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Paediatrics > congenital heart defects > Flashcards

congenital heart defects Flashcards

1
Q

ASD prevalence

A

1.6/1000 live births
females more common

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2
Q

how atrial septum formed

A

two separate endocardial cushions during 4th week of gestation. Septum primum grows from the roof of the atrium down towards the atrioventricular endocardial cushions, closing off the ostium primum. The ostrium secondum grows downwards to septum primum and the space between the septum primum and secondum is foramen ovale

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3
Q

patent foramen ovale

A

Foramen ovale closes after birth when vascular resistance changes…BP increases and pulmonary pressure decreases causing a decrease in right atrium pressure..if not remains open

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4
Q

5 types of ASD (COMMON -> LEAST)

A

Patent foramen ovale
Ostium secundum defect
Ostium primum defect
Sinus venosus defect
Coronary sinus defect

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5
Q

Ostium secundum defect

A

incomplete occlusion of ostium secundum by septum secundum or too much reabsoprtion of septum primum from atrium roof

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6
Q

ostium primum defect

A

septum primum fails to fuse with endocardial cushions, allowing blood to travel from left to right atrium
- can be complete (spans from atrium to ventricles) or partial (just ostium primum)

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7
Q

sinus venous defect

A

superior defect - When superior vena cava (SVC) opening runs on top of oval fossa (foramen ovale remnant) of atrial septum. This renders SVC draining blood from both LA and RA. Usually co-exists with abnormal communication between SVC and right superior pulmonary vein
inferior defect - Less common than superior defect, but occurs when IVC orifice overrides LA & RA. Can co-exist with abnormal communication between IVC and right inferior pulmonary vein

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8
Q

coronary sinus defect

A

an absence in the roof of the coronary sinus. This can be partial or focal, allowing transmission between coronary sinus and left atrium.

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9
Q

risk factors ASD

A

aut dom, treacher-collins syndrome, TAR syndrome - ostium secondum ASD
family history
Maternal smoking in 1st trimester
Maternal diabetes
Maternal rubella
Maternal drug use e.g. cocaine & alcohol

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10
Q

ASD prognosis

A

not by itself life threatening, but co-existing increases mortality, same life expectancy unless diagnosis missed
post surgery - high risk of atrial flutter and AF

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11
Q

sx of large ASD in paeds

A

Tachypnoea
Poor weight gain
Recurrent chest infections

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12
Q

examination ASD

A

Murmur: soft, systolic ejection murmur, best heard over pulmonary valve region (2nd ICS, figure 2).
Wide, fixed split S2
Diastolic rumble in lower left sternal edge in patients with large ASD

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13
Q

investigations ASD

A

ECG - usually normal unless large defect…tall p waves, right BBB, right axis deviation
transthoracic echo is gold standard
cardiac MRI - measure pulmonary v systemic blood flow ratio (Qp/Qs)
CXR - cardiomegaly

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14
Q

initial management ASD

A

ASD < 5mm, spontaneous closure should occur within 12 months of birth.

In adults, if patient is presenting with no signs of right heart failure and a small defect, then monitor every 2 – 3 years with echocardiogram3.

If presenting with arrhythmia, control rhythm with drugs & anticoagulated before definitive surgical treatment

if child has HF - diuretics

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15
Q

definitive management ASD

A

surgical closure if ASD >1CM
via percutaneously (transcatheter) or open chest20 (central stenotomy) using cardiopulmonary bypass. Surgical closure is not recommended in patients where pulmonary hypertension is present (mean pulmonary pressure of 30mmHg), as this can induce RV failure if the ASD is closed up.

Percutaneous closure is carried out in cath lab and chosen method dependent on age of child

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16
Q

complications of percutaneous closure

A

Arrhythmias
Atrioventricular block
Thromboembolism (VTE aspirin)

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17
Q

indications for surgical closure

A

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

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18
Q

consequences of untreated large ASD

A

arryhtmias
pulmonary HTN
Eisenmenger syndrome (presenting with: chronic cyanosis, exertional dyspnoea, syncope, increased risk of infections, increased pulmonary vascular resistance)15
Cyanosis (only if Eisenmenger)
Peripheral oedema (if eventually leading to heart failure)
TIA / stroke

