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Cardiology Flashcards

1
Q

Why are shunts in place in fetal circulation?

A

Blood needs to go via placenta to collect oxygen and nutrients, dispose of carbon dioxide and lactate via the mother.

Blood does not pass through the pulmonary circulation.

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2
Q

What are the 3 fetal shunts present?

A

Ductus venosus - umbilical vein to inferior vena cava, bypassing the liver

Foramen ovale - right atrium to left atrium, bypassing the right ventricle and pulmonary circulation

Ductus arteriosus - pulmonary artery with aortia, bypass pulmonary circulation

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3
Q

What happens to fetal circulation at birth?

A

The first breath expands the alveoli, decreasing pulmonary vascular resistance.

Decrease in resistance causes fall in pressure in right atrium.

LA pressure now greater than the right atrium, squashing the atrial septum and closure of foramen ovale.

Then becomes sealed shut and becomes fossa ovalis.

Increased blood oxygenation causes drop in PGs - which are needed to keep ductus arteriosus open so this shuts - becomes ligamentum arteriosum.

Ductus venosus stops functioning because the umbilical cord is clamped and there is no flow in the umbilical veins - becomes ligamentum venosum.

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4
Q

Describe the path of the fetal circulation

A

Oxygenated blood from the placenta enters umbilical vein, bypasses liver by DV

Then enters vena cava to the heart, bypasses pulmonary circulation via FO into the left atrium, blood that does enter the pulmonary artery passes through DA to re-enter systemic circulation

Deoxygenated blood returns to placenta via umbilical arteries that branch off from internal iliac.

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5
Q

What are the acyanotic heart defects?

A

Left to Right shunts

ASD
VSD
Coarctation of the Aorta
Patent Ductus Arteriosus
Pulmonary stenosis
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6
Q

How do acyanotic defects present?

A

Most are picked up on antenatal screening
Asymptomatic mostly

Possibly
Fail to thrive
Resp infections
Heart failure

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7
Q

Why can you see heart failure with acyanotic heart defects?

A

Increased pressure in pulmonary circulation

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8
Q

What is Eisenmenger’s syndrome?

A

L –> R shunt swap to R –> L if pulmonary pressure rise to exceed systemic pressure

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9
Q

How are acyanotic heart defects commonly investigated?

A

Echo - flow and anatomy
ECG - identity cardiomegaly or pulmonary hypertension
Cardiac catheter - if severe

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10
Q

How would you see cardiomegaly or pulmonary hypertension on ECG?

A

Commonly Left Ventricular hypertrophy - lead I increase, lead III invert

Right ventricular hypertrophy, right axis deviation, p pulmonale, tall R in V1, right ventricular strain

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11
Q

How are acyanotic heart defects managed in the general term?

A

Loop diuretics if heart failure

Surgical closure of defect

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12
Q

What are the risk factors for developing an acyanotic heart defect?

A

Family history
Maternal smoking/toxins/infection/diabetes
Genetic conditions - Down’s

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13
Q

What are innocent murmurs?

A

Flow murmurs, very common in children

Caused by fast blood flow through various areas of the heart during systole

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14
Q

What are the features of innocent murmurs?

A 
Soft
Short
Systolic
Symptomless
Situation dependent - gets quieter with standing, or only appears when unwell
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15
Q

When may a heart murmur in a child need to be investigated?

A

Murmur louder than 2/6 - soft heard in all positions no thrill
Diastolic murmurs
Louder on standing
Other symptoms such as failure to thrive, feeding difficulty, cyanosis or shortness of breath

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16
Q

What are the key investigations to establish the cause of a murmur?

A

ECG
CXR
Echo

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17
Q

What are the differentials for a pan-systolic murmur?

A

Mitral regurgitation
Tricuspid regurgitation
Ventricular septal defect heard at the left lower sternal border

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18
Q

What are the differentials of ejection-systolic murmurs?

A

Aortic stenosis
Pulmonary stenosis
Hypertrophic obstructive cardiomyopathy heard loudest at the fourth intercostal space on the left sternal border

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19
Q

What causes splitting of the second heart sound?

A

Inspiration - diaphragm pulls lungs and heart open, creating negative intra-thoracic pressure
This causes the right side of heart to fill faster, increased volume in right ventricle means it takes longer to empty - delay in pulmonary valve to close.
Pulmonary valve closes later than aortic valve - split sound.

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20
Q

What murmur is heard in an atrial septal defect?

A

Mid-systolic crescendo-decrescendo murmur
Loudest at upper left sternal border
Fixed split second heart sound

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21
Q

What murmur is heard in PDA?

A

May not cause any abnormal heart sounds

More significant - normal first heart sound, and continuous crescendo-decrescendo murmur during second heart sound

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22
Q

What murmur is heard in tetralogy of fallot?

A

Arises from pulmonary stenosis
Ejection systolic murmur
Loudest at pulmonary area - second intercostal space, left sternal border

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23
Q

Why do patients with transposition of the great arteries always have cyanosis?

A

Because the right side of the heart pumps blood directly into the aorta and systemic circulation

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24
Q

What is the most common cause of common mixing? (breathless and blue)

A

Atrioventricular septal defect

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25
Q

What causes a child to be asymptomatic with a murmur?

