Cardio

Question 1

How does the antenatal heart and lungs work?

Answer 1

the lungs are filled with fluid and there is a very high pulmonary vascular resistance

lungs are non-functional

Blood comes from umbilical vein into fetal IVC -> Right atrium

not much blood can reach lungs through pulmonary artery

there are therefore shunts to help mixing of blood

1. Ductus arteriosus - between aorta and pulmonary artery
2. Foramen ovale - between left and right atrium

Question 2

what are cyanotic heart diseases ? list some - how do we tell the difference

Answer 2

cause the baby to become cyanosed

1. Tetralogy of fallot
   
   • presents within months
   • more likely to have murmur!
   • Xray; boot shaped heart
2. Transposition of the great arteries
   
   • presents within days
   • no response of Pa02 to oxygenation (hyperoxia test/ (nitrogen washout test)
   • Xray; egg on a string

Question 3

how do we ivx cyanotic heart diseases?

Answer 3

Antenatally:
- may be identified on anomaly scan 20 wks

Post natally:

• Physical exam; murmur, cyanosis, signs of heart failure
• Xray
  
  • Echocardiogram
  • Hyperoxia test; give high flow O2, will note some response in TOF

Question 4

How do we mx cyanotic heart diseases?

Answer 4

1. Maintain Patent ductus arteriosus until corrective surgery:
   
   • Give IV prostaglandin infusion
2. Surgery

Question 5

What are the acyanotic heart diseases?

how do they present?

Answer 5

Atrial septic defect:
- asymptomatic
- may hear ESM - Ejection systolic murmur @ left sternal edge
Fixed splitting S2

Ventricular septal defect:

• asymptomatic
• pansystolic murmur at LSE aka holosystolic
• rx: spontaneous resolution / surgery

Atrioventricular septal defect

• down syndrome
• pulmonary vascular congestion
• pulmonary htn

Patent ductus arteriosus

• normally closes within 1 month of birth but in PDA it doesnt
• continuous murmur under left clavicle
• give NSAIDs; inhibits prostaglandin

Question 6

what are the consequences of congenital heart disease?

Answer 6

-

Question 7

what factors increase the risk of congenital HD?

Answer 7

Maternal → Rubella infection; DM; Warfarin; FAS - fetal alcohol syndrome

Chromosomal → Down’s; DiGeorge; Edward’s; Patau’s; Turner; William’s; Noonan’s

Question 8

what are the most common congenital heart lesions that cause the following presentations:

1. breathless child
2. blue/cyanotic child
3. breathless and blue

Answer 8

• Left-to-right shunts (breathless child)
○ Ventricular septal defect 30%
○ Persistent arterial duct 12%
○ Atrial septal defect 7%

• Right-to-left shunts (blue/cyanotic child)
○ Tetralogy of Fallot
○ Transposition of the great arteries

• Common mixing (breathless and blue)
○ Atrioventricular septal defect (complete)

Question 9

what are the most common congenital heart lesions that cause the following presentations:

1. asymptomatic with a murmur
2. collapsed with shock

Answer 9

1• Outflow obstruction in a well child (asymptomatic with a murmur)
○ Pulmonary stenosis
○ Aortic stenosis

2• Outflow obstruction in a sick neonate (collapsed with shock)
○ Coarctation of the aorta

Question 10

what is the MOST COMMON presentation of congenital heart disease?

Answer 10

heart murmur

Question 11

what are the causes of murmur in neonates?

Answer 11

Vast Majority:
- Normal benign/innocent aka flow murmurs

- The s's of innocence → 
Soft - eg does not radiate anywhere
Systolic, 
Asymptomatic, 
left Sternal edge - doesnt rule out unless all other features are benign 
Sensitive - to changes in position

so a diastolic murmur is always pathological

Question 12

which CHD present:

A. within first few hours?
B. within first few days?

Answer 12

A• First few hours → pulmonary or aortic atresia, critical stenosis, hyperplastic heart syndrome

B• First few days → TGA, ToF, large PD Arteriosus In premature contraction

Question 13

which CHD present:

• First few weeks
• First few months

Answer 13

A• First few weeks → AS, coarctation

B• First few months → any L to R shunt as pulmonary resistance falls
• Majority of murmurs in paeds are innocent

Question 14

What is EISENMENGER syndrome?

what is. the aetiology?

