CARDIOLOGY

mohammed Alrasheed
PEADIATRICS
 Topic Page number

Congenital Heart Diseases (CHD)

Acyanotic Congenital Heart Diseases (ACHD)

Cyanotic Congenital Heart Disease (CCHD)

Pharyngitis

Acute Rheumatic Fever (ARF)

Infective endocarditis

Heart failure

1 | Pediatrics Cardiology
 Congenital Heart Diseases (CHD)
• Incidence
• 8 per 1000 live born infants have significant cardiac malformation
• The commonest CHD are VSD (30%), PDA (12%), and ASD (7%)
• About 10-15 % have complex lesions with more than one cardiac abnormality

• Risk Factors:
1. Genetic predisposition suggested by family history of CHD
2. Exposures during pregnancy e.g.
o Drugs e.g. warfarin, anticonvulsants, alcohol
o Diseases e.g. maternal rubella (PDA), Maternal Diabetes Mellitus
3. Chromosomal e.g.
o Down syndrome: VSD, mitral regurgitation
o Turner syndrome: Aortic stenosis, coarctation of aorta
o Williams syndrome: Supra valvular aortic stenosis, pulmonary stenosis, hypercalcemia
o Di George syndrome: TOF with Hypocalcemia (convulsion)
o Noonan syndrome: pulmonary stenosis

• Presentation:
• Coincidental; accidental discovery of a murmur in an asymptomatic infant
• Cardiac failure /Cardiogenic shock e.g. in neonate (critical aortic stenosis and severe coarctation) or in
infants (CHD with high pulmonary flow)
• Congestive pulmonary symptoms e.g. CHD with high pulmonary flow
• Cyanosis e.g. congenital cyanotic heart diseases
• Cardiomegaly detectable clinically or during routine chest radiograph
• Certain anatomic diagnosis may not be made by physical examination or chest x ray or ECG alone, so
echocardiography is the mainstay of diagnostic imaging

• Recently:
• Antenatal fetal anomaly ultrasound screening Can diagnose up to 70 % of significant lesions, allowing
earlier diagnosis and planning of management
• Important for any fetus with an increased risk e.g. Down syndrome
• MRI allows 3-dimensional imaging of complex CHD, assessment of hemodynamics, assists interventional
cardiology and reduces the need for cardiac catheterization.

2 | Pediatrics Cardiology
 Acyanotic Congenital Heart Diseases (ACHD)
• Definition:
• ACHD constitutes 80% of all CHD

• Classification:
• ACHD with left to right shunt (Potentially cyanotic CHD)
1. Ventricular septal defect (VSD)
2. Atrial septal defect (ASD)
3. Patent ductus arteriosus (PDA)
• ACHD without shunt
1. Obstructive lesions e.g. Aortic coarctation, Aortic stenosis, Pulmonary stenosis
2. Non obstructive lesions e.g. Dextrocardia, Mitral valve prolapse

• General clinical features

• ACHD with left to right shunt (Potentially cyanotic CHD
1. Degree of the left to right shunt, and consequently clinical manifestations is dependent on:
a. Size of the defect.
b. Pressure gradient across the defect
2. Manifestations of high pulmonary blood flow
a. Poor feeding (sweating and tachypnea) in babies, exercise intolerance and easy
b. fatigability in children
c. Recurrent chest infections & chest wheezes.
d. Recurrent heart failure.
e. Growth failure (faltering of growth)
3. Eisenmenger syndrome
a. Prolonged high Pulmonary blood flow → increased Pulmonary blood pressure → pulmonary
hypertension → with time right heart blood pressure exceed the left side blood pressure →
reversal of shunt → central cyanosis
b. Risk is higher with large unrepaired defects

• ACHD without shunt

1. Severe obstructive lesions can present early in life with heart failure.
2. Low cardiac output manifestations.

3 | Pediatrics Cardiology
 Patent ductus arteriosus (PDA)
• Definition:
• Persistent fetal duct connecting the aorta & the pulmonary artery
• Site: The aortic end is just distal to left subclavian artery. Pulmonary end is at the bifurcation.
• Association: Congenital rubella syndrome & prematures

• Hemodynamics:
• Physiological closure occurs within 24h after birth in response to high O2 tension
• Delayed closure of the duct beyond that age is considered pathological so PDA
• Blood is shunted from the higher pressure of aorta to pulmonary artery → ↑↑Pulmonary blood flow
• Run off of blood from Aorta to the pulmonary artery occurs during systole and diastole but mainly during
diastole → lower diastolic pressure → Hyperdynamic circulation
• Left atrial and left ventricle enlargement dur to volume overload

• General Manifestations:
• Small duct
o Diagnosis: Asymptomatic; discovered accidentally
o Cardiac Examination: Continuous murmur & ± systolic thrill over the upper left sternal border
o Causes no symptoms or hemodynamic changes also normal general examination
o Importance: at risk for infective endocarditis “So needs prophylaxis”
• Big duct
o Diagnosis
▪ Heart failure: “fatigue, forehead sweating with suckling, Feeding difficulties
▪ Chest symptoms: cough, wheezes, recurrent chest infections.
o Cardiac Examination: LVE, Continuous murmur (Machinery), Systolic thrill, loud S2 if PHT
o Hyperdynamic circulation e.g., big pulse pressure, big volume, water hummer pulse

• Complications:
• Heart: Heart failure (LV failure), Infective endocarditis
• Chest: Recurrent chest infections
• Shunt:
o Pulmonary hypertension "Palpable and Accentuated S2"
o Shunt Reversal: Cyanosis Mainly in lower limbs "Differential cyanosis"

• Investigations:
• Chest X-ray:
o Heart: Cardiomegaly (mainly left side)
o Lung: Plethoric lungs, May be chest infection
• ECG: Left ventricular hypertrophy (LVH)
• Echocardiography: Can detect the position and size of the defect. Measure pulmonary pressure, and
explore other associated defects
• Cardiac catheter: Diagnostic & Therapeutic
• Treatment:
• Medical:
o Control heart failure & prevent infective endocarditis (Infective endarteritis)
o Medical closure in preterm by I.V. indomethacin "PGL inhibitor" in the 1st week of life. [Other
drugs: Ibuprofen & Paracetamol]
• Surgery:
o Surgical ligation or catheter insertion (Small PDAs are closed with intravascular coils, Moderate
to large PDAs are closed with an umbrella-like device)
o Irrespective of age, size or symptoms, all PDAs should be closed, preferably before 1 yr of age.

4 | Pediatrics Cardiology
 Ventricular septal defect (VSD)
• Definition: Defect anywhere in the interventricular septum
• Types of VSDs:
1. Perimembraneous defect: Adjacent to tricuspid valve, The commonest type (80%)
2. Muscular defects: Either single or multiple (Swiss cheese).
3. Supracristal defect: also called infundibular or sub-arterial.