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19
Q

ASVD association

A

Down’s syndrome
Heretotaxy syndromes

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20
Q

ASVD pathophysiology

A

Primitive AV canal connects atria and ventricles. At 4-5 weeks of gestation, superior and inferior endocardial cushions of common AV canal fuse and contribute to formation of AV valves and septum…if endocardial cushions do not fuse correctly…causes apical displacement of AV valve and incomplete formation of ventricular septum
if complete failure of superior and inferior endocardial cushiosn to fuse = ASD nad VSD and single common atrio ventricular valve forms
if partialfailure - partial AV canal defect with ASD, a common valvular annulus with 2 separate AV valve orifices and cleft in anterior mitral leaflet

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21
Q

complete AVSD

A

increased shunting of blood from left to right at both atrial and ventricular levels..excessive pulmonary blood flow…HF and increased pulmonary vascular resistance. Also atrioventricular valve regurg

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22
Q

partial AVSD

A

left to right shunting at level of atrial septal defect…volume overload of both right atrium and ventricle…not enough to majorly affect pulmonary artery pressures…no sx until adulthood
regurg from LV to RA through defect…R sided volume overload

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23
Q

investigations AVSD

A

increased distance between aorta and apex of heart - ‘goose neck deformity’ on echo
karyotyping - down’s
ECG - superior QRS axis, prolonged PR, RVH in V1
cxr - cardiomegaly

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24
Q

clinical features AVSD

A

Tachypnoea
Tachycardia
Poor feeding
Sweating
Failure to thrive

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25
Q

examination avsd

A

characteristics of down’s
signs of congestive HF - hepatomegaly, gallop rhythm, oedema,crackles
pallor or harrisons grooves (chronic tachypnoea)
hyperactive precordium
systolic heave along left sternal border
palpable apical thrill

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26
Q

auscultation complete AVSD

A

An accentuated S1
Loud pulmonary component of S2 – In complete AVSD, the second heart sound narrowly splits and P2 increases in intensity (due to elevated pulmonary artery pressure)
Ejection-systolic murmur: best auscultated along Left upper sternal border (pulmonary area) due to increased blood flow through a normal pulmonary valve.
Mid-diastolic murmur: best auscultated along Left lower sternal border and apex due to the increased flow across the common atrioventricular valve.
Holosystolic murmur: best auscultated along Left lower sternal border and at cardiac apex if left atrioventricular valve regurgitation is present.

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27
Q

partial AVSD auscultation

A

Wide and fixed splitting of S2: the character of S2 does not change with inspiration
Ejection systolic murmur: best auscultated at Left upper sternal border due to turbulent blood flow across the pulmonary valve – may radiate to the lung fields.
Mid-diastolic murmur: best auscultated at Left lower sternal border. Usually low pitched and represents significant left AV valve regurgitation.
Holosystolic murmur: may be heard at the apex due to regurgitation through anterior mitral cleft

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28
Q

AVSD management

A

sx relief of HF - diuretics, ACEi, digoxin, adequate caloric intake
complete -> corrective surgery, around 3-6 mths of age
down’s - pulmonary parenchyma hypoplasia…earlier surgery

palliative surgery - pulmonary artery banding..reduce diameter and bloody flow

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29
Q

corrective surgery

A

via median sternotomy under cardiopulmonary bypass:
Closure of inter atrial communication
Closure of inter ventricular communication
Construction of two separate and competent AV valves from available leaflet tissue
via single, double or modified single patch repair

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30
Q

ASVD complications untreated

A

Failure to thrive
Recurrent lower respiratory tract infections
Congestive heart failure
Pulmonary Vascular disease
Eisenmenger’s syndrome

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31
Q

ASVD surgical repair complications

A

Left AV valve regurgitation – this may persist or worsen due to inadequate surgical reconstruction
A residual shunt across ASD or VSD which may require a further repair.
Cardiac conduction defects – Arrhythmias may occur in 10-15% of patients [6]
Sinus node dysfunction resulting in bradycardia
Wound infection due to poor healing

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32
Q

prognosis AVSD

A

mortality rate 2.5%
reoperation due to worsening mitral rerg
lifelong cardiac follow up

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33
Q

hypoplastic left heart syndrome prevalence

A

1 in 5000 live births

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34
Q

risk factors hypoplastic left heart syndrome

A

environmental factors
seasonal variations
in utero maternal infections - rubella, herpes virus, coxsackie, cytomegalovrius

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35
Q

left side of heart to work must have a…

A

a) Patent ductus arteriosus to ensure adequate systemic circulation and
b) A non-restrictive atrial septal defect to ensure adequate mixing of oxygenated and deoxygenated blood.