Answer 14

Eisenmenger’s syndrome is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect (typically by a ventricular septal defect, atrial septal defect, or less commonly, patent ductus arteriosus)

causes pulmonary hypertension, pulmonary congestion, increased resistance in pulmonary artery (thickened walls) and increased Right heart pressure

this leads to eventual reversal of the shunt into a right-to-left shunt (as rt pressure exceeds the left) - around 10-15years of age

this leads to systemic stigmata including:
cyanosis, clubbing, polycythaemia

this happens because the right to left shunt leads to more de-oxygenated blood being pumped around the body = reduced cell function and blue tinge/cyanosis of skin.

Question 15

what is the treatment of EISENMENGER syndrome?

Answer 15

Ideally treat heart defect before R-L shunt develops

After pulmonary hypertension is sufficient to reverse the blood flow through the defect, however, the maladaptation is considered irreversible, and a heart–lung transplant or a lung transplant with repair of the heart is the only curative option.

Transplantation is the final therapeutic option and only for patients with poor prognosis and quality of life.

Question 16

what are the possible signs and sx of EISENMENGER syndrome?

Answer 16

Note: when it is a LR shunt, it is not cyanotic disease until reversal.

Cyanosis (a blue tinge to the skin resulting from lack of oxygen)
High red blood cell count - polycythaemia
Clubbing
Fainting (also known as syncope)
Heart failure
Abnormal heart rhythms

Bleeding disorders
Coughing up blood
Iron deficiency
Infections (endocarditis and pneumonia)
Kidney problems
Stroke - if a peripheral embolus comes into rt side -> left -> brain, instead of becoming a pulmonary embolus

Gout (rarely) due to increased uric acid resorption and production with impaired excretion
Gallstones

Question 17

what is the prognosis of EISENMENGER syndrome?

Answer 17

With increased fetal screening, Left-right shunts can be fixed early meaning that patients wont later develop eisenmengers

If they do develop it, they. usually die in their 40s/50s with heart failure.

variable post transplant

Question 18

what are the Left to right shunts?

Answer 18

These are:
• atrial septal defects (ASDs)
• VSDs
• persistent ductus arteriosus (PDA).

Question 19

what are the types of ATRIAL SEPTAL DEFECT (ASD)?

Answer 19

There are two main types of ASD:
• Ostium Secundum ASD (80%)
• Partial atrioventricular septal defect (AVSD or ostium primum - 20%)

The secundum ASD:
- defect in the centre of the atrial septum involving
the foramen ovale.

Partial AVSD:
- defect of the Atrioventricular septum (between the right atrium and the left ventricle) ->

1. inter-atrial communication between the bottom
   end of the atrial septum and the atrioventricular
   valves (primum ASD)
2. Left AV valve has 3 leaflets & leaks (regurgitant valve).

Question 20

how does ATRIAL SEPTAL DEFECT (ASD) present?

Answer 20

Both types present with similar symptoms and signs

• None /asymptomatic (commonly)
• Recurrent chest infections/wheeze
• Arrhythmias (fourth decade onwards)

• Ejection Systolic murmur (due to ↑ pulmonary valve flow)

+ fixed widely split 2nd HS (you hear Lub-du-dub, instead of Lub-dub)

• AVSD gives an apical pansystolic murmur
from atrioventricular valve regurgitation.

Question 21

what is the aetiology of fixed widely split 2nd HS in ASD?

Answer 21

pulmonary valve closes slightly later than aortic valve so you hear Lub-du-dub, instead of Lub-dub

due to the right ventricular stroke volume being equal in both inspiration and expiration.

Question 22

How do we ivx ATRIAL SEPTAL DEFECT (ASD) ?

