Paeds - cardio

Question 1

what can cause a congenital left to right shunt?

how would they present?

Answer 1

ventricular septal defect
persistent arterial duct
atrial septal defect

they present as breathless

Question 2

what can cause right to lefts shunts?

how would they present?

Answer 2

tetralogy of Fallot
transposition of the great arteries

they would present as blue

Question 3

what are the circulatory changes at birth?

Answer 3

first breath = a fall in resistance to pulmonary blood flow

• -> blood flow through the lungs increases six fold
• ->this results in a rise in left atrial pressure.
• -> mean while the blood returning to right atrium falls as the placenta is excluded from the circulation
• -> the changes in the pressure difference causes the foramen ovale to close
• the DA will normally close within the first few hours or days of life

Question 4

what are the symptoms and signs of heart failure?

Answer 4

symptoms - breathlessness (particularly on feeding or exertion), sweating, poor feeding, recurrent chest infection

signs: poor weight gain or faltering growth, tachypnoea, tachycardia, heart murmur, gallop rhythm, enlarged heart, hepatomegaly, cold peripheries.

Question 5

what usually causes heart failure in the first week of life?

Answer 5

left heart obstruction e.g. coarctation of the aorta

*if obstruction is very severe then arterial perfusion may be predominantly by right to left flow of the blood via the ductus arteriosus (duct dependant circulation) - closure of the duct = severe acidosis, collapse and death unless duct patency is restored

Question 6

after the first week of life what is the most common cause of progressive heart failure?

Answer 6

left to right shunt

this eventually causes pulmonary oedema and breathlessness due to decreased pulmonary vascular resistance

Question 7

what happens if a left to right shunt is left untreated?

Answer 7

children will develop Eisenmenger syndrome which is irreversibly raised pulmonary vascular resistance resulting from chronically raised pulmonary arterial pressure and flow. this lead the shunt reversing to a right to left shunt and results in a blue child. If this develops the only surgical option is a heart-lung transplant if available.

Question 8

what might cyanosis in the new-born infant in the new born be due to?

Answer 8

• cardiac disorders - cyanotic congenital heart disease
• resp disorders - surfactant deficiency, meconium aspiration, pulmonary hypoplasia
• persistent pulmonary hypertension of the new born - failure of the pulmonary vascular resistance to fall after birth
• infection - septicaemia from group B streptococcus and other organisms
• metabolic disease - metabolic acidosis and shock

Question 9

what are the different types of ASD?

Answer 9

secundum ASD

partial/primum ASD

Question 10

what are the symptoms of ASD?

Answer 10

often asymptomatic
may have recurrent chest infections
from the fourth decade they may develop arrhythmias

Question 11

what are the signs of ASD?

Answer 11

an ejection systolic murmur best heard at the upper left sternal edge - due to increased flow across the pulmonary valve because of the left-to-right shunt
a fixed and widely split second heart sound

Question 12

what investigations would you perform for ASD?

Answer 12

CXR - cardiomegaly, enlarged pulmonary arteries and increased pulmonary vascular markings
ECG - right bundle branch block
Echo

Question 13

how do you manage ASD?

Answer 13

if the defect is large enough to cause ventricular dilation treatment will be required.
For secundum ASD this is by cardiac catheterisation with insertion of an occlusive devise

Partial AVSD - surgical correction is needed - usually at about 3-5 years of age to prevent heart failure and arrhythmias

Question 14

what is a small VSD?

Answer 14

smaller than the aortic valve in diameter - up to 3mm

Question 15

how would a small VSD present ?
what would you hear when auscultating a small VSD?
what investigations would you perform and how would you manage one?

Answer 15

often asymptomatic
a loud pan-systolic murmur at lower left sternal edge (loud murmur = smaller defect)
quiet pulmonary second sound
CXR and ECG will be normal
echo will demonstrate precise anatomy of defect

management - often will close spontaneously

Question 16

what is a large VSD?
how would a large VSD present ?
what would you hear when auscultating a large VSD?
what investigations would you perform and how would you manage one?