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36
Q

the right ventricle in HLHS

A

has to support both systemic and pulmonary circulations

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37
Q

when become symptomatic HLHS

A

birth - patent ductus arteriosis is unrestrictive and high pulmonary vascular resistance..adequate systemic perfusion across duct into descending aorta
but PDA closes and pulmonary vascular resistance reduces…decreased systemic perfusion and increased pulmonary flow…cardiogenic shock

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38
Q

spectrum of defects of HLHS

A

Aortic atresia with mitral atresia (most extreme)
Aortic atresia with patent mitral valve
Aortic stenosis with patent mitral valve

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39
Q

common cardiac associations of HLHS

A

coronary cameral fistulas
persistent left SVC
anomalous pulmonary venous drainage

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40
Q

medical conditions associated HLHS

A

CHARGE syndrome
Turner’s
triosomies
microencephaly

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41
Q

clinical features HLHS

A

initially - healthy
then hypoxaemia, acidosis and shock..unless restrictive patent foramen ovale or intact atrial septum since birth…cardiogenic shock

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42
Q

clinical signs HLHS

A

Tachycardia, dyspnea and evidence of pulmonary oedema
Weak peripheral pulses, and vasoconstricted extremities
Loud single S2 (due to aortic atresia)
Hepatomegaly (secondary to congestive heart failure)

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43
Q

investigations HLHS

A

ECG: shows RVH (and occasionally right axis deviation)
CXR: shows pulmonary venous congestion or pulmonary edema. Moderately enlarged cardiac shadow.
ECHO: Diminutive left ventricle, Dilated and enlarged right ventricle, Aortic hypoplasia, Color flow Doppler shows retrograde blood flow in aorta

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44
Q

initial stablisation HLHS

A

secure patency of duct:
Prostaglandin E2 infusion
Diuretics and inotropic support – in case of congestive cardiac failure
Intubation and ventilation – occasionally required for hemodynamic stabilization
Balloon atrial septostomy – may be required in cases of restrictive IAS

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45
Q

definitive stabilisation HLHS

A

conversion of the single ventricle into a systemic ventricle and establishing an obstructed pulmonary blood flow by bypassing the heart.

The definitive repair of HLHS is a 3-staged repair:

Stage 1: Norwood procedure
Stage 2: Glenn procedure (Bi-directional Glenn or Hemi-Fontan)
Stage 3: Fontan Procedure

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46
Q

norwood procedure

A

Atrial septectomy to establish unobstructed pulmonary venous return
Reconstruction of the aortic arch using the main pulmonary artery to establish a systemic circulation
Placement of a modified BT shunt /RV-PA conduit (Sano shunt) to re-establish pulmonary blood flow
Creating a connection between smaller ascending aorta and pulmonary root to establish coronary blood supply (DKS – Dammus-Kaye Stansel)

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47
Q

glenn procedure

A

anastomosis of the superior vena cava to the ipsilateral pulmonary artery and takedown of previously placed shunts to provide pulmonary blood flow

48
Q

fontan procedure

A

this procedure directs inferior vena cava return to the pulmonary vasculature, so that all the systemic venous return runs passively to the lungs, effectively bypassing the right ventricle. This creates a system with a single ventricle pumping blood into separate, systemic and pulmonary circulations aligned in series, thereby relieving cyanosis.

49
Q

alternative to staged repair HLHS

A

heart transplantation- 25% die waiting

50
Q

long term outcomes HLHS

A

estimated three to six year survival rate of 60-70%, for infants undergoing stage 1 repair. Infants who survive the initial 12 months, have a long term survival rate of approximately 90% (to the age of 18 years) [5]. It is also important to note that patients with HLHS who survive the staged palliation are at a risk of neurodevelopmental impairment.

