cardiac (congenital) Flashcards

1
Q

Most common aortic arch branching variant

A

Bovine arch (common origin of brachiocephalic trunk and right common carotid)

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2
Q

Definition of Left or Right arch

A

Relationship of aortic arch to trachea

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3
Q

Important types of right arch (2)

A

Right arch with mirror branching,
Right arch with aberrant left subclavian artery

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4
Q

Right arch w/aberrant left SCA anatomy (2)

A

Left SCA (aberrant) is the last branch.
Forms a vascular ring as the ligamentum arteriosum (on left) completes the ring encircling trachea

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5
Q

Right arch with mirror branching (clinical)

A

Commonly asymptomatic.

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6
Q

Right arch with mirror branching (associations) (2)

A

Strongly associated with congenital heart disease.
Most commonly associated with TOF, but more closely with Truncus.

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7
Q

Left arch, aberrant right subclavian - Trivia (2)

A

Commonest arch anomaly.
Origin of RSCA may be dilated (diverticulum of Kommerell)

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8
Q

Left arch, aberrant right subclavian - anatomy

A

RSCA is last branch, runs posterior to oesophagus and can cause dysphagia lusoria.

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9
Q

Double aortic arch - trivia

A

Commonest vascular ring.

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10
Q

Double aortic arch - clinical

A

Symptoms may begin at birth (tracheal compression, difficulty swallowing).

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11
Q

Double aortic arch - anatomy

A

Arches run posterior to oesophagus and anterior to trachea, encircling both.
Right arch is larger and higher than left.

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12
Q

Subclavian Steal Phenomenon/Syndrome (2)

A

Phenomenon: Occlusion in proximal subclavian (before giving off vertebral) causes retrograde flow in the ipsilateral vertebral artery.
Syndrome: above AND associated cerebral ischaemic symptoms

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13
Q

Subclavian steal - clinical

A

Exercising the affected upper limb triggers symptoms.

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14
Q

Subclavian steal - aetiology (5)

A

98% caused by atherosclerosis.
Other causes include:
- Takayasu (young),
- radiation
- pre-ductal coarctation
- Blalock-Taussig shunt.

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15
Q

CXR: Egg on String

A

Transposition

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16
Q

CXR: Snow man

A

TAPVR (Supracardiac)

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17
Q

CXR: Boot shaped

A

TOF

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18
Q

CXR: Figure 3

A

Coarctation

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19
Q

CXR: Box shaped heart

A

Ebstein

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20
Q

CXR: Scimitar sword

A

PAPVR with hypoplasia

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21
Q

Non-cardiac causes of high output failure (2)

A

Infantile haemangioendothelioma,
Vein of Galen malformation

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22
Q

Cyanotic with right sided arch (2)

A

TOF or Truncus
Increased pulmonary vascularity = Truncus
Decreased pulmonary vascularity = TOF

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23
Q

Cyanotic with left arch and massive heart (2)

A

Ebsteins
OR pulmonary atresia without VSD

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24
Q

Cyanotic with left arch and normal heart, increased pulmonary blood flow (4)

A

TAPVR (esp type 3)
D-transposition
Truncus (R arch)
Tingle ventricle

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25
Q

Cyanotic with left arch and normal heart, decreased/normal pulmonary blood flow (3)

A

TOF
Ebsteins
Pulmonary atresia

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26
Q

Cyanotic congenital heart diseases (6)

A

TOF
TAPVR
Transposition
Tricuspid atresia
Truncus arteriosus
Ebsteins

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27
Q

Non-cyanotic congenital heart diseases (5)

A

ASD
VSD
PDA
PAPVR
Aortic coarctation (adult type - post ductal)

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28
Q

CHF in newborn (5)

A

TAPVR (infracardiac, type III)
Congenital aortic or mitral stenosis
Left hypoplastic heart
Cor triatrium
Infantile (pre-ductal) coarctation

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29
Q

Survival dependent on PDA, VSD, PFO (5)

A

TOF (VSD)
Tricuspid atresia (VSD)
TAPVR (PFO)
Transposition
Hypoplastic left heart

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30
Q

Small Heart Ddx (3)

A

Adrenal insufficiency (addisons)
Cachexia
Constrictive pericarditis

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31
Q

VSD (trivia)

A

Commonest congenital heart disease.

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32
Q

VSD (types & Rx/prognosis) (3)

A

Membranous type is commonest (70%).
Outlet subtypes (infundibulum) must be repaired as right coronary cusp prolapses into the defect.
70% smaller ones close spontaneously

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33
Q

VSD: Imaging (4)

A

CXR: Non specific (large heart, increased vasculature, small aortic knob).
May show splayed carina (big left atrium).

34
Q

PDA (trivia) (2)

A

Normally closes functionally (24hrs) and anatomically (1 month).
Can be kept open or closed with meds.

35
Q

PDA (associations) (3)

A
  • Prematurity
  • Cyanotic heart disease
  • Maternal rubella
36
Q

PDA: Imaging (3)

A

CXR is non-specific:
big heart, increased pulmonary vasculature, large aortic arch (ductus bump)

37
Q

ASD: types (2)

A

Secundum is commonest type (50-70%).
Primum is larger and more likely to be symptomatic. Results from endocardial cushion defect.

38
Q

ASD: Management (2)

A

Only secundum closes without Rx (Primum, AV canal, sinus venosus won’t).
Primums not amenable to device closure due to proximity to AC valve.

39
Q

ASD: trivia

A

Commoner in females

40
Q

ASD: Imaging (4)

A

CXR: Non specific:
Big heart, increased vasculature, small aortic knob.
Small ones won’t show anything.

