APPROACH TO PAEDIATRIC CARDIOLOGY

•	History.
•	Physical examination.
•	Investigations.
•	Management

History

1.	Presenting complaint: reason for current admission.

2.	Diagnosis

a.	When made.

b.	Where.

c.	Symptoms at diagnosis: cyanosis, tachypnoea, poor feeding, failure to thrive, recurrent infection.

d.	Investigations: CXR, ECG, Echo, angiography.

3.	Initial treatment

a.	Surgical: palliative such as balloon septostomy or shunting procedures, or corrective such as patent ductus ligation.

b.	Pharmacological: e.g. digoxin and diuretics and side-effects thereof.

4.	Past history

a.	Aetiology: rheumatic fever, pregnancy complicated by rubella, smoking, alcoholism or drugs such as lithium, warfarin or phenytoin.

b.	Indications for and number of previous hospital admissions.

c.	Pattern and timing of change in condition: when cyanosis or heart failure developed, when and how it was controlled.

d.	Episodes of infective endocarditis.

e.	Other complications of disease: cerebral thrombosis or abscess.

5.	Treatment

a.	Past surgery, complications thereof, plans for further operative procedures.

b.	Past interventional catheter procedures.

c.	Past ablation therapy, pacemaker or defibrillator placement.

d.	Medications, past and present, side-effects of these, monitoring levels, treatment plans for the future.

e.	Exercise restriction.

f.	Recommendations for antibiotic prophylaxis for dental procedures, maintenance of dental hygiene.

g.	Compliance with treatment.

h.	Instructions for air travel and high altitude.

I.	Any recent investigations monitoring treatment, e.g. Holter monitoring.

j.	Any recent changes in treatment regimen, and indications for these.

6.	Current state of health

a.	Symptoms of cardiac failure:

i.	fatigue and shortness of breath.

ii.	cough.

iii.	sweating.

iv.	poor feeding.

v.	recurrent chest infections.

b.	Cyanosis, squatting with ‘Fallot’s turn’.

c.	Any episodes suggestive of arrhythmias: syncope, alteration of consciousness, dizziness, palpitations.

d.	Chest pain.

e.	Headache.

7.	Associated problems

a.	Main diagnosis: Down syndrome, Marfan syndrome.

b.	General problems: poor growth, developmental delay, exercise limitations.

8.	Social history

a.	Disease impact on child:

i.	growth and development.

ii.	schooling.

b.	Disease impact on parents.

c.	Disease impact on siblings.

d.	Social supports.

e.	Coping: contingency plans, parents’ degree of understanding, antibiotic prophylaxis for operations and dental procedures.

9.	Family history

a.	Early cardiovascular death, e.g. hypertrophic cardiomyopathy.

b.	Unexplained death or arrhythmias.

c.	Any known cardiovascular diagnoses or known syndromes.

Clinical Examination

1.	General inspection

a.	Comfortable at rest.

b.	Tachypnoeic, in respiratory distress.

c.	Cyanosis.

d.	Dysmorphic features: Down, Noonan, Turner, Marfan.

2.	Parameters

a.	Height:

i.	short: Down, Noonan, Turner, heart disease causing failure to thrive.

ii.	tall: Marfan.

b.	Weight: failure to thrive in congenital rubella, severe heart disease, cyanosis or congestive cardiac failure.

c.	Head circumference: small in congenital rubella.

3.	Upper limbs

a.	Nails: clubbing in cyanotic heart disease, SBE.

b.	Pulses:

i.	feel both brachial and femoral pulses.

ii.	delay or absent femoral pulse: coarctation of aorta.

iii.	bounding pulses: PDA.

iv.	small pulses: low output heart failure.

c.	Blood pressure:

i.	upper limb: 2/3 of arm centered over artery.

ii.	lower limb: 2/3 of thigh centered over artery.

iii.	all 4 limbs if any suggestion of coarctation.

d.	Normal upper limit for pulse, respiratory rate and BP:

Age group	Pulse rate	Respiratory rate	Systolic BP	
0 – 8 weeks	160	50	70	
Older infant	145	40	85	
Toddler	130	30	100	
Older child	115	20	115	

4.	Head and neck

a.	Eyes:

i.	conjunctival pallor: anaemia.

ii.	scleral icterus: fragmentation haemolysis with artificial valves.

b.	Lips and tongue for cyanosis.

c.	Teeth: dental caries.

d.	Jugular venous pressure.