The other aspect to be considered here is the ongoing ethical debate over the role of investment in a series of complex cardiac procedures to achieve what is deemed to be a palliative circulation

51
Q

tetralogy of fallot prevalence

A

most common cyanotic CHD

52
Q

tetralogy of fallot made of

A

Ventricular septal defect (VSD)
Pulmonary stenosis (PS)
Right ventricular hypertrophy (RVH)
Overriding aorta

53
Q

ToF increased risk

A

males
1st degree fhx
fetal alcohol syndrome
fetal warfarin syndrome
trimethadione
CHARGE syndrome
Di George syndrome
VACTERL association

54
Q

VSD types

A

VSD involves smaller membranous septum or large muscular septum or both parts - permimembranous VSD. or double committed VSD’S

55
Q

VSD cyanotic or acyanotic?

A

if mild - the left ventricular pressures higher than the right ventricle…blood shunts from left to right through VSD so acyanotic
more severe - increased right ventricular pressure shunt reverses from right to left so cyanotic

56
Q

pulmonary stenosis ToF

A

most common site - infundibular septum

57
Q

RVH ToF

A

Hypertrophy of the right ventricle occurs in response to the high pressures it must overcome to pump deoxygenated blood through the RVOTO. This usually develops in utero and may be seen in chest x-rays as the ‘boot’ sign.

58
Q

overriding of aorta ToF

A

caused by an increase in blood flow through the aorta as it receives blood from both ventricles via the VSD.

In severe TOF, multiple aorto-pulmonary collateral arteries (“MAPCAs”) may also form to help increase pulmonary blood flow.

59
Q

clinical features of ToF

A

mild - asx, 1-3 yrs - cyanosis
mod-severe - first few weeks - cyanosis, resp distress, prone to chest infections, failure to thrive
extreme - first few hourse

60
Q

extreme ToF

A

TOF with pulmonary atresia (10% of TOF patients) or absent pulmonary valves (6%).

These are true ‘duct dependent lesions’ as the only way deoxygenated blood can flow into the lungs is through a patent ductus arteriosus (PDA)

61
Q

examination ToF

A

central cyanosis
clubbing
thrill, heave
signs of congestive HF

62
Q

ToF auscultation

A

Loud, single S2: due to closure of aortic valve in diastole with absent/reduced pulmonary valve closure (P2) depending on the degree of stenosis.
Pansystolic murmur: best auscultated either mid or upper left sternal edge (LSE). The smaller the VSD the louder the murmur and vice versa.
Ejection click4: high pitch noise which occurs at the maximal opening of semilunar (aortic or pulmonary) valves. Clicks in TOF occur due to presence of dilated aorta. Normally heart immediately after S1.
Continuous, machinery murmur: occurs in the presence of PDA with extreme forms of TOF, especially those on prostaglandin infusion. Best auscultated at the upper LSE or left infraclavicular area.

63
Q

Cyanotic CHD

A

ToF
Critical PS
Transposition of the Great Arteries (TGA)
Totally anomalous pulmonary venous drainage (TAPVD)
Hypoplastic left heart syndrome (HLHS)

64
Q

ToF investigations

A

Bedside:

ECG: may show signs of right axis deviation and RVH.
Bloods:

Microarray: if genetic syndromes suspected (e.g. dysmorphic features, multiple anomalies).
Radiological:

CXR: may show ‘boot’ shaped heart (RVH) and reduced pulmonary vascular marking (decrease pulmonary blood flow).

cardiac CT.MRI

Cardiac catheter

65
Q

medical management ToF

A

squatting or knees to chest - increased venous return
prostaglandin infusion
beta blockers - reduce HR, and thus venous return
morphine - reduce resp drive
saline - increase pulmonary blood flow

66
Q

surgical management ToF

A

palliative - transcatheter RVOT stent insertion, modified blalock-taussig shunt, insertion of RV to PA conduit
definitive - under cardiopulmonary bypass via median sternotomy, this involves RVOT stenosis resection, RVOT/pulmonary artery augmentation and VSD patch closure

67
Q

complications ToF untreated

A

Polycythaemia
Cerebral abscess
Stroke
Infective endocarditis
Congestive cardiac failure
Death (up to 25% in the 1st year of life4)

68
Q

complications surgery ToF

A

pulmonary regurgitation (PR), arrhythmias, exercise intolerance and sudden death.