• Hemodynamics:
• Left to right shunt is affected by two factors:
o Size of the VSD: the main factors, defect either small or large
o Pressure: the level of the pulmonary vascular resistance
• During systole:
o Pressure in the left ventricle is larger the than the pressure in the right ventricle
o Some of the blood from the left ventricle will leaks to the right ventricle
o Excess blood will reach the lung (lung plethora)
o No shunt occurs during diastole
• This has two net effects
o First; volume overload in the left ventricle → left ventricle will enlarge
o Second; pulmonary hypertension with pressure overload on the right ventricle → right ventricle
will enlarge
• When pulmonary blood pressure increase (pulmonary hypertension)
o First; amount of the blood that enters the shunt will decrease
o Second; shunt reverse (right → left shunt) → cyanosis will occur (Eisenmenger syndrome)

• Clinical picture:
• Small defects (3 - 5 mm size):
o Diagnosis: Asymptomatic, accidentally discovered during routine examination
o Cardiac examination:
▪ Loud harsh pansystolic murmur on lower left sternal border.
▪ Propagate all over the heart.
▪ ± systolic thrill over the lower left sternal border
▪ No cardiomegaly
o Investigations: Normal CXR & ECG, Echo is Diagnostic
o Treatment: No treatment required as spontaneous closure occurs
o Importance: At risk for infective endocarditis * “So needs prophylaxis”

• Moderate to large defects (more than 5mm):

o Symptoms of heart failure
▪ Feeding difficulties, Forehead sweating with suckling
▪ Exertional Dyspnea & Exercise intolerance "Fatigue"
▪ Failure to thrive (FTT): from recurrent chest infections [Fever, cough and chest
retraction]
o Cardiac examination:
▪ Signs of ventricular enlargement: " Biventricular enlargement or mainly left
▪ Systolic thrill over the left parasternal area “Murmur grade IV or V”
▪ Harsh, Pansystolic, all over the precordium, Maximum intensity: Lt parasternal area (3rd
and 4th spaces)
▪ Accentuated P2 due to PHT

5 | Pediatrics Cardiology
 o Investigations:
▪ Chest X-ray:
• Heart: Cardiomegaly, mainly left side or Both
• Lung: plethoric lung. May be chest infection
▪ ECG: Left or biventricular hypertrophy
▪ Echocardiography:
• Can detect the location and size of the defect.
• Measure pulmonary pressure, and explore other associated defects
▪ Cardiac catheterization:
• Invasive with increased risk of infective endocarditis
• Can be used in diagnosis and treatment

o Complications:
▪ Heart:
• Heart failure: Tachycardia, Tachypnea and Tender hepatomegaly (3T)
• Infective endocarditis: suspected if unexplained fever (more than 15 days)
▪ Chest:
• Recurrent chest infections: fever, cough, dyspnea and crepitation
▪ Shunt:
• Pulmonary hypertension "Palpable and Accentuated S2"
• Shunt Reversal (Right to Left) Cyanosis (Eisenmenger Syndrome).
▪ General: Failure to thrive (FTT)

• Treatment
• Small defect
o Reassurance; surgical intervention is not usually recommended
▪ Follow up with ECG & Echo to confirm spontaneous closure
o Avoid infective endocarditis by dental hygiene and antibiotic prophylaxis.
• Large defect
o Medical:
▪ Heart failure: Rest, oxygen, Diuretics, Dilators, Digitalis See HF…
▪ Chest infections: Proper antibiotics, O2, Suction & Physiotherapy
▪ Infective endocarditis: Prophylaxis antibiotics before any surgical procedure
▪ FTT: Proper nutrition (Avoid aspiration) & high calorie diet
▪ Follow up with ECG & Echo to confirm spontaneous closure

o Surgical:
▪ Indications:
1. Symptomatic large defects with uncontrollable heart failure or Growth failure
2. Progressive pulmonary hypertension.
▪ Method:
• Transcatheter device closure at 3-6 months of life
• Surgical closure after reduction of pulmonary hypertension (6 - 12 months)
▪ PHT: Pulmonary artery banding
▪ Eisenmenger syndrome: heart & lungs transplantation "irreversible & fatal condition"

• Prognosis:
• Small VSD: 30-50% of small defects (especially muscular) close spontaneously within the first 2-years of
life
• Large VSD: heart failure in 6 weeks or Eisenmenger syndrome as early as 6 months

6 | Pediatrics Cardiology
 Atrial Septal Defect (ASD)
• Definition: Defect in the inter-atrial septum.
• Types of ASD:
1. Secundum ASD: in the Upper part of the septum "at the site of fossa ovalis" 80% of cases
2. Primum ASD: in the Lower part of the septum, more severe, usually associated with mitral
regurgitation. Usually associated with mitral regurge
3. Sinus venosus defect: Near the opening of SVC
• Hemodynamics:
• Blood is shunted from left atrium to right atrium to the right ventricle through ASD
• This will increase pulmonary blood flow → pulmonary plethora (more with primum ASD)
• Right ventricular enlargement due to volume overload

• Clinical picture:
• Secundum ASD:
o Diagnosis: Asymptomatic, usually presents later after the 3rd or 4th decades "Arrhythmia"
o Cardiac examination:
▪ Usually normal (may be RVH)
▪ No thrill, No murmurs, Wide fixed splitting of S2
o Investigation:
▪ Chest X-ray: Cardiomegaly with RVH & RAD. Plethoric lungs (pulmonary vascular
markings)
▪ ECG: RVH & RAD
▪ Echocardiography is diagnostic
▪ Cardiac catheter: Pre operative/corrective intervention

• Primum ASD:
o Diagnosis:
▪ heart failure: “fatigue, forehead sweating with suckling, feeding difficulties
▪ Chest symptoms: cough, wheezes, recurrent chest infections.
o Cardiac examination:
▪ Cardiomegaly with BVH
▪ Wide fixed splitting of S2, Pansystolic murmur of mitral regurge propagating to axilla
o Investigation:
▪ Chest X-ray: BVH, Plethoric lungs (↑ pulmonary vascular markings)
▪ ECG: BVH, right bundle branch block
▪ Echocardiography is diagnostic
▪ Cardiac catheter: Pre operative/corrective intervention

• Complications: Very rare; more common with primum ASD

• Heart:
o Recurrent heart failure
o Arrhythmias: Atrial fibrillation "AF" & Right Bundle branch block "BBB"
o Infective endocarditis is extremely rare (can occur in primum defects)
• Chest: Recurrent chest infections: Cough, Dyspnea and crepitation
• Shunt:
o Pulmonary hypertension "Palpable and Accentuated S2"
o Eisenmenger Syndrome occur very late; in adulthood, by the 3rd - 4th decade

