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1
Q

What is an atrial septal defect?

A

Birth defect in which there is a hole in the atrial septum.

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2
Q

Clinical features of an atrial septal defect, what murmurs are seen?

A

Often asymptomatic

Recurrent chest infections

Arrhythmias in later life

Ejection systolic murmur, loudest at the left upper sternal edge

Fixed and widely split second heart sound

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3
Q

Investigations of an atrial septal defect

A

Bedside

ECG

Imaging

CXR (may show evidence of heart failure and cardiomegaly)

Echocardiogram

May be done using bubble contrast to better visualise the defect

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4
Q

Management of an atrial septal defect

A

Often do not require treatment if the defect is small

Percutaneous closure of defect

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5
Q

What is an AVSD? Who is it commonly seen in?

A

Congenital heart defect in which there is an abnormal connection between the atria and the ventricles.
Commonly associated with Down syndrome

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6
Q

What does an AVSD present with?

A

Often asymptomatic
Symptoms of heart failure

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7
Q

Investigations of an AVSD

A

Bedside
ECG
Imaging
Echocardiogram

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8
Q

Management of an AVSD

A

Medical management of heart failure
Surgical closure

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9
Q

What is coarctation of the aorta? Who is it commonly associated with?

A

Congenital defect characterised by narrowing of the aorta.
Commonly associated with Turner syndrome

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10
Q

Cardiac defect in Down’s

A

AVSD

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10
Q

Cardiac defect in Turner’s

A

Coaractation of aorta

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11
Q

Clinical features of coarctation of aorta

A

Asymptomatic
Secondary cause of hypertension (should be considered in any young patient presenting with hypertension)
Radio-femoral delay
If severe, can cause heart failure and circulatory collapse

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12
Q

What diagnosis should be considered in any young patient with hypertension?

A

Aortic coarctationIn

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13
Q

Investigations of aortic coarctation

A

Bedside
ECG (features of left ventricular hypertrophy)
Imaging
CXR (rib notching)

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14
Q

ECG sign of aortic coarctation

A

LVH

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15
Q

CXR findings of aortic coarctation

A

Rib notchingM

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16
Q

Management of aortic coarctation

A

Often conservative if asymptomatic
May require surgical resection or stenting if severe

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17
Q

What is congenital complete heart block? what is it often assocaited with?

A

A congenital condition in which the electrical impulses from the atria does not transmit to the ventricles.
Often associated with maternal autoimmune disorders (e.g. SLE) and, in particular, the presence of anti-Ro and anti-La antibodies

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18
Q

Clinical features of congenital complete heart block

A

Bradycardia
Pale
Features of heart failure (e.g. shortness of breath)
Reduced exercise tolerance

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19
Q

Investigations of congenital complete heart block

A

Bedside
ECG
Imaging
Echocardiogram

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20
Q

Management of congenital complete heart block

A

Endocardial pacemaker insertion if symptomatic

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21
Q

What is Eisenmenger syndrome? Give examples of which conditions this can occur in

A

Serious complication of congenital heart disease characterised by reversal of a pre-existing left-to-right shunt. It has a very poor prognosis.

Causes of left to right shunt are PDA, ASD, VSD

THINK: 3 letters

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22
Q

Pathophysiology of Eisenmenger syndrome

A

In patients with a VSD or ASD, the left side of their heart will initially be stronger than the right, so blood from the left side will be shunted in to the right side.
This does not cause any major acute complications, however, it does lead to increased right heart pressures.
This, over time, will lead to right ventricular hypertrophy.
Eventually, the pressures generated by the right heart will exceed the pressures generated by the left heart resulting in reversal of the shunt. This will lead to cyanotic heart disease over time.