Answer 22

IX: CXR, ECG, Echo

• CXR → cardiomegaly, large pulmonary arteries, ↑ pulmonary markings
• ECG → partial RBBB or RAD due to RV enlargement

superior QRS axis position in AVSD

• Echo → see anatomy

Question 23

How do we manage ATRIAL SEPTAL DEFECT (ASD) ?

why do we treat?

Answer 23

→ if significant* cardiac catheterisation (cath lab) with insertion of occlusion device (3yo-5yo)

*(large enough to cause right ventricle dilation)

AVSD: Surgery / surgical correction needed
- if complete, repair at 3-6 months

reasons to treat:
to prevent right heart failure and arrhythmias in later life.
and of course Phtn,eisenmenger etc

Question 24

what are the complications of L-R shunts eg ASD, VSD, PDA?

Answer 24

causes pulmonary hypertension, pulmonary congestion, increased resistance in pulmonary artery (thickened walls) and increased Right heart pressure

Then subsequent:
Eisenmenger
Heart failure
Arrhythmias etc

Risk of Bacterial endocarditis - prevent by good dental hygiene

Question 25

what are the types of VSD?

Answer 25

There is a defect anywhere in the ventricular septum 1. Peri-Membranous - (adjacent to the tricuspid valve) 2. Muscular - (completely surrounded by muscle). They can most conveniently be considered according to the size of the lesion: Small (<3mm, diameter smaller than aortic valve) Large (>aortic valve)

Question 26

How do small and large VSDs present?

Answer 26

Small (<3mm, diameter smaller than aortic valve) o Asymptomatic, loud PSM (pansystolic) at LLSE o Quiet pulmonary 2nd sound o CXR and ECG normal Large (>aortic valve) o Sx: HF + SOB and failure to thrive after 1wo o Sx: Recurrent chest infections o Signs: ↑ HR ↑ RR, Heptatomegaly; 2 murmurs: Soft PSM (pansystolic) - quiter than in small vsd, Apical mid-diastolic murmur o Loud pulmonary HS 2 (due to increased pulmonary pressure)

Question 27

How do we IVX VSDs?

Answer 27

Remember small VSD- cxr, ecg = normal • CXR → cardiomegaly, enlarged pulmonary arteries, increased pulmonary vascular markings + pulmonary oedema * ECG → biventricular hypertrophy by 2months * Echo → anatomy, haemodynamic effects, pulmonary HTN

Question 28

how do we mx VSDs?

Answer 28

Large VSDs Treat HF with diuretics + captopril (ACE-I). (note this is not done in ASD) Surgery at 3-6m to prevent permanent lung damage.* * note there will deffo be a degree of Phtn in these kids - surgery is to prevent Eisenmenger + permanent issues. Again - small VSDs can spontaneously patch up Risk of Bacterial endocarditis - prevent by good dental hygiene increased calories

Question 29

How does PDA present?

Answer 29

Continuous murmur under L. clavicle • Increased pulse pressure, collapsing or bounding pulse • If large will show signs of growth failure and pulmonary HTN

Question 30

How do we IVX and MX PDA?

Answer 30

• CXR and ECG → normal if small, if large, findings same as VSD (cardiomegaly and enlarged pulmonary arteries) • Echo → to distinguish PDA and VSD MX: Use of coil or occlusion device via cardiac catheter at 1 year old. Risk of Bacterial endocarditis - prevent by good dental hygiene

Question 31

what are the components of TETRALOGY OF FALLOT (TOF)?

Answer 31

[1] large VSD [2] overriding aorta - sits midway, so receives blood from both left and right ventricles. [3] sub-pulmonary stenosis (RV outflow obstruction) and resulting 4. RV hypertrophy

Question 32

how does TETRALOGY OF FALLOT (TOF) present?