5.	Chest

a.	Chest asymmetry:

i.	left chest prominence: chronic right ventricular hypertrophy.

ii.	right chest prominence: dextrocardia with chronic ventricular hypertrophy.

b.	Respiratory rate: tachypnoea with left ventricular hypertrophy.

c.	Scars:

i.	median sternotomy: all open heart corrections.

ii.	lateral thoractomy: coarctation repair, pulmonary aryery banding, ligation of PDA, Blalock shunt.

iii.	groin: cardiac catheters.

6.	Palpation

a.	Apex beat: location and quality of impulse.

b.	Heaves: at parasternal border and substernal region.

c.	Thrills: suprasternal and supraclavicular regions.

d.	Palpable P2: feel over pulmonary area for closure of the pulmonary valve.

7.	Heart sounds

a.	Heart sounds 1 and 2: intensity, splitting.

b.	Additional heart sounds: ejection clicks, S3.

8.	Analysis of murmurs

a.	Timing.

b.	Localization.

c.	Amplitude. 

d.	Characterization.

e.	Radiation.

9.	Types of Murmurs

a.	Ejection systolic murmurs:

i.	crescendo-decrescendo in character.

ii.	begins shortly after S1 and finishing close to S2.

iii.	tend to originate from turbulence during ejection of blood from the heart to the great vessels.

iv.	examples: murmurs of pulmonary or aortic valve stenosis.

b.	Pansystolic murmurs:

i.	commence at S1 and continue to S2, usually being of similar amplitude throughout systole.

ii.	may be due to atrioventricular valve incompetence or a VSD and are caused by flow from a high pressure ventricle to a lower pressure area within the heart.

iii.	such murmurs are generally heard over the lower precordium or at the apex.

c.	Diastolic murmurs:

i.	early: aortic or pulmonary incompetence.

ii.	mid: turbulence of blood passing through one of the atrioventricular valves during ventricular filling and reflect either stenosis or increased flow through the valve.

iii.	late: mitral valve prolapse or late diastolic murmur associated with mitral stenosis.

d.	Innocent murmurs:

Murmur	Features	
Still’s murmur	•	ESM at left sternal border.
•	Most common innocent murmur.
•	Low frequency, best heard with bell.
•	Heard from infancy to adolescence.
•	Becomes softer if patient stands and louder when squatting or lying supine.	
Pulmonary flow murmur	•	2nd most commonest.
•	Soft blowing ejection murmur (ESM) at the pulmonary area.
•	Loudest at 2nd and 3rd left infraclavicular space.
•	Common in infants and children.	
Carotid bruit	•	Medium frequency.
•	Rough ESM heard over root of neck.
•	Common in children.	
Venous hum	•	High-pitched.
•	Blowing, continuous murmur heard over the sternoclavicular junctions or over neck. 
•	Occurs in 3 – 8 years old.
•	Intensity increases with sitting or erect, inspiration and turning of head to contralateral side.	

10.	Differentiating Innocent and Pathologic murmurs

Innocent murmur	Pathologic murmur	
•	Early systole.
•	Short duration.
•	Low intensity.
•	Crescendo-decrescendo.
•	Occur in an asymptomatic child with no other CVS abnormalities.	•	All diastolic murmur.
•	All pansystolic murmur.
•	Most late systolic murmur.
•	Loud murmur > 2/6.
•	Continuous murmur except venous hum.
•	Any murmur with CVS signs.	
11.	Heart sounds, Murmurs and their differentials