7 | Pediatrics Cardiology
 • Treatment
• Medical
o Chest infections: Proper antibiotics, O2, Suction & Physiotherapy
o Heart failure: Rest, oxygen, Diuretics, Dilators, Digitalis See HF…
o Infective endocarditis prophylaxis is needed only for primum ASD with mitral regurge
o FTT: Proper nutrition (Avoid aspiration) & high calorie diet
o Follow up with ECG & Echo to confirm spontaneous closure
• Surgery:
o Surgical correction Primum ASD or transcatheter device closure in Secundum ASD
• Prognosis
• 40% of secundum ASD defects close in 1st four years of life spontaneously

VSD ASD PDA

Chamber Both Right & Left ventricles Right ventricle Left ventricle
Apex
Shifted outward & downward Shifted outward Outward & downward
Thrill Palpable systolic thrill NO thrill Palpable systolic thrill
Sounds ↑↑ S2 due to PHT
↑↑ S2 due to PHT ↑↑ S2 due to PHT
Wide fixed splitting
Murmurs Harsh, Holo or Pansystolic, No Murmur Murmur Only
Harsh, Continuous
murmur Propagated all over if associated: MR (Soft) or
(systolic & diastolic)
the precordium VSD (Harsh)
Site 3rd, 4th left intercostal spaces Left infra clavicular
No murmur
in parasternal line area (Machinery)
Lung
Increased (Plethora) Increased Increased
vasculature

8 | Pediatrics Cardiology
 Coarctation of Aorta (CoA)
• Definition:
• Constriction of the aorta to anywhere from aortic arch to aortic bifurcation.
• Association: Turner syndrome, Bicuspid aortic valve

• Types:
• Ductal CoA: adjacent to ductus arteriosus; below origin of left subclavian artery
• Preductal or postductal CoA: far uncommon

• Hemodynamics:
• Hypertension proximal to the CoA (head & upper limbs)
• Hypotension distal to the CoA (lower body & lower limbs)
• Collaterals develop between the proximal & distal aortae (anterior intercostal arteries which are branches
of internal thoracic artery & posterior intercostal arteries which are branches of descending thoracic
aorta)
• Left ventricular hypertrophy: may be failure in severe narrowing (afterload failure)
• In severe coarctation, blood is shunted from the pulmonary artery to the descending aorta via a patent
arterial ductus with subsequent:
o Perfusion of the lower body is dependent on the patent ductus (duct dependent systemic flow)
o May be differential cyanosis (lower limbs blue, upper limbs pink).

• Clinical Picture:
• Mild coarctation
o Asymptomatic, discovered accidentally on routine examination
• Severe coarctation
o Headache and blurring of vision
o Heart failure in early infancy
▪ Poor feeding (exertional dyspnea with suckling or feeding)
▪ Failure to thrive (growth retardation)
▪ Low cardiac output symptoms
• Complicated cases: May present with:
o Systemic hypertension due to renal hypoperfusion.
o Intracranial hemorrhage due to hypertension or associated aneurysm of circle of Willis.

• Sign:
• General:
o Pulse: Femoral pulse is weak & strong early radial pulse “Radio - femoral delay”
o Blood pressure: Low blood pressure in LL, High in the upper limbs
• Palpitation:
o LVH: strong apical pulsations
o Systolic thrill over the left parasternal area
• Auscultation:
o Sounds: Accentuated S2 due to Systemic hypertension
o Murmur: systolic along left sternal border '3rd left space' with maximum intensity in inter-
scapular area.

9 | Pediatrics Cardiology
 • Investigations:
• Chest X-ray:
o Cardiomegaly (LVH), lung congestion
o Rip notching: pressure by enlarged intercostal collaterals is (Rosler’s sign)
• ECG: LVH in older patients
• Echocardiography is Diagnostic
• When echocardiogram is equivocal: spiral CT, MRI and catheter are alternative
diagnostics

• Complication:
• Heart
o Heart Failure (Tachypnea, Tachycardia and Tender enlarged liver)
o Infective endocarditis: Fever (> 2 weeks), purpura & hematuria
• Hypertension & Intracranial Hemorrhage (very dangerous)

• Treatment:
• In neonates with severe coarctation of the aorta
o Infusion of prostaglandin E1 to reopen the ductus and re-establish adequate lower extremity
blood flow.
o Once a diagnosis has been confirmed and the patient is stabilized, surgical repair should be
performed
• Older cases require:
o Control of hypertension and prophylaxis against infective endocarditis.
o Treat complications (heart failure): Rest, oxygen, Diuretics, Dilators & Dopamine
o Surgical repair:
▪ Resection of the narrowed segment of aorta & two ends anastomosed together
(Coarctectomy)
▪ Intravascular stent insertion “Catheterization”
▪ Balloon angioplasty can be used if restenosis (Recurrence)

10 | Pediatrics Cardiology
 Congenital Aortic Stenosis
• Types:
1. Valvular aortic Stenosis: Valve is bicuspid instead of tricuspid.
2. Supra-valvular stenosis
3. Sub-valvular stenosis
• Aortic stenosis may not be an isolated lesion. It is often associated with mitral valve stenosis and
coarctation of the aorta, and their presence should always be excluded

• Hemodynamics:
• During systole: Obstruction of the blood flow from LV to aorta
• Leading to pressure overload on the LV & LV hypertrophy

• Clinical picture
• Asymptomatic if mild degree (accidentally discovered on routine examination)
• Chest pain on exertion
• Heart failure
o Low cardiac output symptoms: easy fatigability & syncopal attacks “fainting”
o Feeding difficulties (exertional dyspnea with suckling or feeding)
o Failure to thrive (growth retardation): Underweight or short stature
• Pulmonary congestive symptoms: cough, wheezes and recurrent chest infections

• Signs: (LVH)
• General:
o Pulse: Small pulse volume
o Blood pressure: Low pulse pressure "Low systolic pressure
• Inspection: Apical pulsations (LT ventricular enlargement)
• Palpation:
o Apex shifted outward & downward (LVH)
o Systolic thrill over the left parasternal area
• Auscultation:
o Weak S2 (aortic component is weak and delayed)
o Harsh, systolic murmur, maximum intensity in the aortic area 1, Propagate to the neck & the
apex

• Investigations
• Chest X-ray: Normal or prominent left ventricle, post-stenotic dilatation of the ascending aorta
• ECG: LVH
• Echocardiography is Diagnostic

• Complications
• Heart failure & infective endocarditis
• Recurrent chest infections or Sudden death

• Treatment:
• Medical: Prophylaxis against infective endocarditis, Treat complications (heart failure)
• Surgical: Balloon valvoplasty or valvotomy or valve replacement For: Symptomatic cases and/ or Pressure
gradient across the valve > 40mmHg

11 | Pediatrics Cardiology
 Congenital Pulmonary Stenosis
• Types:
1. Valvular stenosis: the most common type. The valve leaflets are fused together
2. Supra - valvular stenosis: narrow segment above valve.
3. Sub - valvular Stenosis: muscular hypertrophy below the valve "Infundibular"

• Hemodynamics:
• During systole: obstruction of the blood flow from the RV to pulmonary
• Pressure overload on the RV that will be dilated and may fail.