Answer 16

• bigger than the size of the aortic valve
• symptoms of heart failure with breathlessness and FTT after 1 week old, recurrent chest infections, tachypnoea, tachycardia and enlarged liver from heart failure, active precordium.
• there may be a soft or no murmur = large defect
• there will be a loud pulmonary second sound from raised pulmonary pressure
• CXR - cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings, pulmonary oedema
• ECG - biventricular hypertrophy by 2 months of age
• ECHO - demonstrated anatomy of defect
• Management: diuretic for heart failure (furosemide) often combined with captopril.
  Surgery should be performed at age 3-6 month

Question 17

what is patent ductus arteriosus?

Answer 17

a form of congenital heart defect
generally classed as ‘acyanotic’. However, uncorrected can eventually result in late cyanosis in the lower extremities, termed differential cynaosis.
connection between the pulmonary trunk and descending aorta
usually the ductus arteriosus closes with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance
more common in premature babies, born at high altitude or maternal rubella infection in the first trimester

Question 18

what are the signs of patent ductus arteriosus?

Answer 18

left subclavicular thrill
continuous 'machinery' murmur
large volume, bounding, collapsing pulse
wide pulse pressure
heaving apex beat

Question 19

how do you manage a PDA?`

Answer 19

1st line - IV indomethacin (inhibits prostaglandin synthesis ) - closes the duct in the majority of the cases

*if it is associated with another congenital heart defect amenable to surgery then prostaglandin E1 is usefull to keep the duct open until after surgical repair

use a percutaneous catheter device closure
if baby too small or large duct then use surgical ligation

Question 20

what test can be used in cyanosed infants to test for heart disease?

Answer 20

the hypoxia (nitrogen washout) test
place them in 100% oxygen for 10 mins and then if right radial artery PaO2 from blood gas remains low then it Is cyanotic congenital heart disease

Question 21

what are the 4 cardinal anatomical features of tetralogy of Fallot?

Answer 21

ventricular septal defect (VSD)

• right ventricular hypertrophy
• right ventricular outflow tract obstruction
• pulmonary stenosis
• overriding aorta

Question 22

what are the symptoms of TOF?

Answer 22

cyanosis - hypercyanotic episodes
a loud harsh ejection systolic murmur
in older children there may be clubbing
may have tachypnoea

Question 23

what investigations would you perform for TOF and what would they show?

Answer 23

CXR - boot shaped heart
ECG - will show right ventricular hypertrophy when older
ECHO - will show the cardinal features.

Question 24

how do you manage TOF?

Answer 24

complete surgical repair to close VSD and relieve RV outflow obstruction

Question 25

what is transposition of the great arteries?

Answer 25

the aorta is connected to the right ventricle and the pulmonary arteries are connected to the left ventricle - this means there is two parallel circulation *incompatible with life unless there is a mixing of blood - often there is naturally occurring VSD, ASD, PDA which can achieve mixing short term leads to right to left shunt

Question 26

what are the symptoms of transposition of the great arteries?

Answer 26

neonatal cyanosis usually appears on day 2 when ductal closure leads to reduced mixing of desaturated and saturated blood often a loud, single heart sound

Question 27

how do you manage transposition of the great arteries?

Answer 27

maintain ductus arteriosus with prostaglandin infusion a balloon atrial septostomy all patients will require surgery, arteria switch procedure in the neonatal period

Question 28

what is and AVSD? how does it present how is it managed?

Answer 28

atrioventricular septal defect common in down-syndrome may be complete or partial it will present on antenatal USS screening cyanosis at birth or heart failure at 2-3 weeks of life manage heart failure and fix surgically at 3-6 months

Question 29

what is aortic stenosis?

Answer 29

when the aortic valve leaflets are partly fused together, giving a restrictive exit form the left ventricle often associated with mitral valve stenosis and coarctation of the aorta

Question 30

what are the symptoms and signs of aortic stenosis?

Answer 30

asymptomatic murmur severe stenosis - may lead to exercise tolerance, chest pain on exertion or syncope small volume, slow rising pulse carotid thrill ejection systolic murmur that radiates to the neck delayed and soft aortic second sound apical ejection click

Question 31

how do you manage aortic stenosis and pulmonary stenosis ?

Answer 31

balloon dilatation

Question 32

what are the clinical features of pulmonary stenosis? | what would investigations show?