Heart sound / murmur	CVS lesion	
Apex beat	•	VSD: displaced.
•	PDA: displaced and forceful.
•	TGA: forceful right ventricular impulse at left sternal edge. 	
Parasternal heave	•	VSD.
•	ASD.
•	PS.	
Thrill at suprasternal notch	•	Coarctation of aorta.
•	PS / AS.
•	PDA.
•	VSD.
•	PTA: thrill at base of heart.	
Splitting of S2	•	ASD: fixed and wide splitting.
•	AVSD.
•	PS: with soft S2.	
Loud S1	•	MS.	
Soft S1	•	MI.
•	Myocardial infarct.	
Loud S2	•	Pulmonary hypertension.
•	VSD.
•	AVSD.
•	TGA + TF.
•	PTA.	
Loud A2	•	FT
•	Large VSD.	
Ejection click	•	PS / AS.
•	FT.
•	MVP.	
PSM	•	VSD: loud harsh PSM at lower left sternal edge.
•	AVSD.	
ESM	•	ASD: at pulmonary area.
•	AVSD: at pulmonary area.
•	PS: at pulmonary area radiating to neck and back. 
•	TF: ESM along left sternal edge and in pulmonary area radiating through to the back. 	
MDM	•	VSD
•	ASD: soft MDM at lower end of sternum.
•	AVSD.	
Continuous	•	PDA: continuous machinery murmur at upper left sternal border.	
Quiet praecordium	•	Large VSD.
•	TGA.	

12.	Others

a.	Lung fields: crepitations in LVF.

b.	Liver: palpate edge and measure span – enlarged in RVF.

c.	Splenomegaly in SBE.

d.	Palpate back for sacral edema. 

e.	Manoeurves:

i.	Valsalva: increases intensity of murmur in HCM and MVP.

ii.	exercising child by doing sit-ups to accentuate tricuspid diastolic murmur in an ASD. 

f.	Request for:

i.	urinalysis.

ii.	temperature chart.

iii.	fundoscopy.

13.	Summary

a.	Determine lesion:

i.	cyanotic / acyanotic CHD.

ii.	increased / decreased pulmonary blood flow.

iii.	RVH / LVH.

iv.	is pulmonary hypertension present?

b.	Is CCF present?

c.	Are there associated lesions?

d.	Etiological cause.

Investigations

1.	Chest X-ray

a.	Name and date.

b.	Side marked right.

c.	Centering and penetration of flim.

d.	Cardiac diameter.

e.	Cardiac contour for:

i.	size of pulmonary artery.

ii.	position of aortic arch.

f.	Lung fields: pulmonary vasculature.

g.	Bony structures: rib notching.

h.	Increased pulmonary vasculature occurs in:

i.	truncus arteriosus.

ii.	total anomalous pulmonary venous drainage.

iii.	transposition of great arteries.

I.	Decreased pulmonary vasculature occurs in:

i.	pulmonary atresia.

ii.	tricuspid atresia.

iii.	Ebstein’s anomaly.

iv.	tetralogy of Fallot. 

2.	ECG

ECG finding	Pathology	
Prolonged PR	•	Ebstein’s anomaly.
•	Acute rheumatic fever.
•	Congenital block (maternal SLE)
•	Endocardial cushion defect.	
Partial RBBB 
with LAD
with RAD.
with RAH, delta wave	
•	Ostium primum ASD.
•	Ostium secundum ASD.
•	Ebstein’s anomaly.	
LAD	•	Endocardial cushion defect.
•	Tricuspid atresia.
•	Hyertrophic cardiomyopathy.
•	Inlet VSD.	
RAH
without RVH

with RVH	•	Ebstein’s anomaly.
•	Tricuspid atresia.
•	Pulmonary atresia.
•	Truncus arteriosus.
•	Tetralogy with large VSD.	
Deep Q waves	•	Hypertrophic cardiomyopathy.
•	Transposition of great arteries.	

3.	Echocardiography

a.	Examination of the heart with ultrasound provides detailed and quantitative information about cardiac anatomy and function.

b.	Cross-sectional echocardiography allows the sectional anatomy of the heart to be displayed on a television monitor.

c.	Doppler echocardiography allows quantification of the direction and flow of blood at various sites, providing useful information about the presence of and severity of stenoses.

d.	Types:

i.	M-mode: standard.

ii.	2D: 2 planes of view.

iii.	Doppler: for flow.

e.	Useful in:

i.	determining structural defects.

ii.	volume of each chamber.

iii.	assessing abnormalities in flow and performance.