• Clinical picture:
• Affected children are usually asymptomatic only accidentally diagnosed due to an ejection systolic
murmur over the pulmonary area with muffling of the second heart sound.

• Signs:
• An ejection systolic murmur best heard at the upper left sternal edge; thrill may be present.
• Muffled S2
• An ejection clicks best heard at the upper left sternal edge.
• When severe, there is a prominent right ventricular impulse (heave).

• Investigations:
• Chest X-ray: Normal or post-stenotic dilatation of the pulmonary artery, Cardiomegaly, Oligemic lung field
• ECG: Shows evidence of right ventricular hypertrophy (upright T wave in V1).
• Echocardiography is Diagnostic

• Complications:
• Heart failure & infective endocarditis
• Pulmonary oligemia: high risk for TB infection.
• No recurrent chest infection

• Treatment
• Medical: Prophylaxis against infective endocarditis, Treat complications (heart failure)
• Surgical: Transcatheter balloon dilatation: When obstruction is severe

12 | Pediatrics Cardiology
 Cyanotic Congenital Heart Disease (CCHD)
• Classification

CCHD with decreased pulmonary blood flow CCHD with increased pulmonary blood flow
With RVH With RVH
Fallot tetralogy Transposition of great arteries (TGA)
Pulmonary atresia with VSD Total anomalous pulmonary venous return
With LVH Eisenmenger Syndrome
Tricusped atresia With BVH
Ebstein anomaly Truncus arteriosus
Pulmonary atresia with VSD Single ventricle
Hypercyanotic spells (attacks of increasing cyanosis) Poor feeding “dyspnea on suckling”.
Heart failure is very rare Recurrent chest infections is common
P2 (pulmonary component of S2) → decreased P2 Recurrent heart failure is common
Chest X-ray → lung oligemia P2 → increased
Chest X-ray → plethora.

• Central cyanosis
• Definition
- Bluish discoloration of skin and mucous membranes due to presence of > 5 gm /ml
reduced hemoglobin in the capillary blood.
• Causes
1. Congenital heart diseases are the main cause of chronic central cyanosis
o The commonest cyanotic congenital heart disease is Fallot tetralogy.
o The commonest cyanotic congenital heart disease presenting at birth is TGA.
2. Respiratory failure (usually acute cyanosis)
• Presentation
- Early it may be overlooked and become evident only during crying and feeding
- Then it becomes apparent at rest; noted mainly in inner lips and tongue
• Consequences
1. Cyanotic clubbing, dusky blue skin, gray sclerae with engorged blood vessels
2. Polycythemia
3. Increased risk of Relative iron deficiency and Cerebral stroke

13 | Pediatrics Cardiology
 Fallot Tetralogy
• Definition:
• Commonest cyanotic congenital heart disease with decreased pulmonary blood flow; Composed of
1. Pulmonary stenosis: Mainly infundibular (muscular) but may be valvular
2. Right ventricle hypertrophy: Usually mild RVH, due to right ventricle out flow obstruction
3. VSD: Usually large Lies just below the aortic valve
4. Aorta overrides the ventricular septal defect Receives mixed blood
• Hemodynamics:
• Blood in the right ventricle will pass through two pathways
• Small amount of blood pass to the lung through narrow pulmonary → lung oligemia
• Large amount of blood enters the aorta through big VSD → mixing of blood → central cyanosis
• Large VSD will cause equalization of pressure in both ventricles
• Pulmonary blood flow is maintained In neonate via ductus arteriosus and In older child via Multiple
aortopulmonary collateral arteries arising from the ascending and descending aorta

• Clinical picture
1. Central Cyanosis
• Delayed (1 – 2 months after birth): increasing hypertrophy of the right ventricular infundibulum as
the patient grows, cyanosis occurs later in the 1st year of life
• At birth: if severe degrees of PS, neonatal cyanosis is noted immediately (extreme Fallot)
• Absent: if mild degrees of PS (Acyanotic or pink Fallot)
2. Hyper Cyanotic Spells
• Clinically:
▪ Severe cyanosis: attacks of increased cyanosis and respiratory distress
▪ Spell lasts from a few minutes to a few hours
▪ Severe spell may lead to syncope, convulsions, cerebrovascular stroke or even death
▪ Decreasing murmur intensity
• Incidence: Mainly in the 1st 2 years of life
• Triggered: by crying, feeding or infection
• Mechanism: Infundibular spasm → reduction of an already reduced pulmonary blood
• Treatment:
▪ Position: knee chest position (infant), squatting position (children)
▪ Propranolol: Reduces infundibular spasm
3. Cyanotic Clubbing of fingers and toes: Usually seen after 6 – 12 months (blue clubbing)
4. Squatting position:
• After physical effort o dyspnea increases o the child
• assumes squatting position for the relief of dyspnea
• Theory: Squatting → kink of femoral arteries ↑ systemic vascular resistance ↑ aortic pressure ↑
pulmonary blood flow ↑blood oxygenation.
5. Failure to thrive: shorth stature and underweight due to tissue hypoxia

• Cardiac examination:
• Palpation:
o Normal heart size (may be mild RVH)
o Systolic thrill over left sternal border.
• Auscultation
o S2: single (A2 only is heard), weak pulmonary component
o Murmur: ejection systolic murmur on left sternal border (pulmonary obstruction)

14 | Pediatrics Cardiology
 • Complication:
• Cardiac
o Hyper cyanotic spills
o Infective endocarditis
o Heart failure is rare; may be iatrogenic with big shunt
• Respiratory
o Pulmonary tuberculosis due to pulmonary oligaemia
• Blood:
o Polycythemia due to Hypoxemia (potent stimulation of BM)
o Hyperviscosity: increased the risk for thrombosis
Rrelative Iron deficiency due to consumption of iron by hyperactive BM
• Brain:
o Infraction: stroke
o Abscess: loss of lung filter
o Hemiplegia: due to stroke (thrombosis) or infective endocarditis
• General
o Failure to thrive
o Finger clubbing