41
Q

Hand/thumb defects & ASD

A

Holt Oram

42
Q

Ostium primum ASD or Endocardial cushion defect

A

Downs

43
Q

Sinus Venosus ASD

A

PAPVR

44
Q

AV Canal (association)

A

Strong association with Downs. Most common congenital heart condition with Downs

45
Q

AV Canal (pathyphysiology) - (2)

A

aka endocardiac cushion defect.
Deficient development of part of atrial septum, inter-ventricular septum and AV valves.

46
Q

AV canal Rx (2)

A

Can’t use closure devices.
Complex surgical and medical Rx (beyond scope of FRCR)

47
Q

Unroofed coronary sinus (association)

A

Strong association with persistent left SVC.

48
Q

Unroofed coronary sinus (pathology)

A

Rare ASD, secondary to fenestrated or unroofed coronary sinus.

49
Q

Unroofed coronary sinus (clinical) (2)

A

Paradoxical emboli
Chronic right heart volume overload.

50
Q

PAPVR (anatomy)

A

One or more (of 4) pulmonary veins draining into the right atrium.

51
Q

PAPVR (clinical)

A

Usually no clinical significance.

52
Q

PAPVR (association) (2)

A

Associated with ASD (secundum and sinus venosus types).

53
Q

Right sided PAPVR

A

Sinus venosus ASD

54
Q

RUL PAPVR

A

SVC association with sinus venosus ASD

55
Q

Right sided PAPVR & Pulmonary hypoplasia

A

Scimitar syndrome

56
Q

TAPVR (anatomy/physiology) (3)

A

All of pulmonary veins drain into right side of heart.
Large PFO or ASD (more common) needed for survival.
3 types. All cause increased pulmonary vasculature.
Type 3 causes pulmonary oedema look in newborn.

57
Q

TAPVR (Types) (3)

A

Type 1: Supracardiac
- Commonest, veins drain above the heart.
- Snowman appearance
Type 2: Cardiac
- Second commonest
Type 3: Infracardiac
- Veins drain below diaphragm (hepatic veins or IVC)
- Obstruction on the way back through diaphragm is common, causes pulmonary oedema appearance.

58
Q

Asplenia & associated congenital heart (2)

A

almost 50% have congenital heart. Almost all have TAPVR.
85% also have endocardial cushion defects.

59
Q

Transposition (definition)

A

Aorta arises from right ventricle (defined by moderator band), pulmonary trunk from left ventricle.

60
Q

Transposition (trivia)

A

Commonest cause of cyanosis in first 24h.

61
Q

Transposition (association)

A

Associated with maternal diabetes.

62
Q

Transposition (types) (2)

A

D-type - dependent on PDA.
L-type - compatible with life

63
Q

D-transposition - imaging

A

CXR: Egg on string.

64
Q

D-transposition - anatomy

A

Due to discordance between vessels and ventricles. Creates 2 closed systems (right and left) connected by PDA.

65
Q

D-transposition - Rx (2)

A

Rx: Intra-atrial baffle (Mustard or Senning procedure).
Corrected D-transposition: PA draped over aorta, after surgeon perfoms LeCompte Maneuver

66
Q

L-transposition (anatomy) (2)

A

Congenitally corrected.
Double discordance: Vessels hook up to wrong ventricles, ventricles hook up to wrong atria.

67
Q

Tetralogy of Fallot (TOF) - physiology (4)

A

1-VSD
2-RVOT obstruction (often due to valve obstruction)
3-Overriding aorta
4-RV hypertrophy develops after birth

68
Q

TOF - Clinical (3)

A

Symptom extent depends on severity of RVOT obstruction.
If mild, may have “pink tet” which presents in early adulthood. This is called pentalogy of Fallot if there is an ASD.
Likely to have right arch.

69
Q

TOF - Rx & complication (3)

A

Usually fixed with primary repair.
Shunts like Blalock-Taussig only done if inoperable or to bridge until primary repair.
Commonest complication following surgery is pulmonary regurg

70
Q

Truncus arteriosus (anatomy) (3)

A

Single trunk supplying systemic and pulmonary circulation.
Always has VSD.
Associated with right arch and catch-22 (DiGeorge syndrome)

71
Q

Truncus arteriosus (associations) (2)

A

Right arch
Catch-22 (DiGeorge syndrome)

72
Q

Coarctation (Definition)

A

Narrowing of aortic lumen.

73
Q

Coarctation (types) (2)

A

Infantile (pre-ductal) - can have pulmonary oedema
Adult (ductal)

74
Q

Coarctation - associations (3)

A

Most commonly associated with bicuspid valve (80%).
Commonly associated with Turners (15-20%)
Berry aneurysms

75
Q

Coarctation - imaging (2)

A

Figure 3 sign on CXR
Rib notching, mainly 4-8th ribs, excludes 1-2nd ribs (supplied by costocervical trunk)

76
Q

Hypoplastic left heart (anatomy) (3)

A

Left ventricle and aorta are hypoplastic.
Must have ASD or large PFO.
Usually also have large PDA to supply blood to arch.

77
Q

Hypoplastic left heart (Clinical)

A

Present with pulmonary oedema.
Strong association with aortic coarctation and endocardial fibroelastosis.

78
Q

Hypoplastic left heart (Associations) (2)

A

Strong association with aortic coarctation and endocardial fibroelastosis.

79
Q

Cor Triatriatum Sinustrum (anatomy) (2)

A

Abdnormal pulmonary vein draining into left atrium, with unnecessary fibromuscular membrane, causing subdivision of left atrium.
Causes tri-atrium appearance of heart.

80
Q

Cor Triatriatum Sinustrum (Clinical) (3)

A

Causes unexplained pulmonary HTN in kids.
Acts like mitral stenosis and causes pulmonary oedema.
Fatal within 2 years depending on surgical intervention.

FRCR 2A (Crack The FRCR 2A) (55 decks)

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