Answer 32

R-L shunt MOST common cause of cyanotic congenital heart disease • Classic presentation → cyanosis, hypercyanotic spells, squatting during exercise (Exercise = more deoxygenated blood = more cyanosis as poor oxygenation due to numerous shunts. Squats increase systemic resistance-> more blood shunted from aorta to pulmonary artery/lung = blood is oxygenated ) Presents 1st week of life • ‘Tet spells’ → quick increase in cyanosis, associated with irritability or inconsolable crying, SOB, pallor. THIS is an EMERGENCY - occur becuase there is sudden increase in deoxygenated blood shunted into aorta (levels of mixing always change) • Signs → clubbing, loud harsh ejection systolic murmur at LSE from day 1 of life

Question 33

how do we ivx TETRALOGY OF FALLOT (TOF)?

Answer 33

CXR → small, boot-shaped heart with uptilted apex. Pulmonary artery ‘bay’, concave cavity on left border • ECG → may develop RV hypertrophy when older • Echo → shows cardinal features but cardiac catherization may be required to show detailed anatomy of the coronary arteries.

Question 34

How is TETRALOGY OF FALLOT (TOF) managed?

Answer 34

1• Hypercyanotic spells usually self-limiting: • If prolonged, 15mins+, give sedation and pain relief – morphine, IV propranolol (vasoconstrict), IV fluids, bicarbonate (to correct acidosis) 2. Surgery at 6m (close VSD and relieve R outflow obstruction) - definitive 3. If very cyanosed neonate, - insert BT shunt !! to increase pulmonary blood flow between subclavian and pulmonary (Blalock-Taussig) - this sends the mixed blue/red blood back to lung to be better oxygenated.

Question 35

what are the Right-to-left shunts and how do they present?

Answer 35

These are: • tetralogy of Fallot • transposition of the great arteries. Presentation is with cyanosis (blue, oxygen satura- tions ≤94%, or collapsed), usually in the first week of life.

Question 36

What doese the nitrogen washout test involve?

Answer 36

Hyperoxia (nitrogen washout) test The test is used to help determine the presence of heart disease in a cyanosed neonate. The infant is placed in 100% oxygen (headbox or ventilator) for 10 minutes. If the right radial arterial partial pressure of oxygen (PaO2) from a blood gas remains low (<15 kPa, 113 mmHg) after this time, a diagnosis of ‘cyanotic’ congenital heart disease can be made if lung disease and persistent pulmonary hypertension of the newborn have been excluded.

Question 37

what does TRANSPOSITION OF GREAT ARTERIES involve? presentation?

Answer 37

• Aorta is connected to RV and pulmonary artery is connected to LV • Two parallel circulations – need an ASD/VSD/PDA connection in order to be compatible with life Presentation: • Cyanosis – may be profound and life-threatening • Presentation on d2 of life when DA closes • Large, single 2nd heart sound, usually no murmur

Question 38

what is the ivx and mx of TOGA?

Answer 38

Ix: CXR, ECG, Echo • CXR → normal upper mediastinum, egg on string appearance of cardiac shadow (quite globular), increased pulmonary vascular markings • ECG → usually normal • Echo → essential to demonstrate abnormal connections Management → 1. maintain patency of DA with prostaglandin infusion, 2. atrial septostomy (life-saving in 20%) – balloon catheter. tears foramen ovale flap valve to allow mixing. - Transfer to cardiac centre The above are done in preparation/whilst awaiting: 3. Surgery (arterial switch) in first few days of life: - where PA and aorta are moved to normal position.

Question 39

what is TRICUSPID ATRESIA?

Answer 39

Tricuspid valve doesnt develop This means no blood going to RV in-utero, so RV muscle doesnt develop and remains a sack They need an ASD in order to be compatible with life When born - little blood going to lungs curtesy of VSD - lft ventricle pumps blood through and up into PA

Question 40

how is TRICUSPID ATRESIA managed?

Answer 40

Early palliation with Blalock-Taussig shunt (between subclavian and pulmonary artery) to maintain blood supply to lungs if cyanosed • Pulmonary artery banding to reduce pulmonary blood flow if breathless • Complete corrective surgery not possible with most as there is only 1 functioning ventricle: surgery: connects SVC&IVC to pulmonary artery - relieves cyanosis

Question 41

How does Aortic Stenosis present?

