Infant or child with a heart murmur

Question 1

Characteristic of innocent murmurs

Answer 1

Have no clinical significance
systolic
Localised
Soft
vary with position of the child
vary with respiration
Rest of CVS is normal.
- Venous hum - blowing continuous murmur in systole and diastole and heard below the clavicles, disappears on lying down.
- Pulmonary flow murmur- brief high-pitched murmur at second left intercostal space. Best heard with child lying down
- Systolic ejection murmur - short systolic murmur at left sternal edge or apex. Musical sound, Changes with child’s position. Intensified by fever, exercise and emotion

Question 2

Characteristic of pathological murmur

Answer 2

Pan systolic or diastolic
Are harsh or long
May radiate and have a thrill
Often have associated cardiac symptoms or signs
- Aortic stenosis
- Atrial septal defect
- pulmonary stenosis
- ventricular septal defect
- Coarctation of the aorta

Question 3

Congenital heart disease that present with a murmur

Answer 3

Pulmonary valve stenosis
Atrial septal defect
Ventricular septal defect
Coarctation of the aorta
Patent ductus arteriosus
Aortic valve stenosis
Tetralogy of Fallot
Transposition of great arteries

Question 4

Stage of embryology when heart is developed

Answer 4

3-8 wks, Insults in this time result in CHD

Question 5

Ix for CHD

Answer 5

Echocardiogram
ECG
CXR

Question 6

Signs of cyanotic heart disease

Answer 6

Blue mucous membranes

Nail beds and skin secondary to an absolute concentration of deoxygenated haemoglobin of at least 3g/dL

Question 7

Characteristic CXR finding in CHD

Answer 7

Boot shaped heart = tetralogy of ballot, tricuspid atresia
Egg shaped heart = transposition of great arteries
“Snowman” heart: total anomalous pulmonary venous return.

Question 8

Types of CHD

Answer 8

Acyanotic

• Left to right shunts - most common: Atrial septal defect (ASD) - clinical commonest, Ventricular Septal Defect (VSD) - commonest at birth, Patent Ductus Arteriosus (PDA)
• Obstructions: Coarctation of aorta (CoA) - Turners, Aortic stenosis, Pulmonic stenosis

Cyanotic - 5 T lesion

• Right to Left shunts: Tetralogy of Fallot (FT), Epstein’s anomaly
• Other: Transposition of great arteries, Total anomalous pulmonary venous drainage, Tricuspid atresia, Hypoplastic left heart syndrome,

Question 9

CHD that presents as Heart failure

Answer 9

VSD
ASD
PDA

Question 10

CHD that present with shock

Answer 10

Coarctation of aorta

Aortic valve stenosis

Question 11

CHD that present with cyanosis

Answer 11

Tetralogy of ballot

Transposition of the great arteries.

Question 12

CHD that typically present in the newborn period

Answer 12

Coarctation of the aorta
Transposition of the great arteries
Tetralogy of Fallot
Patent ductus arteriosus

Question 13

Types of ASD

Answer 13

Ostium primum - common in down syndrome
Ostium secundrum - most common 50-70%
Sinus venosus - entry at SVC entrance

Question 14

Symptoms and signs of ASD

Answer 14

Asymptomatic
Murmur
Grade 2/3 
Systolic murmur at 2rd left interspace
Widely split S2 and fixed.
Breathlessness
Tiredness on exertion
recurrent chest infection
HF
Pulmonary HTN 
Pulmonary vascular abnormalities

Question 15

Cx of ASD

Answer 15

Eisenmenger’s complex
infective endocarditis
Paradoxical immobilisation
Cardiac arrhythmias

Question 16

Ix findings in ASD

Answer 16

ECG - Right axis deviation, mild RVH, RBBB

CXR - Increase pulmonary vascular

Question 17

Tx for ASD

Answer 17

80-100%

Question 18

Types of VSD

Answer 18

Membranous 90%

Muscular

Question 19

Clinical presentation of VSD

Answer 19

Asymptomatic
Murmur: Harsh pan systolic at lower left sternal edge, May radiate over chest if large enough
Parasternal thrill
HR 4-6 week if lg
Large
breathlessness on feeding or crying
poor growth
recurrent chest infections