A

Innocent murmur

Outflow obstruction in a well child - pulmonary stenosis or aortic stenosis

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26
Q

What are the causes of a collapsed child with shock - outflow obstruction in a sick neonate?

A

Coarctation of the aorta

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27
Q

What cardiac abnormalities result from rubella infection?

A

Peripheral pulmonary stenosis

PDA

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28
Q

What chromosomal abnormalities can cause cardiac abnormalities?

A

Down’s - ASD, VSD
Edwards
Patau
Turner’s - aortic valve stenosis, coarctation
Noonan syndrome - hypertrophic cardiomyopathy, ASD, pulmonary valve stenosis

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29
Q

What is the presentation of congenital heart disease?

A 
Antenatal cardiac USS diagnosis
Detection of a heart murmur
Heart failure
Shock
Cyanosis
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30
Q

Why might a child with a potential shunt have no symptoms or murmur at birth?

A

Pulmonary vascular resistance is still high

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31
Q

What are the symptoms and signs of heart failure?

A 
Symptoms:
Breathlessness
Sweating
Poor feeding
Recurrent chest infections
Signs
Poor weight gain
Tachypnoea
Tachycardia
Heart murmur, gallop rhythm
Enlarged heart
Hepatomegaly
Cool peripheries
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32
Q

What are the causes of heart failure?

A 
Neonates:
Hypoplastic left heart syndrome
Critical aortic valve stenosis
Severe coarctation of aorta
Interruption of aortic arch

Infants - high pulmonary blood flow
Ventricular septal defect
Atrioventricular septal defect
Large persistent ductus arteriosus

Older children and adolescents
Eisenmenger syndrome - right heart failure
Rheumatic heart disease
Cardiomyopathy

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33
Q

What is the most likely cause of heart failure in the first week of life?

A

Left heart obstruction e.g. coarctation

If the lesion is v severe, then arterial perfusion may be duct-dependent systemic circulation.

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34
Q

What is the most likely cause of heart failure after the first week of life?

A

Left to right shunt
During subsequent weeks, pulmonary vascular resistance falls meaning progressive left to right shunt causing pulmonary oedema and breathlessness
If left untreated will develop Eisenmenger syndrome and shunt now right to left, pt is blue.

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35
Q

What are the causes of cyanosis in a newborn infant with respiratory distress? >60 breaths/min

A

Cardiac disorders - cyanotic congenital heart disease
Respiratory disorders e.g. surfactant def, meconium aspiration
PPHN persistent pulmonary hypertension of the newborn; failure of pulmonary vascular resistance to fall after birth
Infection - sepsis from Group B strep
Metabolic disease - acidosis and shock

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36
Q

When does congenital heart disease present with shock?

A

When duct closes in severe left heart obstruction

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37
Q

What are examples of left to right shunts?

A

Atrial septal defects
Ventricular septal defects
Persistent ductus arteriosus

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38
Q

What is an ASD?

A

Occurs when the septum between left and right atrium is not formed completely
Pressure in LA is greater so oxygenated blood from LA forced through ASD to RA, so acyanotic

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39
Q

What is the pathophysiology of ASD?

A

Two separate endocardial cushions form the atrial septum. Space between is foramen ovale. Does not close.

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40
Q

What are the five types of ASD?

A 
Patent foramen ovale
Ostium secundum defect
Ostium primum defect
Sinus venosus defect
Coronary sinus defect
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41
Q

What causes ostium secundum defect?

A

Incomplete occlusion of ostium secundum by septum secundum, or septum primum reabsorbed too much

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42
Q

What causes ostium primum?

A

Septum primum fails to fuse with endocardial cushions

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43
Q

What are the 2 types of ostium primum defects?

A

Complete AVSD

Partial AVSD - just the ostium primum, intact ventricular septum

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44
Q

What are the types of sinus venosus defects?

A

Superior - superior vena cava opening runs on top of oval fossa
Inferior

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45
Q

What are the risk factors for ASDs?

A 
Autosomal dominance with ostium secundum
Family history
Maternal smoking 1st trimester
Maternal diabetes
Maternal rubella
Maternal drug use e.g. cocaine and alcohol
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46
Q

What are the symptoms of large ASDs in paeds?

A

Vast majority smaller ones are asymptomatic

Tachypnoea
Poor weight gain
Recurrent chest infections

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47
Q

What are the symptoms of untreated large ASDs in adults?

A 
Exercise intolerance
Palpitations
Recurrent chest infections
Fatigue
Syncope
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48
Q

What is heard on auscultation in ASD?

A

Murmur - soft, systolic ejection, best heard over pulmonary region 2nd ICS
Wide fixed split S2
Diastolic rumble in lower left sternal edge if large

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49
Q

What are the differentials for ASD?

A

Atrioventricular septal defect
Ventricular septal defect
Innocent murmur
Pulmonary stenosis murmur - more turbulent

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50
Q

What are the investigations for ASD?

A

Bedside:
ECG usually normal
In large ASD - tall P wave, right bundle branch block, right axis deviation

Imaging:
Transthoracic echo - provides info on size of ASD and direction of flow

Cardiac MRI and CT (not usually imaging of choice due to radiation)
MRI can measure blood flow ratio

CXR can show cardiomegaly, usually normal in children with small shunts

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51
Q

What is the initial management for ASD?