Answer 32

mainly asymptomatic small number of neonates have critical pulmonary stenosis and have duct- dependant circulation ejection systolic murmur best heard at the upper left sternal edge ejection click there may be a ventricular heave when stenosis is severe CXR - normal or post stenotic dilation of the pulmonary artey ECG - evidence of right ventricular hypertrophy

Question 33

what is coarctation of the aorta?

Answer 33

uncommon | narrowing of the aorta distal to the left subclavian artery adjacent to the insertion of the arterial duct.

Question 34

what are the clinical features of coarctation of the aorta?

Answer 34

``` often asymptomatic sometimes when the duct closes the aorta also constricts, causing severe obstruction of the LV outflow systemic hypertension ejection systolic murmur radiofemoral delay ```

Question 35

what would you see on investigations for coarctation of the aorta?

Answer 35

CXR - rib notching due to the development of large collateral intercostal arteries running under the ribs posteriorly to bypass the obstruction, 3 sign - visible notch in the descending aorta at the sit of the coarctation ECGH - LV hypertrophy

Question 36

how do you manage coarctation of the aorta?

Answer 36

insertion of a stent | sometimes surgical repair is required

Question 37

what is interruption of the aortic arch?

Answer 37

Interrupted aortic arch is a very rare heart defect in which the aorta is not completely developed. There is a gap between the ascending and descending thoracic aorta. In a sense it is the complete form of a coarctation of the aorta. usually VSD is present presentation is with shock in the neonatal period - circulatory collapse with absent femoral pulses and absent left brachial pulse

Question 38

how do you manage an interruption of the aortic arch?

Answer 38

maintain ABC prostaglandin infusion complete correction with closure of VSD and repair of the aortic arch is usually performed within the first few days of life

Question 39

what is hypoplastic left heart syndrome?

Answer 39

underdevelopment of the left side of the heart small or atretic mitral valve left ventricle is diminutive and there is usually aortic valve atresia small ascending aorta circulatory collapse - all peripheral pulses absent sometimes detected antenatally no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid CV collapse

Question 40

how do you manage hypoplastic left heart syndrome?

Answer 40

maintain ABC and prostaglandin infusion | Norwood procedure then at 67 months the Glenn or hemi-Fontan procedure and again the Fontan procedure about 3 years

Question 41

what is a sinus arrhythmia?

Answer 41

it is normal in children and it detectable as a cyclical change in heart rate with respiration (acceleration during inspiration)

Question 42

what is the most common childhood arrhythmia?

Answer 42

supraventricular tachycardia

Question 43

what are the clinical features of supraventricular tachycardia?

Answer 43

rapid HR between 250-300BPM can cause poor CO and pulmonary oedema it will typically present as heart failure

Question 44

what will an ECG show in supraventricular tachycardia?

Answer 44

narrow complex tachycardia

Question 45

management of supraventricular tachycardia

Answer 45

``` circulatory and respiratory support vagal stimulation manoeuvres e.g. carotid sinus massage IV adenosine electrical cardioversion flecainide ```

Question 46

what is congenital complete heart block?

Answer 46

rare condition usually related to the presence of anti-RO or anit-LA antibodies in maternal serum - this mothers will either manifest or latent connective tissue disorders the antiboides appear to prevent the normal development of the conduction system in the developing heart with atrophy and fibrosis of the AV node all children with symptoms will require the insertion of an endocardial pacemaker

Question 47

hoe does long QT syndrome present in children?

Answer 47

associated with sudden loss of consciousness during exercise, stress or emotion usually in late childhood may be mistakenly diagnoses as epilepsy inheritance is AD

Question 48

what is rheumatic fever?

Answer 48

there is an abnormal immune response to a streptococcal infection usually develops 2-6 weeks following the infection it is usually a pharyngeal infection - then polyarthritis, mild fever and malaise develop

Question 49

what is the evidence for a recent streptococcal infection?

Answer 49

raised ASO titre or other streptococcal antibodies positive throat swabs positive rapid group A strapt antigen test

Question 50

what are the major and minor criteria for rheumatic fever?