4.	Cardiac catheterization

a.	Allows detailed measurement of intracardiac pressures, quantification of shunts assessment of valve gradients and pulmonary hypertension.

b.	Allows for angiographic demonstration of the anatomy of cardiac defects and abnormalities in the pulmonary or systemic arteries.

c.	May be used for therapeutic purposes such as balloon valvuloplasty or placement of a device to close a PDA or ASD. 

d.	Usage:

i.	oximetry: measure PO2 in various chambers.

ii.	manometry: measure pressure and the gradient across chambers.

iii.	angiography: determine structural and functional abnormalities.

iv.	therapy: PS ballooning, balloon atrial septostomy in TGA.

e.	Indications:

i.	all cyanotic CHD.

ii.	for acyanotic CHD: PS, large VSD, coarctation.

Management Issues

1.	General development, growth and nutrition.

2.	Prophylaxis against endocarditis risk

a.	Dental procedures and dental care:

i.	prophylaxis is usually given as amoxycillin, one hour before the procedure, orally, or when anaesthesia is needed, parenterally.

ii.	the usual oral dose is 3g for a child over 10 years, and 1.5g if under 10 years of age.

b.	Genitourinary and gastroenterological surgery: give parenteral gentamicin and ampicillin.

c.	Prosthetic heart valves: highest risk of acquiring endocarditis.

d.	Non-cardiac surgery: patient groups of concern are those with congestive heart failure, pulmonary hypertension, severe cyanosis.

3.	Infections

a.	Common infections:

i.	gastroenteritis can lead to dehydration and result in thromboses in polycythaemic patients; care must be taken to avoid dehydration.

ii.	gastroenteritis can also lead to hypokalaemia in patients taking diuretics, or cause toxicity in patients taking digoxin.

iii.	any febrile illness may precipitate cardiac decompensation via the increase in the body’s metabolic needs.

b.	Cerebral abscess:

i.	children over 2 years of age with cyanotic congenital heart disease have an increased incidence of brain abscess.

ii.	they may present with fever, headache, seizures or focal neurological signs.

4.	Social issues

a.	Impact of disease:

i.	the child may be pampered and overprotected as a young child.

ii.	during adolescence, issues such as anxiety regarding contraception, sexual performance and peer approval become important.

b.	Schooling: 

i.	future employment.

ii.	symptoms which warrant referral for a medical opinion.

c.	Exercise: avoidance if there is severe pulmonary hypertension.

d.	Adolescence:

i.	non-compliance.

ii.	peer group acceptance.

iii.	non-participation in sporting activities.

iv.	delayed puberty.

v.	life insurance.

vi.	driver’s licence.

e.	Pregnancy:

i.	contraindications: severe pulmonary hypertension, congestive cardiac failure and cyanosis.

ii.	teratogenic drugs, e.g. warfarin should be ceased for pregnancy. 

f.	Travel: patients with congestive cardiac failure , cyanosis or pulmonary hypertension must avoid high altitudes and require supplemental oxygen to be available during air travel.

g.	Genetic counseling:

i.	most cardiac defects have a multifactorial inheritance, with a recurrence risk of between 1% and 4%. 

ii.	exception: hypertrophic cardiomyopathy which has an autosomal dominant mode of inheritance. 

5.	Drugs

a.	Commoner medications for congenital heart disease are digoxin, diuretics and antiarrhythmics.

b.	Consider appropriate dose and adverse side effects, monitoring of levels and of electrolytes.

c.	In children with prosthetic valves receiving warfarin therapy, the prothrombin ratio should be checked regularly.

6.	Contraindications of oral contraceptive pill:

a.	Prosthetic heart valves.

b.	Cyanotic heart disease.

c.	Pulmonary hypertension.

d.	Smokers.