• Investigations:
1. Chest X-ray: boot shaped heart, Oligaemic lung fields, Heart size is normal or mild RVH
2. ECG shows RVH
3. Echocardiography: Two-dimensional Echo is Diagnostic
4. CBC: high Hb and Hct (due to polycythemia), ↓MCV (relative iron deficiency)
5. ABG: hypoxemia and acidosis

• Treatment:
A. Emergency treatment of hyper cyanotic spills
1. Put the pt. in chest knee or squatting position
2. Morphine: sedation and pian relief (for stress)
3. Propranolol: relax the pulmonary spasm
4. IV alpha agonist: phenylephrine → Increases systemic vascular resistance
5. IV Fluid to correct dehydration
6. Sodium bicarbonate to correct acidosis
7. Intubation and ventilation for spells resistant to the above management
8. Avoid digitalis as it may induce infundibular spasm
9. Arrange for surgery
B. Avoid cerebral thrombosis by
1. Treatment of relative iron deficiency: 6mg/kg iron supplement
2. For severe polycythemia
a. Adequate hydration
b. Phlebotomy
c. Partial exchange transfusion with albumin or saline.
C. Prophylaxis & treatment of infective endocarditis
D. Surgical: Indicated as soon as the spells begin
1. Palliative shunts: Anastomosis between subclavian artery & ipsilateral pulmonary artery using
(Modified Blalock Taussig) to allow n pulmonary blood flow
2. Total repair: infundibulum resection and closure of VSD Can be done between 4 months to 2 years
according to severity and available cardiac center

15 | Pediatrics Cardiology
 Transposition of Great arteries (TGA)
• Description:
• Aorta arises from right ventricle
• Pulmonary artery arises from left ventricle
• Two isolated circulation (pulmonary and systemic) work in parallel → incompatible with life
• As the condition incompatible with life the pt. need one or more shunt between the aorta and
pulmonary
• Mixing of blood occur via Patent Foramen Oval, VSD or PDA

• Incidence: Common in infant of diabetic mother.

• Types:
• Isolated TGA
• TGA With VSD
• TGA With VSD and pulmonary stenosis
• Corrected TGA
• Clinical picture:
• Isolated (intact ventricular septum)
▪ Medical emergency
▪ Severe cyanosis at birth
▪ With ductus closure → marked cyanosis with acidosis and hypoglycemia
▪ Accentuated S2 (anteriorly placed aorta)
▪ No murmur, No thrills

• TGA With VSD

▪ Milder cyanosis
▪ Manifestations of increased pulmonary blood flow → recurrent chest infection
▪ Heart failure
▪ VSD murmur
• Complication:
• Heart failure and infective endocarditis
• Recurrent cheat infection
• Thrombosis due to polycythemia → stoke, brain abscess
• Investigations:
• Chest x ray: Cardiomegaly (egg on side), Plethoric lung
• ECG: RVH.
• Echo: Diagnostic
• Cardiac catheter: Preoperative
• Treatment:
• Isolated (intact ventricular septum)
1. Maintain PDA → PGE1 infusion & avoid O2.
2. Urgent shunt: Rashkind balloon atrial septostomy: create large ASD to allow free intra cardiac mixing.
3. Total correction: either Arterial switch operation (anatomical correction) OR Atrial switch operation

• TGA With VSD

1. Treatment of heart failure (3D)
2. Avoid cerebral thrombosis (see Fallot tet)
3. Precautions against infective endocarditis
4. Arterial switch operation

16 | Pediatrics Cardiology
 Truncus arteriosus (TA)
• Description:
• One arterial trunk leaves the heart giving rise to both aorta & pulmonary artery
• Large VSD below the trunk
• One semilunar valve (Truncal valve)
• Clinical picture:
• Cyanosis: variable onset (usually minimal esp. in neonate & infants).
• Features of increased pulmonary blood flow → recurrent chest infection
• S2 single.
• VSD murmur.
• Chest X-ray: Right sided aortic arch in 50% of cases.
• Treatment:
• Treatment of heart failure.
• Surgical correction.

Total anomalous pulmonary venous return (TAPVR)

• Pulmonary veins drain into right side of the heart either:
1. In superior vena cava (supra cardiac)
2. In coronary sinus (cardiac)
3. In inferior vena cava (infra cardiac)
• ASD allow blood in right atrium to pass to left atrium ➔ blood mixing o cyanosis.
• Chest X-ray Snowman in snowstorm or Figure 8 shaped heart.
• Echo is diagnostic

Ebstein's Anomaly
• Composed of:
• Right ventricular outflow tract obstruction, Tricuspid regurgetation.
• Huge right atrium, Small right ventricle
• Patent foramen oval
• Clinically:
• May be asymptomatic
• Mild cyanosis
• Atrial arrythmias
• Splitting of S1 and S2
• Pansystolic murmer (tricusped regurge)
• May be heart failure.
• Investigation:
• Chest X-ray: May be huge cardiomegaly
• ECG: RAD, RBBB
• Echocardiography and color Doppler: diagnostic

• Management:
• Medical treatment is useful only for arrhythmia management.
• Surgical intervention is difficult and controversial with a significant risk.

17 | Pediatrics Cardiology
 Pharyngitis
• Definition
- Group A Beta-hemolytic streptococci: (GAS) is Streptococcus pyogenes responsible for
bacterial pharyngitis.
- GAS pharyngitis is linked with Rheumatic fever (RF) and Rheumatic Heart Disease (RHD).
• Pharyngitis/Tonsillitis
- Very common in children and young adults
- 70% caused by viruses AND 30% are caused by GAS
- The presence of GAS in throat may reflect either true infection or a carrier state
- Only patients with true infections need to be given antibiotics
- GAS are highly transmissible and spread rapidly with the M types constantly changing
• Clinical Features of GAS Pharyngitis
1. Sore throat in patients 5-15 years
2. Fever, pharyngeal exudates, cervical lymph node and skin rash: may be present
3. No runny nose or cough
• Note:
- Pharyngitis includes inflammation of tonsils and paratonsilar tissues
- Tonsillectomy does not prevent GAS pharyngitis
• Diagnosis of GAS Pharyngitis
1. Definite diagnosis: throat culture: Not practical, takes
3-7 days, expensive
2. Rapid antigen test: expensive,not reliable
3. We rely on clinical diagnosis
• Treatment
- Single injection of benzathine penicillin after sensitivity
test. Or Oral penicillin (10 Days)
• Primary prevention of acute rheumatic fever
1. Prompt diagnosis and treatment of GAS pharyngitis
2. Can we prevent GAS pharyngitis?
▪ Vaccine research is going on
▪ Not yet ready

