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cardiovascular phase2a > Structural heart defects > Flashcards

Structural heart defects Flashcards

1
Q

What type of care issues may arise from congenital heart disease?

A 
Intellectual disability
Psychosocial issues
Transition
Explaining lesion and prognosis
Building independence/self resilience
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2
Q

What complication may occur in later life in people who suffered from tetralogy of Fallot as a child?

A

Pulmonary valve regurgitation - requires surgery to fix

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3
Q

What are the possible causes of congenital heart disease?

A 
Maternal prenatal rubella infection
Maternal alcohol misuse
Maternal drug treatment and radiation
Genetic abnormalities
Chromosomal abnormalities
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4
Q

Which congenital heart diseases would be classes as acyanotic with shunts?

A

Atrial septal defects
Ventricular septal defects
Patent ductus arteriosus
Partial anomalous venous drainage

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5
Q

Which congenital heart diseases would be classed as acyanotic without shunts?

A

Coarctation of aorta

Congenital aortic stenosis

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6
Q

Which congenital heart diseases would be classed as cyanotic with shunts?

A

Fallot’s tetralogy
Transposition of great vessels
Severe Ebstein’s anomaly

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7
Q

Which congenital heart diseases would be classed as cyanotic without shunts?

A

Severe pulmonary stenosis
Tricuspid atresia
Pulmonary atresia
Hypoplastic left heart

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8
Q

What are the symptoms and signs of congenital heart disease?

A 
Central cyanosis
Pulmonary hypertension
Clubbing of fingers
Paradoxical embolism
Polycythaemia
Growth retardation
Syncope
Squatting
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9
Q

How may a congenital heart disease present?

A 
Endocarditis
Progression of valvular lesions
Atrial and ventricular arrhythmias
Sudden cardiac death
Right heart failure
End-stage heart failure
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10
Q

What happens to blood in the heart in ventricular septal defects?

A

Blood moves from high pressure in left side to low pressure in right through defect in septum

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11
Q

What are the haemodynamic consequences of large defects?

A

Pulmonary hypertension and eventual Eisenmenger’s complex

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12
Q

How would a large non-restrictive VSD present?

A

Heart failure symptoms in childhood - LA and LV dilatation due to LV volume overload

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13
Q

What type of murmur is associated with small restrictive VSDs?

A

Loud pan-systolic

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14
Q

What may be seen on CXR and ECG in someone with a large VSD?

A

Prominent pulmonary arteries
Cardiomegaly
LVH or RVH on ECG

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15
Q

What type of intervention is used for VSDs?

A

Surgical patch repair

Device closure if isolated muscular VSD

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16
Q

What are the indications for intervention in VSD?

A

LA and LV enlargement with/without early LV dysfunction
Reversible pulmonary hypertension with residual L-R shunt and no significant desaturation with exercise
Infective endocarditis

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17
Q

What are the 3 main types of atrial septal defects and where would they be found?

A 
Sinus venosus defects - superior/inferior part of septum near SVC/IVC
Ostium secundum defects - mid-septum
Ostium primum (AV septal) defects - lower part of atrial septum
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18
Q

What symptoms would occur in an adult with an unprepared ASD?

A

Dyspnoea and exercise intolerance
May develop atrial arrhythmias from RA dilatation
Increased pulmonary vascular flow

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19
Q

What investigations would be carried out for ASD and what would they show?

A

CXR - prominent pulmonary arteries
ECG - RBBB and right axis deviation (left deviation in ostium primum)
Echocardiography - hypertrophy and dilatation of right heart and pulmonary arteries
CMR and CT

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20
Q

How would you calculate the left to right shunt in ASDs?

A

Subcostal view with 2D and colour Doppler on echocardiography

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21
Q

What are the indications for intervention in ASDs?

A

ASD with significant L-R shunting > RA/RV enlargement - should be closed irrespective of symptoms
Thromboembolic events, including patients with patent foramen ovale

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22
Q

What type of intervention can be used for ASDs?

A

Device closure using transcatheter clamshell device for secundum ASDs
Surgical closure for all other types

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23
Q

What problem occurs in patent ductus arteriosus?

A

Failure of ductus arteriosus to close after birth > persistent communication between proximal left pulmonary artery and descending aorta > continuous L-R shunt

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24
Q

What drug can be given in patent ductus arteriosus to stimulate the closure of the ductus arteriosus?

A

Indometacin (prostaglandin inhibitor)

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25
Q

What are the characteristic clinical signs of PDA?

A

Bounding pulse and continuous ‘machinery murmur’

As pulmonary hypertension develops, murmur becomes softer

26
Q

What investigations should be done in PDA and what would they show?

A

CXR - prominent aorta and pulmonary arterial system
ECG - LA abnormality and LVH
Echocardiography - dilated LA and LV with right heart changes occurring late

27
Q

What are the indications for intervention in PDA?

A

LV dilatation

Mild to moderate pulmonary arterial hypertension

28
Q

What intervention would be used for PDA?

A

Percutaneous devices

29
Q

Small PDA defects may predispose to…

A

Endarteritis

30
Q

What is coarctation of the aorta?

A

Narrowing of the aorta at or just distal to insertion of ductus arteriosus

31
Q

Which syndrome is associated with coarctation of the aorta?

A

Turner’s syndrome

32
Q

In 80% of cases of coarctation of the aorta, what is wrong with the aortic valve?