69
Q

total anomalous pulmonary venous drainage prevalence

A

0.6-1.2 per 10,000 live births
male 4:1 female

70
Q

risk factors total anonamalous pulmonary venous drainage

A

exposure to lead, paint, paint stripping chemicals, pesticides

71
Q

total anomalous pulmonary venous drainage definition

A

no direct communication between pulmonary veins and left atrium…instead drains into systemic venous tributaries or RA…cyanosis

72
Q

TAPVD pathophysiology

A

At around 4 weeks of gestational age, the primitive pulmonary vein develops as an outpouching from the left atrium.
This primitive pulmonary vein then connects with the pulmonary venous system that was formed earlier on in development from the splanchnic plexus. The pulmonary portion then separates from the splanchnic plexus.
In a case of TAPVD, this pulmonary vein either does not form, or, if formed does not connect with pulmonary venous system. This leaves the pulmonary venous system connected to the systemic venous drainage.
As a consequence the right side of the heart (right atrium and ventricle) remains enlarged at birth (due to all the pulmonary venous return draining to the right side).
If the pulmonary venous system is neither connected to the primitive pulmonary vein nor the systemic venous system, then it results in either an intra-uterine death or an early neonatal death.

73
Q

types of TAPVD

A

supracardiac
cardiac
infracardiac
mixed

74
Q

factors influencing timings/severity of presentation

A

pulmonary venous obstruction - whether at supracardiac, cardiac or infracardiac level
inter-atrial communication - resticted - compromises systemic output..elevated right atrial pressures..congestion
left to right shunting- if unobstructed form, net left to right shunt…RVH and failure

75
Q

unobstructed TAPVD sx and signs

A

Symptoms:

These infants are relatively stable at birth.

Asymptomatic at birth
Mild cyanosis (usually detected on pulse oximetry screening)
Symptoms due to pulmonary over-circulation: increased work of breathing, recurrent respiratory infections, poor feeding and failure to thrive.
Signs:

Fixed splitting of second heart sound (due to volume overload of RV)
Ejection systolic murmur (due to physiological pulmonary stenosis)
Hepatosplenomegaly (due to right sided heart failure)
Tachypnea

76
Q

obstructed TAPVD sx and signs

A

Obstructed TAPVD
Symptoms:

These infants present severely ill at birth.

Severe cyanosis
Respiratory failure
Shock
Signs:

Prominent second heart sound
Soft, continuous murmur heard over area of obstructed anomalous vertical vein
Weak pulses, low blood pressure and cool peripheries
Hepatomegaly (due to direct venous congestion)

77
Q

TAPVD investigations

A

ECG - tall R waves in V1 and peaked P waves
CXR - cardiomegaly, ‘snowman’ from dilation fo veins
echo - definitive diagnosis

78
Q

initial management TAPVD

A

obstructive - mechanical ventilation, correction of metabolci acidosis, prostagalndin E1, mainly - ECMO, cardiac catheterisation
unobstructed - sx relief such as diuretics

79
Q

TAPVD definitive repair

A

Connect the common pulmonary venous channel to the left atrium
Divide the vertical pulmonary vein, and
Close an residual inter-atrial communication (if present)

80
Q

post op complication TAPVD

A

Pulmonary venous obstruction at the site of surgical repair – requiring long term re-intervention.
Sinus node dysfunction – due to localised disruption at the site of repair.
Routine post-op complications: including post-op sepsis and wound infection.

81
Q

TAPVD prognosis

A

ECMO improved
The early mortality (within 30 days of surgical correction) rates are less than 10% and the late mortality rates are 5%. Long term survival rates to adolescence are between 85-90%

82
Q

classification of transposition of great arteries

A

60% - aorta is anterior and to right of pulmonary artery (dextro-transposition)
aorta can be anterior and left (levo-transposition
in one third - coronary artery anatomy abnormal

83
Q

most common cause of cyanosis in new born

A

transposition of great arteries

84
Q

TGA prevalence

A

20-30 per 100,000 births
male infants higher

85
Q

dextro TGA

A

the pulmonary and systemic circulation run in parallel, causing oxygenated blood to recirculate only in the pulmonary circulation and deoxygenated systemic blood to bypass the lungs. This results in cyanosis unless there is mixing of oxygenated blood and deoxygenated blood.