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 Acute Rheumatic Fever (ARF)
• Definition:
- Autoimmune inflammatory disease affecting mainly the heart and joints & less frequently CNS, skin and
subcutaneous tissue

• Etiology:
- Delayed process following upper respiratory infection with B. Hemolytic streptococci.
- Latent period of 2-4 weeks between upper respiratory tract infections or scarlet fever

• Risk factor:
1. Age: 5-15 years, infants and adults are rarely affected.
2. Sex: Equal in both, but chorea is more common in girls.
3. Family history may be positive
4. More common in developing countries

• Pathogenesis:
- It is believed to be caused by antibody Cross-reactivity Or Antigenic similarity
- Antibodies formed against streptococcal antigens:
o Cross react with human Tissue antigens in the Heart, joints, skin and CNS
o There is molecular mimicry between streptococci and tissue antigens 'myosin'
- Genetic susceptibility: Several genes responsible for the autoimmune reaction have been described
recently

• Pathology:
- Proliferative lesion with Aschoff nodules as in the heart and subcutaneous nodules that heal by fibrosis
- Exudative (in joints) with inflammatory edema resolve without residual
- Valvulitis due to endocardial inflammation may destroy valves resulting in valve regurgitation (in acute
stage) OR end up in valve fibrosis and stenosis OR a mix of both

• Clinical Picture:
- A latent period of 2-4 weeks usually exists between pharyngitis and clinical symptoms of ARF

• Major Criteria of Rheumatic Fever:

1. Arthritis:
- The commonest presenting sign 75%
- joint is "Painful, Red, Hot, Tender, Swollen with Limited mobility"
- Polyarthritis: affecting more than one joint. May be Mono-arthritis as well
- Affect mainly the large joints usually affected, (knee, ankle, wrist, elbow)
- Migratory: affect joint few days & leave the joint to affect another joint
- Dramatic response to salicylates (within 48 hours.). Also, may resolve spontaneously
- Fate: Leaves the joint completely free (No damage / No deformity)
- Arthritis now refers to polyarthritis in low-risk populations, but to monoarthritis or polyarthralgia in
moderate/high-risk populations

2. Pancarditis
- The 2nd common (50%) and most serious manifestation of ARF
- Inflammation of endocardium, myocardium and pericardium
- Carditis may be silent or late onset appearing after 6 weeks – 6 months
- Carditis is now defined as clinical and/or subclinical (echocardiographic valvulitis)

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 • Pericarditis:
▪ Precordial Pain (Stitching pain not related to respiration)
▪ Pericardial rub on auscultation
▪ Pericardial Effusion may complicate the condition: Dull aching pain, Distant heart sounds, rub sound
disappears
• Myocarditis
▪ Tachycardia out of proportion to age & fever
▪ Heart failure indicates severe cardSitis with Marked dyspnea, enlarged tender liver, Gallop rhythm,
muffled heart sounds, Cardiomegaly
• Endocarditis:
▪ Endocarditis is the most consistent
▪ Left side more commonly affected, also Mitral valve more affected than aortic:
▪ Acute stage (Inflammation)
o Carey coombs murmur": inflamed edematous leaflets = MS → mid-diastolic
o Mitral regurge: Damage of the cusps → holosystolic murmur propagating to the axilla with
muffled 1st heart sound.
▪ Chronic stage (Fibrosis)
o Incompetence (Regurgitation): mitral regurge is the most common lesion
o Stenosis: Mitral stenosis in prolonged or recurrent disease "Fibrosis"
o Double lesion "Both stenosis & Regurge"
• Subclinical carditis:
▪ Significant cardiac affection detected by echocardiography with absence of any murmur.
▪ Recently considered as a major criterion

3. Rheumatic Chorea:
▪ Occur in 15% of cases (due to affection of Basal Ganglia)
▪ Self-limited, more in Girls
▪ May be the only manifestation of rheumatic fever? Explain: Occur weeks or months after pharyngitis
so, other criteria are usually lacking
▪ Echo is essential in chorea patients to exclude subclinical carditis
▪ Manifestations include:
1. Abnormal movements;
o Irregular, Involuntary, Static, Sudden jerky, Semi-purposeful movements
o Increased by emotional stress, decreased by rest
2. Hypotonia:
o Tongue:
▪ Darting tongue: The tongue cannot be protruded for longer than few seconds
▪ Speech abnormalities (dysarthria)
o Hands:
▪ Inability to maintain grasping. The tone ↓ & ↑ continuously "Milking sign"
▪ Also, piano movements of the extended hands
▪ Cannot maintain arms extended → spooning & pronation of hands (pronator sign)
▪ Boat-shaped hands: flexion at the wrist+ hyperextension at the fingers
o Knee: Pendular knee jerk
3. Emotional changes (Lability):
o Sudden laughing or crying with no cause
o Poor school performance.

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 4. Erythema Marginatum (< 5%)
- Large erythematous macules, With clear or pale centers and sharp
borders (map like appearance)
- Mainly in the trunk, Proximal parts of the limbs
- Evanescent (may come and go for several months)
- Not affecting the face, not pruritic
5. Subcutaneous Nodules (< 1%)
- Appear several weeks after the attack
- Over the extensor surfaces of tendons near bony prominences.
- Criteria: Size > 2 cm, Firm, freely mobile, and painless.
- Usually associated with severe carditis → poor prognosis

• Minor criteria of Rheumatic Fever

A. Clinical
1. Fever: of > 38° C
2. Arthralgia
o Monoarthralgia: Moderate/High-Risk populations only include
o Polyarthralgia for Low-Risk populations
o Can’t be used as minor manifestation in presence of arthritis

B. Investigations
1. ECG: Prolonged P-R interval
o Can’t be used as minor manifestation in presence carditis
2. Elevated acute phase reactants
o ↑ESR; >30 mm/hr
o ↑C reactive protein
• Evidence of recent Group A Streptococcal (GAS) infection
1. Positive throat culture
2. Elevated streptococcal antibody titers: ASO, Anti (DNase), Anti hyaluronidase
3. Recent history of scarlet fever

• Diagnosis of ARF using modified Jones criteria (2015)

A. For diagnosis of initial attack of ARF
- 2 major manifestations, Or
- 1 major and 2 minor manifestations
- Plus Evidence of recent GAS infection
B. For diagnosis of recurrent attacks of ARF
- 2 majors, Or
- 1 major and 2 minor Or
- 3 minor manifestations (only in the Moderate/High-Risk population)
- Plus Evidence of recent GAS infection
C. Exception of jones criteria
- There are 3 circumstances in which the diagnosis of acute rheumatic fever can be made without strict
adherence to the Jones criteria
1. When chorea is the only major manifestation of acute rheumatic fever
2. When indolent carditis is the only manifestation
3. Patients with recurrences of acute rheumatic fever in particularly high-risk populations.