A

Conservative:
If <5mm, spontaneous closure should occur within 12 months of birth

In adults, if no signs of right heart failure and small defect, echo every 2-3 yrs

If arrhythmia - control rhythm with drugs and anticoagulate before surgical treatment

Medical:
With heart failure, diuretics
Endocarditis prophylaxis not currently required

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52
Q

What is the definitive management for ASD?

A

Surgical closure >1cm

Percutaneously via transcatheter or open chest using bypass

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53
Q

What are the complications of percutaneous closure of ASD?

A

Arrhythmias
Atrioventricular block
Thromboembolism - VTE aspirin

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54
Q

What are the indications for surgical closure of an ASD?

A

TIA/stroke
Ostium primum defects
Sinus venous defects
Coronary sinus defects

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55
Q

What are the complications of untreated large ASDs?

A 
Arrhythmias - caused by atrial stretch leading to abnormal foci developing
Pulmonary hypertension
Eisenmenger syndrome
Cyanosis if Eisenmenger
Peripheral oedema
TIA/stroke
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56
Q

What is the presentation of Eisenmenger syndrome?

A 
Chronic cyanosis
Exertional dyspnoea
Syncope
Increased risk of infections
Increased pulmonary vascular resistance
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57
Q

Give 4 causes of acyanotic heart disease?

A
  • atrial +/- ventricular septal defects
  • PDA
  • aortic stenosis
  • pulmonary valve stenosis
  • coarctation of the aorta
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58
Q

Give 6 differentials for a blue baby

A

central: CO poisioning, sepsis, polycythameia, congenital heart disease
resp: penumonia, pneumonthorax, ARDS, pulmonary atresia, foreign body inhalation
Neuro: asphyxia, seizures, sedatives

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59
Q

What could cause a systolic murmur in a child? (4)

A
  • non pathological, innocent murmur
  • VSD
  • outflow tract obstruction
  • mitral regurg
  • PDA
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60
Q

what could cause a diastolic murmur in a child? (3)

A
  • venous hum (non pathological)
  • Aortic regurg
  • Atrial septal defect
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61
Q

What is tetralogy of fallot?

A
  • VSD + pulmonary stenosis
  • leads to ventricular hypertrophy and overriding aorta
  • blood flows mostly into aorta and baby relies on PDA to get blood to lungs
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62
Q

How will a child with a mild (“pink”) TOF present?

A
  • asymptomatic at birth as plenty blood can still get out pulmonary artery
  • heart failure will progress as they grow and the heart cannt meet demands
  • usually presents age 1-3 with odema, SOB, fatigue, failure to thrive, basal crackles, hepatomegaly, murmur
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63
Q

how may a moderate- severe TOF present?

A
  • moderate presents with resp distress or cyanosis in first few weeks as the ductus arteriosus starts to close
  • severe TOFs with pulmonary atresia is usually detected antenatally, if not theyll present with resp distress/ cyanosis within the first few hrs of life
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64
Q

What are the risk factors for TOF?

A

Males
1st degree family history

Teratogens - alcohol, warfarin, trimethadione (antiepileptic drug used in treatment resistant epilepsy)

Genetics
CHARGE syndrome
Di George syndrome

Associated congenital defects

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65
Q

What is perimembranous VSD?

A

When VSD involves parts of membranous and muscular septum

Commonest type associated with TOF

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66
Q

Why might a patient be acyanotic with VSD?

A

Significant size VSD, causes systolic pressures between the ventricles to equalise
In mild TOF, the left ventricular pressures remain higher than the right, so blood shunts from left to right

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67
Q

Why might a patient be cyanotic with VSD?

A

More severe disease
Increased right ventricular pressure - secondary to PS
The shunt direction reverses from right to left allowing mixture of deoxygenated and oxygenated blood
so cyanotic

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68
Q

Where is the most common site of pulmonary stenosis?

A

Infundibular septum

right ventricular outflow tract

69
Q

What occurs in TOF as a result of pulmonary stenosis?

A

Impaired flow of deoxygenated blood into main pulmonary artery
May be severe enough to cause intermittent RVOT obstruction
Causes hypoxic episodes - tet spells

70
Q

What is the boot sign in TOF?

A

Seen on CXR
Develops in utero
Hypertrophy of the right ventricle due to high pressures to pump deoxygenated blood through the RVOTO

71
Q

What is the presentation of extreme TOF?

A

Pulmonary atresia or
Absent pulmonary valves

These are true duct dependent lesions - only way deoxygenated blood can flow into lungs is through patent ductus arteriosus

If not discovered in antenatal scans, presents in first few hours of life with resp distress, cyanosis

72
Q

What are tet spells?

A

Hypoxic spells
Peak age of incidence between 2-4 months of life

Paroxysm of hyperpnoea - rapid deep respirations, right to left shunting so CO2 accumulates driving the central resp centre

Irritability - prolonged, unsettled crying

Increasing cyanosis

73
Q

What can be found in TOF on examination?

A

Central cyanosis, clubbing

Palpation - thrill or heave (RVH)

Auscultation
Loud single S2 - due to closure of aortic valve in diastole

Pansystolic murmur - smaller the VSD the louder the murmur

Ejection click due to presence of dilated aorta

Continuous machinery murmur in the presence of PDA with extreme forms of TOF, especially those on PG infusion

Signs of congestive heart failure - sweating, pallor, tachycardia, hepatosplenomegaly, generalised oedema, bilateral basal crackles or gallop rhythm

74
Q

What are the differentials for TOF?