Answer 41

• Can involve fusion of 1-3 aortic valve leaflets Clinically: • Asymptomatic murmur unless: • If severe → decreased exercise tolerance, chest pain, syncope • If critical and duct-dependent, neonates may present with severe heart failure, leading to shock • Small volume, slow-rising pulses, carotid thrill, ejection systolic murmur max at upper right sternal edge, rad to neck, delayed and soft aortic sound, apical ejection click

Question 42

how do we. ivx and mx Aortic Stenosis?

Answer 42

• CXR → normal or prominent LV with post- stenotic dilatation of ascending aorta • ECG → may show LVH - left ventricular hypertrophy Management → regular clinical, echo assessment to decide whether or not to intervene, may require balloon valvotomy. Eventual aortic valve replacement

Question 43

how does PULMONARY STENOSIS present?

Answer 43

Clinically: • Most asymptomatic, dx is clinical • Neonate with critical pulmonary stenosis, who are duct dependent will present in first few days of life with cyanosis • Signs → ejection systolic murmur at upper left sternal edge +/- thrill, ejection click, if severe may get heaves (RV impulse)

Question 44

How do we ivx and mx PULMONARY STENOSIS?

Answer 44

IX: CXR, ECG • CXR → normal or post-stenotic dilataion of pulmonary artery • ECG → evidence of RV hypertrophy (upright T wave in V1) Management → trans-catheter balloon dilatation

Question 45

What is COARCTATION OF AORTA (COA) and how does it present?

Answer 45

• Due to arterial duct tissue encircling the aorta at point of insertion of the duct (aortic isthmus) • When the duct closes, aorta constricts, causes severe LV outflow obstruction Clinically: • Examination normal on first day of life - Neonates present with acute circulatory collapse at 2d of age when the duct closes - > shock + poor perfusion * Sick baby with severe heart failure * Absent femoral pulses /diminished peripheral pulses * Severe metabolic acidosis

Question 46

how do we ivx and mx COARCTATION OF AORTA (COA)?

Answer 46

Clinical diagnosis CXR → cardiomegaly from heart failure and shock - Figure of 3 sign ECG is normal Management → - Prostaglandin infusion to maintain duct latency -> surgical repair: - resect constricted segment + anastomoses of vessels

Question 47

what effects does COARCTATION OF AORTA (COA) have on the heart?

Answer 47

LV hypertrophy LV HTN Heart failure - reduced peripheral perfusion

Question 48

what are the associations of COA?

Answer 48

Seen in 15-20% Turners syndrome Bicuspid aortic valve VSD

Question 49

what is HYPOPLASTIC LEFT HEART SYNDROME?

Answer 49

When left heart and all associated structures - mitral, aortic valve, aorta are small/underdeveloped. this means cant pump blood to the body Survives due to ASD & PDA which allows oxygenated blood from left to reach the right and be pumped up - through PDA into descending Aorta. This is called a Duct-dependent systemic circulation TROUBLE: PDA has to close after birth.

Question 50

how do we ivx and mx HYPOPLASTIC LEFT HEART SYNDROME?

Answer 50

Antenatal US screen. Closure of PDA = 1. Profound acidosis 2. Rapid cardiovascular collapse if no flow through left side 3. Weakened or absent ALL peripheral pulses Since they are okay initially, it can be missed at birth. 1. Prostaglandin infusion 2. Surgery - Norwood procedure followed by Glenn or hemi-Fontan at 6m and again (Fontan) at 3y

Question 51

Outflow obstruction can present with a sick or a well child. what are the differentials for both?

Answer 51

Well: 1. Aortic stenosis 2. Pulmonary stenosis Sick (HF+shock): 1. Coarctation of the aorta 2. Hypoplastic left heart syndrome 3. Interruption of the aortic arch

Question 52

which are the Left heart outflow obstruction in the sick infant – duct-dependent lesions ? how do we differentiate and what is the OVERALL mx?