21 | Pediatrics Cardiology
 • Prognosis:
- Arthritis sub side within days to weeks even without treatment.
- Chorea subsides within few months without residuals.
- Erythema Marginatum may come and go for several months (Evanescent)
- Only carditis can cause permanent damage especially in recurrences which Suggested by:
o Appearance of new murmurs.
o Change in character of already existing murmur

• Complications:
1. Rheumatic activity: 'Recurrence' If no adequate prevention so more damage to cardiac valves and
myocardium
2. Rheumatic heart disease:
o Usually occur with repeated attacks of rheumatic activity.
o The valves (mitral or aortic) show incompetence with volume overload on LV.
o Stenotic lesions are usually seen in adolescence as they need time to develop.
3. Infective endocarditis that occurs on damaged valves.
4. Congestive heart failure or early arrhythmias complications

• Differential diagnosis:
A. Other causes of arthritis
- Rheumatoid arthritis:
o Involve small peripheral joints.
o Non migratory
o Chronic arthritis; last > 6 weeks.
o Damage and deformity of the joint
o Absent dramatic response to salicylates within 48 hours.
o Associations e.g. Spiking fevers, lymphadenopathy, and splenomegaly
- Transient synovitis (toxic synovitis)
o Post-infectious arthritis, typically affects the hip, often after an upper respiratory tract infection
o Acute onset of severe pain in the hip, with referred pain to the thigh or knee, lasting
approximately 1 wk
- Reactive arthritis related to gastrointestinal infections (Shigella, Salmonella, Yersinia)
- Septic arthritis (usually monoarthritis )
B. Other causes of carditis
- Vial carditis
- Infective endocarditis
- Drug induced.
C. Other causes of chorea
- Wilson disease
- Cerebral palsy

• Treatment of Rheumatic Fever:

A. Treatment of Acute Attack
- Eradicate group A Streptococci from the URT
o Single dose of IM benzathine penicillin (If allergic to penicillin give Oral erythromycin for 10 days)
- Suppressing inflammation:
o Aspirin in anti-inflammatory doses (75-100mg/kg/d) for 4-6 weeks.
- Supportive treatment
o Bed rest 4-6 weeks.
o Treatment of congestive heart failure.

22 | Pediatrics Cardiology
 B. Specific treatment
- Carditis:
a. Salicylates: used in mild carditis without cardiomegaly nor heart failure
b. Corticosteroids (Prednisone)
▪ Indications: Moderate to severe carditis (with cardiomegaly), Heart failure.
▪ Dose: 2 mg/kg /day for 2-3 weeks. Followed by half the dose for 2-3 weeks. And then tapering of
the dose by 5 mg/day every 2-3 days. At the beginning of tapering; salicylates is started at dose
of 50 mg/kg/d and continued for 6 weeks to avoid rebound phenomenon.
- Heart failure:
▪ Complete bed rest in semi-setting position, oxygen, fluid and salt restriction
▪ Diet: Adequate calories and small frequent meals to allow weight gain
▪ Daily weight monitoring is essential in hospitalized patients to follow hidden edema
▪ Diuretics: Furosemide 1- 2mg/kg/day oral or IV (preload reduction)
▪ Diuretics: Captopril 1-2 mg/kg/day oral (after-load reduction)
▪ Digitalis: Digoxin 0.01 mg/kg/day oral or IV (avoid hypokalemia)
▪ Dopamine: 10 - 20 μ/kg/min IV infusion “Inotropic agent that ↑ contractility”
- Rheumatic Chorea:
▪ Avoid emotional stress.
▪ Control abnormal movements: Phenobarbitone Or Haloperidol [Carbamazepine or valproic acid
can be considered in severe cases]
▪ Long-acting penicillin prophylaxis.
- Treatment of complications:
▪ Treatment of heart failure; see before.
▪ Treatment of infective endocarditis
▪ Treatment of valve lesions

• Prophylactic treatment for ARF:

A. Primary prevention: prevent 1st attack by
- Hygienic housing.
- Proper treatment of Strept. infection: penicillin or erythromycin for 10 days.
B. Secondary prevention:
- Prevent recurrence of Rheumatic fever by: Long-acting penicillin (Benzathine penicillin)
▪ Dose: 600.000 units for those < 27 kg and 1.2 million unit for those > 27 kg
▪ Route: Single injection, I.M every 3-4 weeks.
▪ Alternatives: Oral penicillin V or Erythromycin
▪ Duration:
• Without carditis: 5 yr. or until 21 yr. of age
• With carditis but without residuals: 10 yr. or until 21 yr. of age
• With carditis and residual heart disease: 10 yr. or until 40 yr. of age [Sometimes lifelong
prophylaxis]

C. Prevention of infective endocarditis:

- Oral amoxicillin 50 mg/kg "single large dose 'one hour before tooth extraction

23 | Pediatrics Cardiology
 Infective endocarditis
• Definition
- Infection of the valvular & mural endocardium
- Infection can be bacterial, viral, or fungal
• Incidence:
- children of any age with CHD or Rheumatic are at risk of infective endocarditis.
- The risk is highest when ↑ pressure gradient & small sized lesions & Less fibrosis.
• Pathogenesis:
- For endocarditis to occur, 2 factors are needed:
- Bacteremia (even transient) + cardiac structural abnormality → Infective endocarditis
• Causative organism:
1. Streptococcus viridians:
o The commonest organism 50%
o Follows dental caries, dental surgery, dental extraction, tonsillectomy
2. Staphylococcus aureus
o The 2nd most common. May infect even normal heart
o Mainly postoperative
3. Staphylococcus epidermidis
o Risk is high with prosthetic valve and central venous catheter
4. Group D enterococci
o More often after lower bowel or genitourinary procedures
5. HACEK group: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, and Kingella
6. Fungal ‘’Candida albicans’’: seen more frequent in immunosuppressed patients & post
open heart surgery
• Pathophysiology
1. Turbulence of blood flow across stenotic or incompetent valves damages the
endothelium
2. Platelets and fibrin adhesion forming thrombi
3. Circulating bacteria adhere and grow in these thrombi forming vegetations:
a. Valve destruction (Local)
b. Embolization to any organ in the body (Distant)
c. Septic embolic phenomena: osteomyelitis, meningitis, and glomerulonephritis
4. Immunologic response with deposition of immune complexes leading to vasculitis and
rash
• Clinical picture:
• Should be suspected: In any cardiac patient with UN-explained prolonged fever (> 2
weeks)
• General manifestations:
1. Toxemia:
o Fever, Malaise, Anorexia & Arthralgia are the presenting symptoms