A

Other cyanotic CHD
- critical PS, transposition of great arteries, hypoplastic left heart syndrome

Isolated VSD - does not cause cyanosis as shunt is left to right, unless there is Eisemenger syndrome

Sepsis - any resp distress and hypoxia

75
Q

What are the investigations for TOF?

A

Bedside - ECG showing right axis deviation, RVH

Bloods - microarray if genetic syndromes suspected

Radiological -
CXR - boot shaped heart, reduced pulmonary vascular marking (decrease pulmonary blood flow)

Echo
Cardiac CT angiogram
Cardiac MRI

Interventional radiology with cardiac catheter to measure haemodynamics

76
Q

What is the medical management of TOF?

A

Squatting/knees to chest - helps venous return, increases systemic resistance

PG infusion - helps maintain PDA if more severe form.
Either alprostadil or dinoprostone

Beta-blockers - propranolol used in tet spells, reduces the heart rate

Morphine - reduces resp drive, reduces hyperpnoea

Saline 0.9% bolus
Used in tet spells as a volume expander to increase pulmonary blood flow

77
Q

What is the surgical management of TOF?

A

Palliative
Transcatheter RVOT stent to relieve RVOTO, buys time until child is bigger

Modified Blalock-Taussig shunt mimics as PDA, increases pulmonary blood flow before definitive repair

Insertion of RV to PA conduit or PA banding

Definitive repair
Cardiopulmonary bypass via median sternotomy; RVOT resection, augmentation, VSD patch closure

78
Q

What are the complications of TOF?

A 
Polycythaemia
Cerebral abscess
Stroke
Infective endocarditis
Congestive cardiac failure
Death - up to 25% in 1st year of life

Pulmonary regurgitation, arrhythmias, exercise intolerance, sudden death even post corrective surgery

79
Q

What is required for follow up for TOF?

A

Regular ECG
ECHO
Cardiopulmonary exercise testing in exercise tolerance

80
Q

What are the side effects of prostaglandin infusions?

A
  • apnoeas
  • bradycardia
  • hypotension
81
Q

What is AVSD?

A

Defect of atrioventricular septum and abnormalities of the AV valves - mitral and tricuspid valves

82
Q

What are the types of AVSDs?

A

Partial AVSD

Complete AVSD

83
Q

What is the epidemiology of AVSD?

A

Strong association with Down’s
Complete can occur with heterotaxy syndromes - abnormal arrangement of internal thoracic-abdominal organs across axis of body

84
Q

What is the aetiology of AVSD?

A

Normally primitive AV canal connects atria to ventricles
At 4-5 wks gestation, superior and inferior endocardial cushions fuse

Defects due to failure of endocardial cushions to fuse correctly

Complete failure to fuse causes an ASD (primum atrial septal defect) and VSD (ventricular septal defect) and a single common atrio ventricular valve

85
Q

What occurs in complete AVSD?

A

Increased shunting of blood from left to right side of heart
Pulmonary vascular resistance decreases normally over first 6 weeks, patient develops large left to right shunt
Causes excessive pulmonary blood flow, heart failure, elevated pulmonary vascular resistance
Incompetent abnormal valve, regurgitation

86
Q

What occurs in partial AVSD?

A

Left to right shunting
Volume overload of right atrium and right ventricle
Pulmonary artery pressures remain normal
So symptoms may be minimal

87
Q

What structural changes may occur in AVSDs?

A

Increased distance between aorta and apex of the heart
Results in elongation of left ventricular outflow tract
Aortic valve displaced anterosuperior - goose neck deformity

88
Q

What are the clinical features of AVSD?

A 
Tachypnoea
Tachycardia
Poor feeding
Sweating 
Failure to thrive - due to excessive metabolic requirements and poor calorie intake
89
Q

What can be observed on examination in AVSD?

General
Inspection
Palpation
Auscultation

A

Undernourished child
May have characteristics of Down’s
Congestive HF - hepatomegaly, gallop rhythm, oedema, crackles

Inspection - pallor, Harrison grooves

Palpation -
Hyperactive precordium
Prominent systolic heave
Palpable apical thrill

Auscultation
Complete:
Accentuated S1, loud S2
Ejection systolic murmur
Mid-diastolic murmur
Holosystolic murmur
Partial:
Wide and fixed splitting SW, does not change w inspiration
Ejection systolic murmur
Mid-diastolic murmur
Holosystolic murmur
90
Q

What are some differentials for AVSD?

A

Isolated atrial septal defect
Isolated ventricular septal defect
Patent ductus arteriosus - machinery murmur throughout systole and diastole
Paediatric heart failure
Sepsis
Causes of poor weight gain, failure to thrive e.g. malabsorption, nutritional deficiencies

91
Q

What are the investigations for AVSD?

A

Bloods - blood sample for karyotyping e.g. for Down’s

ECG - complete AVSD
Superior QRS axis
Prolonged PR interval due to atrial enlargement
P wave morphology
Right ventricular hypertrophy

Radiology - CXR cardiomegaly, pulmonary artery prominent

ECHO

92
Q

What is the medical management for AVSD?