Answer 52

1. Coarctation of the aorta 2. Hypoplastic left heart syndrome 3. Interruption of the aortic arch All have Circulatory collapse COA: Absent femoral pulses HLHS: All peripheral pulses absent IOA: Absent femoral pulses and absent left brachial pulse Mx of all: Maintain ABC - RESUSCITATE Prostaglandin infusion

Question 53

what are the differentials for circulatory collapse in a kid? if suspecting this in initial presentation, how do we manage?

Answer 53

sepsis metabolic cardiac (duct dependent conditions, cardiomyopathy, arrhythmias) cerebral bleed MX: Maintain ABC - RESUSCITATE Prostaglandin infusion

Question 54

what is sinus rhythm?

Answer 54

Heart contractility generated by the sinus node sinus tachycardia - example of cause = fight or flight situation sinus bradycardia - rest

Question 55

what is sinus arrhythmia ?

Answer 55

A sinus arrhythmia is an irregular heartbeat that's either too fast or too slow. This can be associated with breathing or other factors. On an ECG you will see variation in R-R interval

Question 56

what does sinus arrhythmia mean in kids?

Answer 56

Sinus arrhythmia is normal in children and detectable as cyclical change (up to 30bpm) in HR with respiration – acceleration on inspiration and slowing on expiration because their pace maker/ baroreceptors are very sensitive

Question 57

How does sinus arrhytmia present ?

Answer 57

no symptoms just an ecg pattern sign of good heart health

Question 58

what is a Supraventricular tachycardia?

Answer 58

its an abnormally fast heart rhythm arising from improper electrical activity in the upper part of the heart.

Question 59

what are the types of SVT?

Answer 59

Thhere are 4 main types: AVNRT: atrial fibrillation, paroxysmal supraventricular tachycardia (PSVT), atrial flutter, and AVRT: Wolff–Parkinson–White syndrome. There is a re-entry circuit originating in the atrtioventricular nodes - due to accessory pathways

Question 60

how does SVT present?

Answer 60

This is the most common childhood arrhythmia. The heart rate is rapid, between 250–300 beats/min. 1. presents with symptoms of heart failure in the neonate or young infant: - poor cardiac output and pulmonary oedema. 2. cause of hydrops fetalis and intrauterine death also possibly: palpitations, feeling faint, sweating, shortness of breath, or chest pain

Question 61

how do we ivx SVT?