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 o Pallor and Poor appetite (wight loss), Pale clubbing
2. Splenomegaly: due to toxemia, abscess or infraction may occurs
3. Sub-conjunctival hemorrhage: due to vasculitis
4. Splinter hemorrhage: Linear hemorrhage under the nail bed
5. Skin:
o Petechial rash
o Janeway lesions: Painless hemorrhagic lesion with necrotic center on the palms
o Osler's nodules: intra-cutaneous nodules at finger tips (Raised, small & painful)
• Cardiac manifestations
1. Appearance of new murmurs
2. Change in the character of previous murmurs
3. Sea gull murmur (musical) due to rupture of valve leaflets.
4. Acute Heart failure (Tachycardia, Tachypnea, Tender enlarged liver), Arrhythmias.
• Embolic manifestations:
1. Cerebral:
o Stroke (hemiplegia, convulsions) or Intracranial hemorrhage
o Abscess or mycotic brain aneurysms
2. Central retinal:
o Blindness
o Roth spots "retinal hemorrhage with clear center.
3. Pulmonary embolism: Cough, dyspnea, hemoptysis & chest pain
4. Peripheral vessels:
o Absent peripheral pulsations "Dorsalis pedis or Radial pulse"
o Digital gangrene with amputation
5. Renal embolism: Hematuria
• Investigations
1. Blood cultures: (very important)
o Time: before starting antibiotic treatment. And after proper sterilization
o Numbers: Multiple cultures from different sites should be taken (3-5 cultures)
2. Echocardiography: (very important)
o Importance:
• Detect vegetations
• Detects underlying cardiac lesions
• Detects complication e.g., valve dysfunction or leak, myocardial abscess
o Type: trans-thoracic or better to be done trans-esophageal if prosthetic valve.
3. CBC: Anemia: normocytic normochromic, with leukocytosis
4. Acute phase reactant: ↑↑ ESR & Positive +ve CRP
5. Other investigations:
o CT brain: if brain embolism "hypodense area".
o Fundus examination: to detect retinal hemorrhage.
o Pulmonary scanning: if pulmonary embolism.
o Chest radiograph: bilateral infiltrates, nodules, pleural effusions

25 | Pediatrics Cardiology
 o Abdominal US: if renal or splenic affection occurs.
o Doppler US: if vascular obstruction
o Urinalysis (hematuria; indicating GN or kidney infarcts or RBCs cast)
• Diagnosis: using Duke criteria
• Major criteria
1. Positive blood culture:
o Typical organism in 2 separated culture or
o Persistency +ve positive blood cultures drawn >12 hours apart
o Three or more positive cultures taken over > 1 hr.
o Single positive blood culture for Coxiella burnetii
2. Positive Echocardiography: Evidence of endocarditis
o Vegetations (Vegetation is the Hallmark of IE, It consist of platelets, fibrin,
cellular debris and the bacteria).
o Aortic root abscess or myocardial abscess
o New regurgitation (detected by Echo Not by auscultation).
o Prosthetic valve dehiscence
• Minor criteria: (TIMER)
1. Temperature: 38O C
2. Immunological manifestations: Glomerulonephritis, Roth spots, Splinter hemorrhage,
Osler nodules & Skin Petechiae
3. Microbiological evidence: positive blood culture but does not meet the criteria of
typical blood culture.
4. Embolic manifestations: Cerebral infarction or hemorrhage, Retinal infarction,
Conjunctival hemorrhage, Splenic infarctions, Renal infarction, Necrotic skin lesions or
gangrene, Janeway lesions
5. Risk factor: e.g., CHD, immunocompromised.
• Interpretation of Duke Criteria
• Definite infective endocarditis
o Two majors. Or
o One major + 3 minor. Or
o Five minors
• Possible infective endocarditis
o One major + One minor Or
o Three minors
• Treatment
• Supportive treatment:
o Bed rest: Until heart failure is controlled and ESR becomes normal
o Diet: Salt and fluid restriction if congestive heart failure
o Drugs: Antipyretics for fever.
• Specific treatment: (Antibiotics)

26 | Pediatrics Cardiology
 1. Empirical therapy till culture results
o Anti-staphylococcal penicillin (methicillin) + Aminoglycoside (Gentamycin)
o Suspected methicillin resistant S.aureus 'MRSA: Vancomycin+ Gentamycin
2. Specific therapy According to culture
o Streptococcus viridans / Enterococci:
• Penicillin G or ampicillin or ceftriaxone for 4 weeks AND Gentamycin for 2
weeks
o Staph. aureus:
• Vancomycin for 6 weeks AND gentamycin for 5 days.
o HACEK:
• Ceftriaxone alone or ampicillin + gentamycin for 4 weeks
• Vancomycin if penicillin or ceftriaxone are not tolerated
o Fungal infection:
• Amphotericin B
• Surgical treatment:
o Removal of vegetation & valve replacement if:
1. Failure of medical treatment
2. Occurrence of Complications
o Heart failure: due to destruction of valve leading to regurgitation.
o Arrhythmia: caused by heart block that occurs due to development of
aortic root abscess (Prolonged PR interval), the abscess compresses the
myocardium and the conductive system leading to heart block.
o Recurrent embolization: due to vegetation.
3. Large vegetation > 10 mm
4. Special situations: e.g., Fungal IE, Prosthetic valve IE.
• Prognosis:
- Worse with:
o Acute staph. aureus endocarditis.
o Fungal endocarditis
o Prosthetic valve endocarditis.
o Resistant microorganisms
o When HF is present.
- Mortality 20 – 30 %
• Prevention of infective endocarditis:
1. Good oral hygiene "very important" The most important factor for prevention
2. Dental procedures:
o Oral amoxicillin 50 mg/kg "single large dose' one hour before dental procedures.
3. GIT or Genitourinary procedures:
o 30 minutes before procedure: IV ampicillin (50 mg/Kg) and IV gentamycin
o 6 hours after procedure: oral amoxicillin (25 mg/Kg)

27 | Pediatrics Cardiology
 Heart Failure
• Definition:
- It is failure of the heart to maintain COP adequate for tissue perfusion

• Causes
- Congenital heart diseases are the commonest causes of heart failure in infancy
- Rheumatic heart diseases (in school age)
- Infective endocarditis
- Myocarditis
- Dilated cardiomyopathy.
- Severe anemia
- Dysrhythmia: Supraventricular tachycardia, Complete heart block
- Nutritional e.g. Beri Beri, Kwashiorker, Keshan disease (selenium deficiency)

28 | Pediatrics Cardiology