Question 20

Cx of VSD

Answer 20

Increase pulmonary HTN -> Eisenmenger’s syndrome

Question 21

Ix of VSD

Answer 21

CXR - increase pulmonary marking, cardiomegaly
ECG - RVH, LVH
Echo - confirms diagnosis

Question 22

Tx of VSD

Answer 22

Many close spontaneously (small)
Prevention of endocarditis
Large membranous defect and cardiac failure need medical tx for CHF and Surgical closure by 1 year

Question 23

Clinical presentation of PDA

Answer 23

Murmur: Continuous machinery murmur below left clavicle
bounding pulse
Asymptomatic
Apnoea or decreased HR episodes
Poor feeding
Accessory muscle use
Tachycardia
Hyperactive precordium
Wide pulse pressure

Question 24

Ix findings in PDA

Answer 24

ECG - LAE, LVH, RVH
CXR - mildly enlarged heart, increase pulmonary vasculature, prominent pulmonary artery
Echo - diagnostic test

Question 25

Tx for PDA

Answer 25

Premature - spontaneous closure or medical closure with indomethacin or ibuprofen: PGE2 antagonist only effective in premature. Restrict fluids blood transfusion if anaemic Treat HF - furosemide Surgery - if medical treatment fails, significant HF, ventilator dependence or prolonged failure to close. surgical ligation or by transcatheter occlusion device.

Question 26

Clinical presentation of coarctation of aorta

Answer 26

Mild HF Murmur - absent or systolic with late peak over apex, left axilla, left back Older children develop HTN severe Infant goes into shock when ductus arteriosus closes Key features - WEAK OR ABSENT FEMORAL PULSES - RADIAL FEMORAL DELAY Ass with turner’s syndrome (bicuspid aortic valve and aortic dissection.

Question 27

Ix of coarctation of aorta

Answer 27

ECG - infancy RVH, Childhood LVH | Echo - diagnostic

Question 28

Tx of coarctation of aorta

Answer 28

Immediately - IV prostaglandin E2 = keeps ductus arteriosus patent Surgery - Neonate for repair, Older children stunt or balloon to dilate.

Question 29

Clinical presentation of Aortic stenosis

Answer 29

- Mild - asymptomatic with murmur - ejection systolic on RUSB +/- ejection click, radiates to neck and A2 soft and delayed - Severe - shock or HF when duct closes - Older children Syncope decreased exercise tolerance dizziness exertion chest pain - peripheral pulse sml volume - Reduced BP - Palpable thrill at L sternal border and carotid artery

Question 30

Ix findings for aortic stenosis

Answer 30

CXR - prominent LV and ascending aorta ECG - LVH Echo

Question 31

Cx of aortic stenosis

Answer 31

Sudden death

Question 32

Tx of aortic stenosis

Answer 32

Severe - ballon valvoloplasty or open heart surgery | Avoid strenuous activity or sport = sudden death.