86
Q

levo TGA

A

ventricles have switched places as opposed to the arteries and thus this is acyanotic as deoxygenated blood can return from the systemic circulation and enter the pulmonary circulation to be oxygenated before entering the systemic circulation again. ….results in tricuspid regurg and HF

87
Q

maternal risk factors TGA

A

Age is over 40 years old
Maternal diabetes
Rubella
Poor nutrition
Alcohol consumption

88
Q

clinical features TGA

A

cyanosis - 1st 24 hrs
congestive HF
Prominent right ventricular heave
Single second heart sound, loud A2
Systolic murmur potentially if VSD present
No signs of respiratory distress

89
Q

ddx cyanosis

A

ToF
TGA
tricsupid atresia

90
Q

investigations TGA

A

pulse oximetry
echo - definitive as shows abnormal position of aorta and pulmonary arteries
CXR - ‘egg on a string’

91
Q

initial management TGA

A

emergency prostaglandin E1 infusion
correct met acidosis
emergency atrial balloon septostomy

92
Q

definitie management TGA

A

surgical correction - arterial switch operation
long term follow up

93
Q

prognosis TGA

A

survival >90% at 20 years

94
Q

long term conseqeunces TGA

A

Neopulmonary stenosis
Neoaortic regurgitation
Neoaortic root dilatation
Coronary artery disease
further syrgery - balloon angioplasty
obstructed coronary arteries
sudden cardiac death
high frequency of neurodevelopmental abnormalities
(esp if low gestational age and high pre op lactate)

95
Q

tricuspid atresia prevalence

A

1 per 10,00 births

96
Q

tricuspid atresia pathophysiology

A

The tricuspid valve is absent and the right ventricle is hypoplastic due to absence of the inflow into the right ventricle. In the vast majority of cases there will be a ventricular septal defect (VSD) present and the size of this will affect the size of the right ventricular cavity. In all cases there must be an inter-atrial communication to allow systemic venous return out of the heart via the left atrium and ventricle.
70% of cases - great arteries are normally related
30% of cases - great arteries transposed

97
Q

clinical features tricsupid atresia

A

poor feeding
progressive cyanosis
reduced sats
Palpation: Systolic thrill associated with pulmonary stenosis is rarely palpable. Hepatomegally may be present if the inter-atrial communication is inadequate or if late diagnosis and child is in heart failure
Auscultation: Single S2 with pan-systolic murmur due to VSD, best heard at left lower sternal edge. There may also be a continuous, mechanical murmur from the patent ductus arteriosus (PDA)
Signs of heart failure

98
Q

investigations triscupid atresia

A

ECG - ‘superior’ QRS
CXR - reduced of increased pulmonary marking, heart size normal or increased
echo - atretic tricuspid valve

99
Q

tricuspid atresia inittial management

A

IV PGE1 infusion - prevent closure of PDA
balloon arterial septostomy

100
Q

tricuspid atresia surgical management

A

As Tricuspid Atresia causes hypoplasia of the right ventricle, patients embark on the uni-ventricular surgical palliation. The Fontan circulation is the final stage for this pathway and is palliative and not curative. The overall aim of the Fontan circulation is for passive flow of the systemic venous return to go directly to the lungs and therefore avoid the heart. This therefore means that the single ventricle is only required to pump blood to the body, decreasing its workload and so protecting it from earlier failure.

However, for the Fontan circulation to work, you must allow time for the pulmonary vascular resistance to drop. The pulmonary pressures need to be low in order to facilitate forward flow from the low pressure systemic veins. Therefore these patients undergo a series of operations, firstly to augment pulmonary blood flow and allow this time and then to create the Fontan circulation in stages.