A

Bicuspid and potentially stenotic or endocarditic

33
Q

What conditions are associated with coarctation of the aorta?

A

Patent ductus arteriosus
Ventricular septal defect
Mitral stenosis or regurgitation
Circle of Willis aneurysm

34
Q

What symptoms may be present in coarctation of the aorta?

A

Headaches and nosebleeds due to hypertension

Claudication and cold legs due to poor blood flow

35
Q

What would be found on physical examination in someone with coarctation of the aorta?

A

Hypertension in upper limbs and weak, delayed pulses in legs (radiofemoral delay)
Murmur
Right arm hypertension
Buzzes over scapula and back from collateral vessels

36
Q

When may asynchronous radial pulses in left and right arms be seen?

A

Coarctation of the aorta proximal to left subclavian

37
Q

What investigations would be done for coarctation of the aorta and what would be found?

A

CXR - dilated aorta indented at site of coarctation, rib notching
ECG - LVH
Echocardiography - coarctation and associated abnormalities
CT and CMR - demonstrate coarctation and quantify flow

38
Q

When would intervention be required in someone with coarctation of the aorta?

A

Peak-peak gradient across coarctation >20mmHg and/or proximal hypertension

39
Q

What intervention is preferred by most centres for coarctation of the aorta in neonates as well as older children and adults?

A

Surgical repair

40
Q

What alternative intervention can be used for older children and young adults for coarctation of the aorta?

A

Balloon dilatation and stenting

41
Q

What does Fallot’s tetralogy consist of?

A

Large mal-aligned VSD
Overriding aorta
RV outflow tract obstruction
RVH

42
Q

When would cyanosis develop in Fallot’s tetralogy?

A

Increased right sided pressure causing right to left shunt

43
Q

What are Fallot’s spells and how are they relieved?

A

Episodes of severe cyanosis in children due to spasm of sub pulmonary muscle; relieved by increasing systemic resistance by squatting

44
Q

What happens to the heart in complete transposition of great arteries?

A

RA connects to morphological RV > aorta
LA connects to morphological LV > pulmonary artery
Blood circulates in two parallel circuits: deoxygenated blood > systemic; oxygenated blood > lungs

45
Q

What procedure is carried out in babies as soon as they are born with TGA?

A

Atrial septostomy: Rashkind’s balloon used to dilate foramen ovale and main saturations at 50-80% until definitive procedure can be performed

46
Q

What procedure is performed within the first 2 weeks of a baby’s life with TGA?

A

Arterial switch procedure - aorta reconnected to LV and pulmonary artery connected to RV; coronary arteries reimplanted

47
Q

What limits the life expectancy in people with TGA?

A

Eventual failure of systemic RV

48
Q

What happens in congenitally corrected TGA?

A

Systemic venous return to RA enters morphological LV > pulmonary artery
Pulmonary venous blood returns to LA then enters morphological RV > aorta

49
Q

What is ccTGA often associated with?

A 
Cardiac lesions
Systemic (tricuspid) AV valve abnormalities with valve insufficiencies
VSD
Sub-pulmonary stenosis
Complete heart block
Wolff-Parkinson-White syndrome
Dextrocardia
50
Q

What interventions may some people require if they have ccTGA?

A

Pacemaker

Surgery for regurgitant tricuspid

51
Q

What would be seen in a child with a large VSD?

A

High pulmonary blood flow
Breathless
Poor feeding
Failure to thrive

52
Q

What are the clinical signs of a large VSD?

A 
Small breathless skinny baby
Increased respiratory rate
Tachycardia
Cardiomegaly
Murmur varies in intensity
53
Q

What are the clinical signs of a small VSD?

A

Loud systolic murmur
Thrill (buzzing sensation)
Well grown
Normal heart size/rate

54
Q

What are the clinical signs of ASD?

A

Pulmonary flow murmur
Fixed split second heart sound
Big pulmonary arteries on CXR
Cardiomegaly on CXR

55
Q

What symptoms and signs would be seen in someone with a complete AV septal defect?

A 
Breathless as neonate
Poor weight gain
Poor feeding
Torrential pulmonary blood flow
Needs repair or PA band in infancy
56
Q

What symptoms and signs would be seen in someone with a partial AV septal defect?

A

Presents like small VSD/ASD

Can present in late adulthood

57
Q

What symptoms and signs would be seen in someone with a large PDA?

A

Torrential flow from aorta to pulmonary arteries in infancy

Breathless, poor feeding, failure to thrive

58
Q

What symptoms and signs would be seen in someone with a small PDA?

A

Usually asymptomatic

Murmur found incidentally

59
Q

What are some of the long-term problems that can arise from coarctation of the aorta?

A

Hypertension - early coronary artery disease, early strokes, sub arachnoid haemorrhage
Re-coarctation requiring repeat intervention
Aneurysm formation at site of repair

60
Q

What are the clinical signs of severe pulmonary stenosis?

A 
Right ventricular failure as neonate
Collapse
Poor pulmonary blood flow
RVH
Tricuspid regurgitation
61
Q

What treatment would be used for pulmonary stenosis?

A

Balloon valvuloplasty
Open valvotomy
Open trans-annular patch
Shunt (to bypass blockage)

62
Q

Describe what happens in Eisenmenger’s syndrome

A

High pressure pulmonary blood flow > damages to delicate pulmonary vasculature > resistance to blood flow through the lungs increases > RV pressure increases > shunt direction reverses > cyanosis

cardiovascular phase2a (14 decks)

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