A

For symptomatic relief of heart failure, buys time for child to grow, gain optimum weight before corrective surgery

Diuretics - furosemide loop diuretic to relieve pulmonary congestion, reduces preload
Can cause hypokalaemia so spironolactone added

ACEi - captopril, reduces systemic vascular resistance, reduces afterload reduction, more blood through left ventricular outflow tract and less in left to right shunt
Can increase K so stop spironolactone with this

Digoxin - acts on cardiac muscle, increases systolic contraction, increases inotropy without increasing oxygen consumption, slow HR

Adequate caloric intake
High energy formula feeds, NG tube if cannot tolerate oral

93
Q

What is the usual approach for management of heart failure with an AVSD?

A

Start with diuretic and afterload reducing agent

Digoxin later added if further improvement needed

94
Q

What is the surgical management of AVSD?

A

Complete AVSD needs corrective surgery; performed prior to development of irreversible pulmonary vascular obstructive disease, median age of surgery 3-6 months

Palliative surgery -
Pulmonary artery banding, decreases diameter and reduces the pulmonary blood flow to relieve symptoms e.g. if premature, low birth weight, allow time to mature

Corrective surgery -
Via median sternotomy under cardiopulmonary bypass

Closure of atrial and ventricular communications, construct two separate and competent AV valves from available leaflet tissue

Single patch to close ASD and VSD, double patch, modified patch

95
Q

What are the complications of untreated AVSD?

A 
Failure to thrive
Recurrent lower resp tract infections
Congestive heart failure
Pulmonary vascular disease
Eisenmenger's syndrome
96
Q

What complications may occur following AVSD surgical repair?

A

Left AV valve regurgitation, due to inadequate surgical reconstruction
Residual shunt
Arrhythmias
Sinus node dysfunction - results in bradycardia
Wound infection

97
Q

What is a VSD?

A

Hole in the septum separating the left and right ventricles

Can occur as isolated lesion or alongside other CHDs

98
Q

What occurs in a small VSD?

A

Restrictive VSD - blood flow through is minimal, so no significant increase in pulmonary blood flow
Asymptomatic

99
Q

Why do most patients with VSD experience symptoms?

A

Due to increased flow of blood through pulmonary circulation

Pressure in left ventricle greater than right so shunting is from left to right

100
Q

What symptoms are seen in a moderate-sized VSD?

A

Flow of blood through VSD is great enough to cause significant increase in blood flow through pulm circ.
Shunt occurs in systole, so blood pumped directly into pulm circ and does not affect right ventricle
Dilatation of left atrium and ventricle because receiving large vol of blood, so can cause heart failure

101
Q

What symptoms are seen in large VSDs?

A

Significant amount of blood passing from left to right ventricle
Develop early heart failure and severe pulmonary HTN
Symptoms of cardiac failure evident after first few weeks of life

102
Q

What are the risk factors for VSD?

A

Maternal diabetes uncontrolled during pregnancy
Maternal rubella infection during pregnancy
Alcohol fetal syndrome
Uncontrolled maternal phenylketonuria PKU
Family history
Down’s, trisomy 18 and 13

103
Q

What are the clinical features of a small VSD?

A

Mild or no symptoms

Systolic murmur detected in routine examination

104
Q

What are the clinical features of moderate VSD?

A

Babies may have excessive sweating, easily fatigued
Tachypnoea
Especially notable when feeding
Obvious by 2-3 months as pulmonary vascular resistance decreases, and increase in left to right shunting

105
Q

What are the clinical features of large VSD?

A

Symptoms of congestive heart failure
SOB, problems feeding, developmental issues, freq chest infections

Eisenmenger’s syndrome can develop if large VSD left untreated - cyanosis

Intolerance to exercise, dizzy, chest pain, oedema, bluish complexion.

May have haemoptysis in severe cases.

106
Q

What is seen on general inspection in VSD?

A

Undernourished - fatigue during feeding so undernourished
Sweat on forehead - due to increased sympathetic activity, compensatory mechanism for decreased cardiac output
Increased work of breathing - due to pulmonary congestion
Cyanotic

Clubbing due to long standing arterial desat, but may be too mild to cause bluish complexion

Tachypnoea - left sided heart failure

107
Q

What is seen on palpation in VSD?

A

Palpate precordium, check peripheral pulses

Pulse rate - raised in congestive heart failure
Precordial palpation above where heartbeat felt; moving too much - hyperactive precordium, due to volume overload in left side of heart

Thrills - max intensity in lower left sternal border

108
Q

What is heard on auscultation in VSD?

A

Systolic murmur between S1 and S2
It is found at lower left sternal border
High pitched uniform blowing sound

Either holosystolic/pansystolic extends all the way from S1-S2

Or early systolic ends in middle or early systole, occurs when there is lower than normal pressure difference on the sides of the defect
e.g. neonate with large VSD

Or can have a diastolic murmur due to increased blood flow through mitral valve, mitral stenosis
Apical rumble/humming sound

109
Q

What are the differentials for VSD?

A

Mitral regurgitation
Tricuspid regurgitation
Atrial septal defect - murmur higher up, ejection systolic
Patent ductus. arteriosus
Pulmonary stenosis - ejection systolic murmur
Tetralogy of Fallot - symptoms more severe

110
Q

What are the investigations for VSD?