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23
Q

Clinical features of Eisenmenger syndrome

A

Cyanosis
Features of heart failure
Arrhythmia

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24
Investigations of Eisenmenger syndrome
Bedside ECG Evidence of right ventricular hypertrophy Imaging Echocardiogram Cardiac catheterisation
25
What may ECG and echo show in eisenemnger syndrome?
RVH
26
Management of eisenmenger syndrome
Difficult to treat once it is established The only curative option is a heart-lung transplant
27
What is hypoplastic left heart syndrome?
Congenital heart defect characterised by underdevelopment of the left side of the heart resulting in features of left heart failure soon after birth.
28
Clinical features of hypoplastic left heart syndrome, why do these occur?
Cardiovascular collapse when the duct closes This is because there is little output coming from the left ventricle, therefore, the right ventricle and the ductus arteriosus are responsible for maintaining the systemic circulation.
29
Management of hypoplastic left heart syndrome
Surgical correction (Norwood procedure)
30
Norwood procedure
Surgical correction of hypoplastic left heart syndrome
31
What is an interrupted aortic arch? What is it associated with?
Rare congenital heart defect in which there is no connection between the section of the aorta on either side of the ductus arteriosus Associated with 22q11.2 deletion syndrome (DiGeorge Syndrome)
32
Clinical features of interrupted aortic arch
Features of heart failure and cardiogenic shock in the neonatal period Features of DiGeorge syndrome (hypocalcaemia, palatal defects)
33
Cardiac defect in DiGeorgre syndrome
Interrupted aortic arch
34
Management of interrupted aortic arch
Prostaglandin infusion to maintain the ductus arteriosus Surgical correction
35
What is patent ductus arteriosus?
Persistence of the ductus arteriosus (connecting the pulmonary artery to the aorta) after birth. It normally closes within 4 hours of birth.
36
RFs for patent ductus arteriosus
Prematurity Maternal rubella
37
Clinical features of patent ductus arterious
Often asymptomatic and incidentally noted during routine neonatal examination Can lead to features of heart failure (shortness of breath resulting from pulmonary oedema)
38
Management of PDA
Pharmacological Closure --> NSAIDs (e.g. IV Indomethacin, ibuprofen) Surgical ligation or percutaneous catheter-guided closure
39
What is pulmonary stenosis? How does it usually present?
Narrowing of the pulmonary valve Usually asymptomatic Ejection systolic murmur Evidence of right ventricular hypertrophy
40
Whaat mrumur is heard in PDA?
Gibson murmur --> continous, machine like
41
What murmur is heard in ASD?
ESM + fixed S2 splitting
42
Investigations for pulmonary stenosis
Bedside ECG (right ventricular hypertrophy) Imaging CXR Echocardiogram
43
Management of pulmonary stenosis
Transcatheter balloon dilatation
44
Criteria for Rheumatic Fever
Jones
45
What is in the criteria for Rheumatic fever?
46
What organism causes rheumatic fever?
Step pyogenes (group A strep)
47
Investigations for rheumatic fever
Bedside ECG Bloods CRP ESR ASO Titre Imaging Echocardiogram
48
Management of rheumatic fever
Antibiotics 1stLine: Benzathine Benzylpenicillin Alternative: Phenoxymethylpenicillin, Erythromycin Arthritis NSAIDs and Aspirin Cardiac Complications Anti-arrhythmics ACE inhibitors Diuretics Chorea Anticonvulsants
49
What antibiotics are used in rheumatic fever?
1stLine: Benzathine Benzylpenicillin Alternative: Phenoxymethylpenicillin, Erythromycin
50
How is arthritis in rheumatic fever treated?
NSAIDs and Aspirin
51
How are cardiac complications in rheumatic fever treated?
Anti-arrhythmics ACE inhibitors Diuretics
52
How is chorea in rheumatic fever treated?
Anticonvulsants
53
What are the 3 types of cyanotic heart disease?
ToF TGA TA
54
What are the 3 types of left to right shunt? What type of heart disease do they cause?
PDA, ASD, VSD They cause non-cyanotic heart disease
55
What are the two types of outflow obstruction? what do they cause?
Valve stenosis, aortic coarctation non-cyanotic heart disease
56