Question 33

Clinical presentation of pulmonary stenosis

Answer 33

``` Murmur short systolic ejection murmur Upper left chest Conducted to back Preceded by ejection click Thrill palpable in severe Wide split S2 on expiration mild - asymptomatic Severe - palpable in pulmonary area, CHF ```

Question 34

Ix of pulmonary stenosis

Answer 34

CXR - dilated pulmonary artery, severe - enlarge R atrium and ventricle Echo to diagnosis

Question 35

Tx of pulmonary stenosis

Answer 35

Severe - balloon valvuloplasty, usually successful.

Question 36

Causes of cyanotic heart disease

Answer 36

One big trunk - trunks arteriosus Two interchanged vessels - transposition of the great vessels Three leaflets - tricuspid atresia Four anatomical abnormalities - tetralogy of Fallot Five words - Total anomalous pulmonary venous return

Question 37

What is Tetralogy of Fallot

Answer 37

Causes - genetic and seen in fetal alcohol syndrome. Defective septum development - classic large VSD - venticular septum defect RVH (result) - Pulmonary infundibular stenosis Overriding aorta - aorta gets blood from both right and left

Question 38

Clinical features of tetralogy of fallot

Answer 38

intermittent Cyanosis since birth Clubbing Murmur - ejection systolic murmur, loud P2 Hyper cyanotic spells on exertion which are relieved by squatting down presents common at 2-4 months. most common cyanotic CHD beyond infancy

Question 39

Cx of tetralogy of Fallot

Answer 39

Subacute bacterial endocarditis - right to left shunt predisposes to infection

Question 40

Ix findings of tetralogy of Fallot

Answer 40

ECG - RVH | CXR - boot shaped heart and oligaemic lung fields, decreased pulmonary vasculature, right aortic arch

Question 41

Tx for tetralogy of Fallot

Answer 41

Mx of spells - O2, knee chest position, fluid bolus, morphine sufate, propanolol surgical correction at 2-3 months old or earlier if marked cyanosis. Protective factors if patients have PDA and ASD may be there (protective)

Question 42

Clinical features of transposition of great vessels

Answer 42

severe Cyanosis and acidosis since birth. Chronic heart failure Right sided ventricular hypertrophy Pulmonary hypertension IF VSD murmur - cyanosis = presents with CHF in first couple of weeks If no VSD = no murmur but marked cyanosis.

Question 43

Ix finding for transposition of great vessels

Answer 43

ECG- RAD, RVH or normal | CXR - Egg shaped heart with narrow mediastinum

Question 44

Tx for transposition of great vessels

Answer 44

Emergency Prostaglandin infusion ventilatory circulatory support Surgery - Requires atrial septostomy urgently followed by definitive switch operation within the first couple of weeks- where two great vessels are switched over and coronary arteries are reconnected to the new aorta. Prognosis Die in the first month is not treated with surgery.

Question 45

Neonatal circulation

Answer 45

Placenta -> umbilical vein -> ductus veniosus ->IVC ->RA -> foramen ovale or ductus arteriosus to systemic circulations -> descending aorta -> common iliac -> to umbilical arteries

Question 46

Diagnostic criteria for Acute rheumatic fever

Answer 46

``` 2 major or 1 major & 2minor +evidence of preceding GAS infection. Major manifestations Carditis- excluding subclinical evidence of rheumatic valve disease of Echo Polyarthritis Sydenham Chorea Erythema marginatum Subcutaneous nodules Minor Fever ESR >/= 30mm/hr or CRP >/= 30mg/L Prolonged P-R interval on ECG Polyarthralgia or aseptic mono-arthritis ```

Question 47

Diagnostic criteria for actor rheumatic fever in high risk groups

Answer 47

2 major or 1 major & 2minor +evidence of preceding GAS infection. Major Carditis - including subclinical evidence of rheumatic valve disease on echocardiogram Polyarthritis, aseptic mono-arthritis or polyarthralgia Sydenham Chorea Erythema marginatum Subcutaneous nodules Minor Fever ESR >/= 30mm/hr or CRP >/= 30mg/L Prolonged P-R interval on ECG

Question 48

Ix for rheumatic fever

Answer 48

``` Echo WCC ESR CRP Blood cultures if febrile ECG Throat swab Anti-streptolysin serology (anti-streptolysin O, Anti-DNase B titres) ```

Question 49

Mx of rheumatic fever

Answer 49

Regular secondary prevention treatment for people at risk of recurrent ARF First line = Benzathine penicillin G - 1200 000 U (body wt >/= 20 kg) IM 4 weekly or 3 weekly in selected groups. second line if IM routine not possible or refused Phenoxymethylpenicillin (penicillin V) 250mg Oral Twice daily If penicillin allergy = Erythromycin 250mg oral twice daily Duration of treatment all people with ARF or RHD for minimum of 10 years after most recent episode of ARF or until age 21 yrs (which ever is longer) Status after initial period has elapsed No RHD or mild RHD = discontinue at that time Moderate RHD: continue until age 35 yr Severe RHD: continue until age 40 yrs or longer Acute management - Bed rest - anti-inflammatory drugs e.g. aspirin - Corticosteroids 2-3 weeks - diuretics/ACEi if HF - Antibiotics eg penicillin V for 10 days