A

Bedside - ECG
Left ventricular hypertrophy or bilateral vent hypertrophy

Bloods -
Sepsis screen, U&Es for kidney function prior tx with diuretics or ACEi
Microarray for genetics

Radiology - 
CXR - large VSDs cardiomegaly, pulmonary oedema, pleural effusion
ECHO
Cardiac CT
MRI

Cardiac catheterisation helps with treatment

111
Q

What is the medical management for VSDs?

A

If small and symptomatic be ok, but maintain good oral hygiene to prevent IE.

Won’t close the defect, but relieve symptoms to buy time.

Increased caloric density of feeds for weight gain, may need to be NG with CHF

Diuretics - furosemide, add spironolactone for K sparing

ACEi - reduce left to right shunt, reduce afterload
Discontinue spironolactone

Digoxin to treat CHF
Increases heart muscle strength, maintains normal rhythm

112
Q

What is the surgical management of VSD?

A

If medium or large, or causing symptoms.

Surgical repair via median sternotomy and cardiopulmonary bypass, use of patch or stitches to close

Catheter less common as often not suitable, inserted via femoral artery, mesh

Hybrid - interventional cardiology

Palliative surgery with pulmonary artery banding and then full surgery later

113
Q

When is surgical closure of VSD indicated?

A

Qp/Qs (pulmonary to systemic blood flow ratio) of 2.0 or more

114
Q

What long term management if required for VSD?

A

Good dental hygiene to reduce risk of endocarditis
Avoid non medical procedures e.g. piercings and tattoos

Regular cardiac follow up

115
Q

What are the complications of VSD if left untreated?

A 
Congestive heart failure
Growth failure
Aortic valve regurgitation due to prolapse of valve
Pulmonary vascular disease
Freq chest infections
Infective endocarditis
Arrhythmias
Sudden death
116
Q

What are possible complications following surgical repair of VSD?

A

Permanent heart block requiring a pacemaker
Other arrhythmias
Wound infection
Reoperation of significant residual VSDs

117
Q

What is the pathophysiology of a PDA?

A

Pressure in aorta is higher than that in pulmonary vessels, blood flows from aorta to pulmonary artery - left to right shunt.

So pressure in pulmonary vessels is raised, leads to pulmonary hypertension and right sided heart strain

So leads to right ventricular hypertrophy

Then increased blood flow through pulmonary vessels and returning to left side leads to left ventricular hypertrophy

118
Q

What is the presentation of a PDA?

A

SOB
Difficulty feeding
Poor weight gain
LRTI

Murmur if more severe
Normal S1 and continuous crescendo-decrescendo murmur that may continue during S2 making it hard to hear

119
Q

How can a PDA be diagnosed?

A

ECHO

Use of doppler can assess size and characteristics of the left to right shunt

120
Q

What is the management of PDA?

A

For premature very low birth weight infants. - prophylactic therapy with intravenous indomethacin - COX inhibition and reduction in prostaglandins

Acute treatment for premature infants - indomethacin or ibuprofen, surgical ligation

For term infants and children - percutaneous catheter device closure, diuretics furosemide

121
Q

When does the PDA usually close?

A

Within first 48 hours of life

122
Q

What are the risk factors of PDA?

A

Prematurity
Maternal rubella
Female sex
RDS

123
Q

What are the diagnostic factors of PDA?

A 
Presence of risk factors
SOB
Failure to thrive
Exercise intolerance
Widened pulse pressure
Gibson machinery murmur
Apnoea in premature infants
124
Q

What are the investigations for PDA?

A

CXR
ECG
Echo

Cardiac catheterisation and angiography

125
Q

What is coarctation of the aorta?

A

Congenital condition where there is narrowing of the aortic arch
Usually around the ductus arteriosus

126
Q

What is the presentation of coarctation of the aorta?

A

In neonate - may only be weak femoral pulses
Four limb BP shows high BP in the limbs supplied from arteries coming before narrowing, and low in those afterwards

Systolic murmur below left clavicle and scapula

Tachypnoea
Increased work of breathing
Poor feeding
Grey and floppy baby

Left ventricular heave due to left ventricular hypertrophy
Underdeveloped left arm due to reduced flow to left subclavian
Underdeveloped legs

127
Q

What are common diagnostic factors in coarctation of aorta?

A

HTN at young age, or resistant to treatment
Diminished lower extremity pulses
Differential upper and lower extremity BPs

Systolic ejection murmur
Male sex
Could have Turner’s, Di George

128
Q

What are the risk factors for coarctation of aorta?

A 
Male sex
Young age
Turner's
Di George's
Hypoplastic left heart
Shone's complex
PHACE syndrome
129
Q

What are the investigations for coarctation of the aorta?

A

ECG, CXR, Echo

CT, MRI, cardiac catheterisation

130
Q

What is the management of coarctation of the aorta?

A

Critical - maintenance of ductal patency with alprostadil to maintain DA and then surgical repair

If not critical - surgical repair better than transcatheter

131
Q

What occurs in congenital aortic valve stenosis?

A

Born with narrow aortic valve; restricts blood flow from left ventricle into aorta

Aortic sinuses of Valsalva are the valve leaflets - can be a varied number of leaflets

132
Q

What is the presentation of aortic valve stenosis?