Answer 61

``` ECG: -Narrow complex tachycardia (<120 ms / 3 smallsquares) +/- delta wave in WPW, T-wave inversion due to ischemia -250–300 beats/min. ``` • Do an echo – but rarely structural cause

Question 62

How do we mx SVT?

Answer 62

If paroxysmal: Often stops spontaneously • 1st - Vagal stimulating manoeuvres – valsalva manuvre, carotid sinus massage, ice pack on face, works in 80% - perform all with continuous ecg monitoring If the effects of reflex vagal stimulation are transient or ineffective: • 2nd - IV adenosine – safe and effective to induce AV block and terminate tachycardia by breaking re-entry circuit, - give incrementally in increasing doses - give whilst running continuous ECG -Do not use Adenosine in irregular rhythm If cant use adenosine/ineffective: 3rd - IV Verapamil (generally avoided unless specialist) If manuvres + drugs dont work: * 4th - DC cardioversion * 5th - if reccurrent; maintain with flecainide or sotalol

Question 63

what are differences in mx if not paroxysmal SVT?

Answer 63

usually start with Adenosine no manuvres • Digoxin is contraindicated in WPW - can try for atrial fibrillation and flutter * Can stop TX at 1yo * If relapse or at risk – use radiofrequency ablation or cryoablation of accessory pathway

Question 64

what causes congenital complete heart block?

Answer 64

Anti RO, La This antibody appears to prevent normal development of the electrical conduction system in the developing heart, with atrophy and fibrosis of the atrioventricular node.

Question 65

How does congenital complete heart block?

Answer 65

It may cause fetal hydrops, death in utero and heart failure in the neonatal period. However, most remain symptom free for many years, but a few become symptomatic with presyncope or syncope.

Question 66

How is congenital complete heart block treated?

Answer 66

Surgery - endocardial pacemaker

Question 67

how does long QT syndrome present?

Answer 67

Long QT syndrome may be associated with sudden loss of consciousness during exercise, stress or emotion, usually in late childhood. It may be mistakenly diagnosed as epilepsy. It is a commom cause of sudden death! Ivx for this if family history of sudden death.

Question 68

What is the aetiology of long QT syndrome?

Answer 68

Inheritance is autosomal dominant; there are several phenotypes. It has been associated with erythromycin therapy, electrolyte disorders and head injury.

Question 69

what is rheumatic fever?

Answer 69

A multisystemic inflammatory reaction that can take place 2-4 weeks after a Group A strep throat infection or Strep pyogenes

Question 70

what si the aetiology of rheumatic fever?

Answer 70

Group A β-haemolytic streptococcus, now rare in developed world • Children age 5-15y • Long term fibrosis and scarring leads to mitral stenosis other valves can also be affected .The heart is involved in about half of the cases. Damage to the heart valves, known as rheumatic heart disease (RHD), usually occurs after repeated attacks but can sometimes occur after one.[1] The damaged valves may result in heart failure, atrial fibrillation and infection of the valves.

Question 71

how does rheumatic fever present? WHAT is diagnostic criteria?

Answer 71

Modified Jones criteria Major criteria are as follows: Carditis (clinical or echocardiographic diagnosis) 50% Polyarthritis (not monoarthritis) 80%* Chorea (rare in adults) Erythema marginatum (uncommon; rare in adults) Subcutaneous nodules (uncommon; rare in adults) Minor criteria are as follows: Polyarthralgia (cannot count arthritis as a major criterion and arthralgia as a minor criterion) Fever exceeding 38.5°C Elevated ESR (>60 mm/hr) or CRP level (>3 mg/L) Prolonged PR interval aka 1st degree heart block Fever, multiple painful joints, polyarthritis (tender joints, swelling), *migratory - it moves Need 2 major or 1 major + 2 minor + evidence of preceding group A strep infection (↑ ISO titre or other streptococcal Abs or group A strep on throat culture)

Question 72

what are the complications of rheumatic fever?

Answer 72

heart failure, atrial fibrillation Endocarditis - infection of the valves Sydenhams chorea - (2-6 months later) Pericarditis (endocarditis, myocarditis, pericarditis),

Question 73

how do we mx rheumatic fever?

Answer 73

Acute episode – 2. If confirmed :1st - Antibiotics - Benzylpenicillin or erythromycin + high dose aspirin if arthiritis + diuretic & ACE inhibitor if heart failure +/- steroids if SEVERE carditis anticonvulsants if sever chorea Rest and Analgesia (for arthiritis) (paracetamol, codeine if >12) (unconfirmed/generally) • After resolution – prevent reoccurrence with monthly injections of benzylpenicillin prophylaxis