What is the most common form of congenital cyanotic heart disease? What is it characterised by?
Tetralogy of Fallot Large VSD Overriding Aorta (caused by VSD) Pulmonary Stenosis Right Ventricular Hypertrophy (caused by pulmonary stenosis)
57
Clinical features of ToF
Infants develop cyanotic episodes often precipitated by exertion (e.g. feeding) - tet spells May take up to 1 month for symptoms to be noted
58
When does cyanosis present in ToF? How does this compare to TGA and TA?
within days to months TGA - hours TA - minutes
59
Tet spells
ToF
60
What murmur heard in ToF
ejection systolic murmur at left sternal edge
61
Investigations in ToF
Bedside ECG (often normal) Imaging CXR (boot-shaped heart) Echocardiogram
62
CXR finding in ToF
boot shaped heart
63
Management of ToF
If the patient is profoundly cyanotic, they should be urgently started on a prostaglandin infusion (this keeps the ductus arteriosus open, thereby allowing blood from the systemic and pulmonary circulation to mix Surgical correction
64
What is TGA?
Congenital cyanotic heart disease in which the main arteries leaving the heart are connected the wrong way around. This means that blood is pumped from the right side of the heart directly into the systemic circulation, and blood from the left side of the heart is pumps back into the pulmonary circulation. In essence, the two circuits are independent, and life is only sustained in utero due to mixing of blood via other connects (e.g. ductus arteriosus, septal defects)
65
Clinical features of TGA
Cyanosis soon after birth (usually within 2 days, as the ductus arteriosus closes) NO murmur heard on auscultation
66
cyanotic heart disease with no murmur
TGA
67
Investigations for TGA
Bedside ECG (often normal) Hyperoxia Test High-flow oxygen is applied to the patient but it fails to improve the saturation measured by the peripheral saturation probe as there is no connection between the pulmonary and systemic circulations. Imaging CXR (egg-on-a-string appearance) Echocardiogram
68
How to assess whether cause is respiratory or cardiac in neonatal cyanosis?
Hyperoxia test Do ABG on heel of baby, check initial pO2 and then give 100% o2 for 10 mins, then another ABG If o2 increases  respiratory  not enough o2 to go into blood, then when supplemented issue resolves If o2 remains low  cardiac issue as even with supplemental o2, can’t get it to peripheries
69
CXR finding in TGA
egg-on-a-string appearance
70
Management of TGA
Acute Management of Cyanosed Infant Promptly start prostaglandin infusion to keep the ductus arteriosus open Balloon atrial sepstostomy may be considered to maintain connection between atria Surgical correction (arterial switch)
71
What is tricuspid atresia? What does it result in?
Congenital heart defect in which the tricuspid valve fails to develop resulting in atrialisation of the right ventricle. NOTE: As there is no connection between the right atrium and the right ventricle, there has to be a ventricular septal defect to allow blood to mix.
72
Clinical features of TA
Cyanosis Shortness of breath
73
Management of TA
Prostaglandin infusion to maintain a patent ductus arteriosus Surgical Intervention Blalock-Taussig shunt (creates connection between subclavian artery and pulmonary artery) Cavopulmonary anastomosis Fontan procedure (redirect IVC and hepatic vein blood flow into the pulmonary circulation)
74
Blalock Taussing shunt
creates connection between subclavian artery and pulmonary artery --> used in surgical management of TA
75
What is VSD? How does it present?
Congenital heart defect in which there is a hole in the ventricular septum. Often asymptomatic Features of heart failure (e.g. shortness of breath) Pansystolic murmur at the lower left sternal edge
76
What murmur in VSD?
Pansystolic murmur at the lower left sternal edge
77
Investigations in VSD
Bedside ECG Often normal May show evidence of left or right ventricular hypertrophy Imaging CXR Often normal but may show cardiomegaly and other changes associated with heart failure Echocardiogram
78
Management of VSD
Defects often close spontaneously Surgical closure of defect

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