A

Mild asymptomatic and an incidental murmur on exam

Significant - fatigue, SOB, dizziness, fainting.
Worse on exertion as outflow cannot keep up

Severe will present with heart failure within months

Ejection systolic murmur
Loudest in aortic area
Crescendo-decrescendo
Radiates to the carotids

Ejection click just before murmur
Palpable thrill
Slow rising pulse, narrow pulse

Acute collapse in neonatal period, or in an older child e.g. child with chest pain and exertion

133
Q

What is the management of aortic valve stenosis?

A

Diagnosis by echo
Regular follow up with echo, ECG, exercise testing

Percutaneous balloon aortic valvoplasty
Surgical aortic valvotomy
Valve replacement
Ross procedure - disconnect pulmonary valve from right side, then insert valve replacement on pulmonary side

Moderate can monitor in clinic, monitor for endocarditis

Family screening for bicuspid aortic valve

134
Q

What are the complications of aortic stenosis?

A 
Left ventricular outflow obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death
135
Q

What occurs in pulmonary valve stenosis?

A

Pulmonary valve consists of three leaflets; in PVS leaflets can develop abnormally, become thickened or fused.
So narrowed between right ventricle and pulmonary artery

136
Q

What conditions are associated with pulmonary valve stenosis?

A

Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome

137
Q

What is the presentation of pulmonary stenosis?

A

Often completely asymptomatic, incidental finding

Symptoms of fatigue, SOB, dizziness and fainting

Ejection systolic murmur heard loudest at pulmonary area
Palpable thrill in pulmonary area
Right ventricular heave due to right ventricular hypertrophy
Raised JVP, giant a waves

Critical collapse in neonatal period when arterial duct closes

138
Q

What is the management of pulmonary stenosis?

A

Can be an increased risk of endocarditis

Diagnosis with echo

Watching and waiting if mild pulmonary stenosis

Balloon valvuloplasty via venous catheter through femoral vena into inferior vena cava and right side of heart

Open heart surgery

139
Q

What are the 5 Ts of cyanotic congenital heart disease?

A 
Tetralogy of Fallot
Transposition of Great Arteries
Truncus arteriosus
Total anomalous pulmonary venous return
Tricuspid valve abnormalities and hypoplastic right heart syndrome
140
Q

What is transposition of the great arteries?

A

Ventriculoarterial discordance
Aorta arises from the morphologic right ventricle and pulmonary artery from morphologic left ventricle

Potentially fatal parallel circulation
Deep hypoxaemia from lack of mixing, results in lactic acidosis and demise.

141
Q

What is the classification of transposition of great arteries?

A

Dextro-TGA aorta from RV, PA from LV causes deoxygenated blood from right heart to be pumped immediately through aorta and oxygenated blood into lungs through pulm artery.

Levo-TGA is double discordance; acyanotic defect because morphological left and right ventricles with their corresponding atrioventricular valves are also transposed. Systemic and pulmonary circulation connected.

In dextro the aorta is anterior and to right of PA
In levo- aorta is anterior and to the left of PA

142
Q

What anatomic sites may allow for mixing of blood and therefore life can be sustained in TGA?

A

Patent foramen ovale or atrial septal defect
Ventricular septal defect
Patent ductus arteriosus

143
Q

What are the risk factors for TGA?

A 
Maternal risk factors
Age over 40 years old
Maternal diabetes
Rubella
Poor nutrition
Alcohol consumption
144
Q

What are the clinical features of TGA?

A

Cyanosis appears in first 24 hrs if no mixing at atrial level

Mild cyanosis particularly when crying might be evident

Signs of CHF - tachypnoea, tachycardia, diaphoresis, failure to gain weight - may become evident over first 3-6 weeks as pulmonary blood flow increases

On examination:
Prominent right ventricular heave
Single second heart sound
Systolic murmur potentially if VSD present
No signs of resp distress
145
Q

What are the differentials of TGA?

A

TOF - CXR shows boot shaped heart in TOF, ECHO shows pulmonary cyanosis, overriding aorta, RVH

Tricuspid atresia - ECG shows left axis deviation in this

146
Q

What are the investigations for TGA?

A

Pulse oximetry shows cyanosis
Can be discrepancy between upper and lower limbs
Capillary blood gas may show metabolic acidosis with decrease pO2.

Echo definitive for diagnosis, shows abnormal position of aorta and pulmonary arteries
CXR - egg on a string
Narrowed upper mediastinum, cardiomegaly, increased pulmonary vascular markings

147
Q

What is the management of TGA?

A

Initial:
Emergency prostaglandin e1 infusion keeping DA open
Correct metabolic acidosis
Emergency atrial balloon septostomy to allow for mixing

Long term:
Surgical correction - arterial switch operation, usually performed before age of 4 weeks
Long term follow up and counselling in future if pt wishes to get pregnancy

148
Q

What are the long term consequences of TGA?

A

Prognosis depends on complexity and how it was repaired

Neopulmonary stenosis
Neoaoertic regurgitation
Neoaortic root dilatation
Coronary artery disease

Obstructed coronary arteries
Neurodevelopmental abnormalities
Low gestational age and high pre-operative lactate important predictors of poor developmental outcome

149
Q

What is Ebstein’s anomaly?