Question 74

when can we suspect infective endocarditis in children? what is the aetiology?

Answer 74

• Increased risk with congenital HD -except secundum ASD • Suspect in any child with sustained fever, malaise, increased ESR, unexplained anaemia or haematuria if hx of rheumatic fever ``` The most common causative organism is α- haemolytic streptococcus (Streptococcus viridans). ```

Question 75

what may be some clinical signs of infective endocarditis in kids?

Answer 75

* Fever * Anaemia and pallor * Splinter haemorrhages in nailbed * Clubbing (late) * Necrotic skin lesions (Fig. 18.21) * Changing cardiac signs * Splenomegaly * Neurological signs from cerebral infarction * Retinal infarcts * Arthritis/arthralgia * Haematuria (microscopic).

Question 76

how do we ivx infective endocarditis in kids?

Answer 76

Diagnosis with multiple blood cultures (before ABX) and Echo to identify vegetations

Question 77

how do we mx infective endocarditis in kids?

Answer 77

1. High dose Penicillin in combo with aminoglycoside (gentamicin or streptomycin) for 6w IV. 2. surgical removal of infected prosthetic material eg prosthetic valves, shunts etc. (bcos abx may not give complete eradication in this case) Advice: The most important factor in prophylaxis against endocarditis is: - good dental hygiene that should be strongly encouraged in all children with congenital heart disease along with avoidance of: - body piercing - tattoos.

Question 78

what are the signs and symptoms of heart failure in kids?

Answer 78

• Signs of heart failure: SOB, poor feeding, recurrent chest infections, fatigue • Symptoms of heart failure: poor weight gain, ↑RR, ↑ HR, murmur, gallop rhythm, signs of venous congestion, enlarged heart, hepatomegaly, cool peripheries, insufficient CO, respiratory distress, pallor, FTT

Question 79

how does cardiac function change in Heart failure?

Answer 79

Systolic: ↓ CO, ↓ Contractility, ↑ afterload, ↑ SVR • Diastolic: ↓ ventricular compliance; ↓ ventricular filling • Chronic: myocardial ischemia (from cytotoxic mechanisms or ↑ apoptosis - ↑ fibroblasts, ↑ dilatation, ↑ afterload

Question 80

how do we manage Heart failure?

Answer 80

1a. Reduce preload – DIURETICS – frusemide or thiazides 1b• Increase contractility with INOTROPES – digoxin, dopamine, dobutamine, milrinone • Reduce afterload – ACEI, ARB • Monitor and optimise electrolytes, anaemia, nutrition & growth

Question 81

what is Henoch schonlein purpura? Aetiology?

Answer 81

Most common vasculitis of childhood – affects the SMALL vessels • Boys 3-10y, peaks in winter, often preceded by URTI!! • IgA and IgG complex and deposit in organs activating complement

Question 82

How does HSP present? Henoch

Answer 82

classic tetrad of : 1.Rash - palpable purpura (extensor surfaces of legs, arms, buttocks, ankles; initially urticarial then becomes maculopapular, spares trunk) 2. Abdominal pain - Colicky abdo pain – haematemesis, melena, intussusception 3. Arthritis/arthralgia - joint pain and swelling (knees and ankles) 4. Glomerulonephritis

Question 83

what ivx to do in HSP? results?

Answer 83

Urinalysis: casts, proteinuria, RBCs 24 hr urine might be elevated if renal involvement ---- skin/renal biopsy - IgA deposition (in the mesangial region) ultrasound - if thinking intussuception etc

Question 84

How do we manage HSP?

Answer 84

with joint pain alone 1st line – analgesics with severe oedema or scrotal involvement 1st line – oral corticosteroids eg Prednisolone with severe abdominal pain 1st line – corticosteroids Adjunct – surgical consultation with rapidly progressive nephritis 1st line – corticosteroids plus immunosuppressants Prednisolone + Cyclophosphamide or Azathioprine If renal failure: dialysis / transplant

Question 85

The following conditions are associated with which CHDs: Edward’s; Patau’s;

Answer 85

Edward’s; Complex - range Patau’s; complex in these conditions 60-80% of time they present with heart defect

Question 86

The following conditions are associated with which CHDs: Turner; William’s; Noonan’s

Answer 86

Turner; AS -Aortic valve stenosis, COA - coarctation of the aorta (15% of time), bicuspid aortic valve William’s; Supravalvular aortic stenosis, peripheral pulmonary artery stenosis (85% of time) Noonan’s: Hypertrophic cardiomyopathy, ASD/ atrial septal defect, pulmonary valve stenosis (50%)

Question 87

The following conditions are associated with which CHDs: Downs syndrome

Answer 87

Downs syndrome; Atrioventricular septal defect (AVSD), | followed by VSD

Question 88

3 main septic risk factors in neonates?

Answer 88

GBS Prolonged rupture of membranes Chorioamnionitis