101
Q

complications post-fantan atresia of tricsupid

A

Early:

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

Supraventricular arrhythmias (also an early complication)
Protein losing enteropathy, a result of persistent pleural effusion which carries a poor prognosis
Progressive drop in arterial saturations resulting from obstruction in venous pathways

102
Q

VSD prevalence

A

4.2 per 1000 births

103
Q

VSD shunt

A

increased flow through pulmonary circulation…pressure of left ventricle greater than right..left to right

104
Q

VSD size of defect

A

very small/restrictive - no significant increase in pulmonary blood flow…asx
moderate - : The flow of blood through the VSD is great enough to cause a significant increase in blood flow through the pulmonary circulation. As the shunt is happening in systole, the extra volume of blood is pumped directly to the pulmonary circulation, so there is no initial effect on the right ventricle..risk of congestive HF and arryhtmias
large - early HF and severe pulmonary HTN

105
Q

eisenmenger’s syndrome

A

a condition where the pressure in the right ventricle exceeds that of the left ventricle and is caused by a significant gradual increase in the pulmonary vascular resistance.
It results in a shunt reversal, with deoxygenated blood flowing from the right ventricle into the left ventricle and entering the systemic circulation. This causes decreased systemic oxygen saturation and these patients become cyanotic.

106
Q

risk factors VSD

A

maternal DM uncontrolled
maternal rubella infection
foetal alcohol syndrome
uncontrolled maternal PKU
family hx of VSD
down’s etc

107
Q

clinical features VSD

A

small - mild or asx
moderate - excessive sweating, faitgued, tachypnoea
large - similar to congestive HF, developmental issues with weight and height, chest infections, eisenmenger’s can develop

108
Q

physical examination VSD

A

fatigue during feeding
sweat
increased work of bteathing
cyanotic
signs of chromosomal disorders
clubbing - long standing arterial desaturation
tachypnoea
tachycardic
hyperactive precordium
trill in lower left sternal border

109
Q

auscultation VSD

A

Systolic Murmurs:

Systolic murmurs occur between the S1 and S2 heart sounds.

Location: Lower left sternal border
Quality: A uniform, high pitched sound, often described as a blowing sound.
The murmur is either holosystolic or early systolic:

Holosystolic (Pansystolic) murmur: Starts at S1 and extends all the way to S2. This is the most likely type of murmur to be heard with VSD.
Early systolic murmur: Starts at S1 and ends in the middle or early systole. It usually occurs when there is lower than normal pressure difference between the two sides of the defect. Scenarios where this type of murmur may be heard include in a neonate with a large VSD and in children or adults with a very small VSD or a large VSD accompanied by pulmonary hypertension.
Diastolic murmur:

An apical mid diastolic murmur may be heard with VSD.

Cause: Increased blood flow through the mitral valve causing a relative mitral stenosis
Location: The heart apex
Timing: Early to mid-diastole
Description: It starts with an abnormally loud S3. Though often referred to as an apical rumble, it is said to sound more like a hum than a rumble.

110
Q

investigations VSD

A

ECG - signs of LVH
blood - septic screen, kidney function (diuretics, ACEi)
CXR - normal or cardiomegaly
echo - golf standard
cardiac Ct angio
MRI
cardiac catherterisation

111
Q

medical management VSD

A

increased caloric density
diuretics
ACEi
digoxin

112
Q

surgical manegemtn VSD

A

indicated when Qp/Qs of 2.0 or more
surgical repair - patch material or stiches via open heart surgery
catheter procedure
hybrid approach
pulmonary artery banding - palliative
risk of endocarditis so good dental hygeine onoging

113
Q

left untreated VSD

A

Congestive heart failure
Growth failure
Aortic valve regurgitation due to prolapse of a valve leaflet through the defect
Pulmonary vascular disease that in severe cases can lead to Eisenmenger’s Syndrome
Frequent chest infections
Infective Endocarditis
Arrhythmias
Sudden death

114
Q

surgical complications VSD

A

Permanent heart block requiring pacemaker (in 0.0-2.1% of patients) (10), or other arrhythmias
Wound infection
Reoperation of significant residual VSDs

115
Q

prognosis VSD

A

75% of small VSDs and especially the ones located in the muscular part of the interventricular septum, close spontaneously by the age of 10 years (11) and adults with closed VSD are expected to have a normal lifespan (12).

When surgical closure is required, if the surgery is done early before any serious heart or lung problems develop and no complications arise, then the outlook is positive.

The prognosis is much worse for patients who develop pulmonary hypertension and Eisenmenger’s Syndrome. These patients have progressive exercise intolerance and a worsening right ventricular function that can reduce the life expectancy to 20-50 years (13).

116
Q
A

Paediatrics (17 decks)

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