A

A congenital heart condition where the tricuspid valve is set lower in the right side of the heart towards the apex

Leads to a bigger right atrium and a smaller right ventricle

Leads to poor flow from right atrium to right ventricle and poor flow to pulmonary vessels

Right to left shunt across atria via atrial septal defect
Bypasses lungs and leads to cyanosis

150
Q

What condition is Ebstein’s anomaly associated with?

A

Wolff-Parkinson-White syndrome

151
Q

What is the presentation of Ebstein’s anomaly?

A 
Evidence of heart failure
Gallop rhythm on auscultation - addition of third and fourth heart sounds
Cyanosis
SOB, tachypnoea
Poor feeding
Collapse or cardiac arrest

If associated atrial septal defect too, present few days after birth when ductus arteriosus closes because prev was allowing blood flow from aorta to pulmonary vessels to be oxygenated and minimising. cyanosis

152
Q

How is Ebstein’s anomaly diagnosed?

A

Echocardiogram

153
Q

What is the management of Ebstein’s anomaly?

A

Medical management to treat arrhythmias and heart failure
Prophylactic abx for infective endocarditis
Definitive management and surgery to correct defect

154
Q

What is tricuspid atresia?

A

Absence of tricuspid valve associated with hypoplasia of right ventricle

155
Q

What is the pathophysiology of tricuspid atresia?

A

Valve is absent, right ventricle hypoplastic due to absence of inflow into right ventricle

Often a VSD also present

Needs to be inter-atrial communication to allow systemic venous return out of heart via LA and LV.

Most cases PA arises from hypoplastic left ventricle so there is reduced pulmonary flow

30% cases great arteries transposed, systemic perfusion poor

Commonly associated with coarctation of aorta or interrupted aortic arch

156
Q

What are the clinical features of tricuspid atresia?

A

Poor feeding if late diagnosis

On examination:

Progressive cyanosis
Could palpate a systolic thrill with pulmonary stenosis but rare, hepatomegaly if heart failure

Single S2 with pan-systolic murmur due to VSD on auscultation
Continuous mechanical murmur if PDA

157
Q

What are the investigations for tricuspid atresia?

A

ECG with superior QRS
CXR showing reduced or increased pulmonary markings, heart size may be normal or increased

ECHO
Atretic tricuspid valve
Mandatory right to left flow across atrial septum
Small right ventricle
Enlarged right atrium, left atrium and left ventricle

158
Q

What is the initial management of tricuspid atresia?

A

IV PGE1 infusion to prevent closure of PDA

Balloon atrial septostomy if inter-atrial communication inadequate

159
Q

What is the surgical management of tricuspid atresia?

A

Fontan circulation is final stage and is palliative not curative.

  1. At birth - shunt between subclavian artery and pulmonary artery to increase forward flow into pulmonary arteries.
  2. At 3-6 months - BGS shunt creates an end-to-side anastomosis between the superior vena cava and pulmonary artery
  3. Definitive procedure at 3-4 years - Fontan procedure to divert inferior vena cava flow directly to the PA by bypassing the right atrium or creating a tunnel within it.

Extra-cardiac conduit has lower operative mortality, lower incidences of early and late arrhythmias, improved haemodynamics.

Patients are started on anti-coagulation.

160
Q

What are the early complications following the Fontan procedure?

A

Low cardiac output and heart failure
Persistent pleural effusion and chylothorax
Thrombus formation in venous pathways

161
Q

What are the late complications following the Fontan procedure?

A

Supraventricular arrhythmias
Protein losing enteropathy - result of persistent pleural effusion
Progressive drop in arterial saturations resulting from obstruction in venous pathways

162
Q

What is the nitrogen washout test?

A

Used to determine presence of heart disease in a cyanosed neonate

Placed in 100% oxygen for 10 mins
If right radial arterial pO2 remains low from a blood gas - cyanotic heart disease can be made if lung disease and PPH can be excluded

If pO2 <20 kPa, not cyanotic

163
Q

What are the causes of common mixing?

Blue and breathless

A

Complete atrioventricular septal defect

Complex congenital heart disease e.g. tricuspid atresia

164
Q

What are the causes of outflow obstruction in the sick infant?

A

Coarctation of the aorta
Interruption of the aortic arch
Hypoplastic left heart syndrome

165
Q

What is hypoplastic left heart syndrome and its management?

A 
Underdevelopment of the entire left side of the heart
Mitral valve is small or atretic
Left ventricle is diminutive
Aortic valve atresia
Ascending aorta very small
Coarctation of the aorta

Sickest of all neonates, with duct-dependent systemic circulation
No flow through the left side of the heart = ductal constriction leads to acidosis and cardiovascular collapse

Weakness or absent peripheral pulses

Norwood procedure, then shunt, then Fontan at 3 years

166
Q

What is the most common childhood arrhythmia?

A

Supraventricular tachycardia

Presents with symptoms of cardiac failure; poor cardiac output and pulmonary oedema

Cause of hydrops fetalis and intrauterine death

167
Q

What will an ECG show in SVT?

A

Narrow complex tachycardia
250-300 bpm

Delta wave in WPW
If severe heart failure - inverted T waves

168
Q

What is the management of SVT?

A

Circulatory and resp support
Vagal stimulating manoeuvres
IV adenosine terminates tachycardia
Electrical cardioversion with synchronised DC shock if adenosine fails

Paediatrics (17 decks)

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