Answer 50

Two major or one major and two minor MAJOR: erythema marginatum - uncommon early manifestation rash on trunk and limbs, Sydenham's chorea - a late feature - involuntary movements and emotional liability, polyarthritis, pancarditis (endocarditis, myocarditis, pericarditis), subcutaneous nodules MINOR: fever, raised ESR or CRP, arthralgia, prolongued PR interval

Question 51

what are the symptoms of chronic rheumatic fever?

Answer 51

the most common long-term damage is mitral stenosis from scarring and fibrosis.

Question 52

how should you manage rheumatic fever?

Answer 52

acute management is with bed rest and anti-inflammatory agents if there is evidence of active myocarditis then bed rest and limitation of exercise are essential aspirin good for supressing inflammatory response in joints and heart - it needs to be given in high dose so needs to be monitored if fever and inflammation do not resolve rapidly then corticosteroids will be required if there is persistent infections then antibiotics should be given following resolution of an acute infection, recurrence should be prevented with monthly injections of zathine penicillin

Question 53

what are risk factors for infective endocarditis?

Answer 53

congenital heart disease rheumatic fever valve disease prosthetic valves IVDU

Question 54

when should you suspect infective endocarditis?

Answer 54

it should be suspected in any child or adult with fever, malaise, raised ESR, unexplained anaemia or haematuria

Question 55

what are the most common causes of infective bacterial endocarditis?

Answer 55

``` streptococcus viridans - linked to poor dental hygiene staphylococcus aureus (common in IVDU) ```

Question 56

what are the clinical signs of infective endocarditis?

Answer 56

``` fever anaemia and pallor splinter haemorrhages in nailbed clubbing necrotic skin lesions changing cardiac signs splenomegaly neuro signs from cerebral infarction retinal infarcts arthritis/arthralgia haematuria ```

Question 57

how should you diagnose infective bacterial endocarditis ?

Answer 57

blood cultures should be taken before starting Abx detailed cross-section echo may confirm diagnosis but will not exclude diagnosis acute phase reactants are raised

Question 58

how should you treat bacterial endocarditis?

Answer 58

high dose IV Abx - penicllin/vancomycin for a min of 4-6 weeks

Question 59

what are some causes of heart failure in children?

Answer 59

``` large left to right shunt left sided obstructive lesion cardiomyopathy myocarditis endocarditis Kawasaki disease tachyarrhythmias ```

Question 60

what are the clinical features of heart failure in children ?

Answer 60

``` sweating breathless, tachypnoea, coughing, lung creps poor feeding, poor weight gain and FFT hepatomegaly cardiomegaly tachycardia, gallop heart rhythm ```

Question 61

what are some causes of acyanotic heart disease?

Answer 61

``` VSD ASD PDA pulmonary valve disease coarctation of the aorta aortic stenosis hypoplastic left heart syndrome hypertrophic obstructive cardiomyopathy dextrocardia ```

Question 62

what are some causes of cyanotic heart disease?

Answer 62

tetralogy of fallot transposition of the great arteries tricuspid atresia

Question 63

what are the principle causes of pericardial inflammation?

Answer 63

- Infections (coxsackie B , EBV, strept, TB, toxoplasmosis - connective tissue (RA, rheumatic fever, SLE, sarcoidosis - metabolic - hyperuricaemia, hypothyroidism - malignancy - radiotherapy

Question 64

what would an ECG show when there is pericarditis?

Answer 64

low voltage QRS complex

Question 65

what are the clinical features of pericarditis?

Answer 65

typically sharp pain which is exacerbated and exaggerated by lying down and relieved by sitting or leaning forward. Pain is often referred to left shoulder

Question 66

how is pericarditis managed?

Answer 66

analgesia anti-inflammatory drugs pericardiocentesis for pericardial effusion

Question 67

what might myocarditis be due to ?

Answer 67

infections Kawasaki drugs - Adriamycin connective tissue disease: SLE, RA, rheumatic fever, sarcoidosis

Question 68

what are the classifications of cardiomyopathy?

Answer 68

hypertrophic dilated restrictive

Question 69

what are the causes of dilated cardiomyopathy?

Answer 69

inherited secondary to metabolic disease direct result from a viral infection