Cardiac disorders in children

Dr.MC Yam
Department of Paediatrics
Heart problem in children

 Congenital Heart Disease

• Acyanotic
• Cyanotic
 Acquired Heart disease
• Kawasaki disease
• Rheumatic fever
• Cardiomyopathy
• Infective endocarditis
• Hypertension
• Cardiac arrhythmias
 CHD is the most commonest congenital
malformation
 Incidence of congenital heart disease:
• worldwide: 6-10 per 1,000*
• HK: 6.35 per 1,000**
* at least 36% were considered as major cardiac
malformations
* Congenital Heart Disease in 56,109 Births - Incidence and Natural
History
Mitchell SC, et al
Circulation 1971; 43:323-332
** Echocardiography as a tool for determining the incidence of congenital
heart disease in newborn babies : a pilot study in Hong Kong
Sung RY, et at
Int J Cardiol 1991; 30:43-47
 Aetiology of Congenital
Heart Disease
• Majority remains unknown and most cases are
sporadic
 Chromosomal abnormalities
• Down syndrome - atrioventricular septal defect
 Familial
 Environmental
• Congenital rubella infection - Patent ductus arteriosus
• Maternal collagen disease - Complete congenital
heart block
 Presentation of heart disease in
newborn

 Asymptomatic heart murmur

 Respiratory distress (heart failure)
 Cyanosis
 Shock
Asymptomatic murmur

 The most common presentation of heart disease

in newborn
 It is the murmur itself which draws the doctors
attention to the possibility of a cardiac defect
 Other manifestations of heart disease are
usually absent or mild
 Differential diagnosis of
asymptomatic murmur

 Ventricular septal defect

 Fallots tetralogy
 Pulmonary stenosis
 Patent ductus arteriosus
 Aortic stenosis
 Atrial septal defect
 Innocent murmur
Heart failure

 Cardinal signs
• Tachypnoea
• Tachycardia
• Hepatomegaly
 Feeding difficulty
 Slow weight gain
 Failure to thrive
Differential diagnosis of respiratory
distress
 Large left to right shunt (VSD, PDA, AP window)
 Hypoplastic left heart syndrome
 Aortic arch interruption
 Coarctation of aorta
 Complex defect
Cyanosis

 Deoxyhaemoglobin >5g/dl
 Central cyanosis sometimes will be the dominant
features, especially during feeding
 Other manifestations of cardiac distress or
failure are absent or less prominent
Differential diagnosis of cyanosis

 Fallot’s tetralogy
 Transposition of great arteries
 Pulmonary atresia
 Tricuspid atresia
 TAPVD (obstructed)
 Complex heart lesion
Shock

 Typically in those ductal dependent heart lesions

 Occurs in 4-7 days after birth
 Desperately ill, with generalized pallor, mottling
or cyanosis and cold peripheries
 Pulses are either extremely weak or impalpable
 Respiration is laboured or gasping
 Frequently hypotonic and unresponsive
Differential diagnosis of shock
 Hypoplastic left heart syndrome
 Aortic arch interruption
 Coarctation of aorta
 Complex defect
Presentation of CHD in childhood

 Symptoms related to heart failure

• Shortness of breath during exercise
• Feeding problems
• Growth retardation
• Delayed development
• Frequent chest infection (viral/bacterial)
 Chronic lung disease
Presentation of CHD in childhood

 Symptoms related to hypoxaemia

• Exercise intolerance
• Growth retardation
• Cyanotic spells
Treatment of CHD

 Surgery is the definitive treatment for most of the

CHD
 Medical/palliative
• Diuretic
 Frusemide
 Spironolactone
• Digoxin
• Vasodilators
 ACEI
 Hydrallazine
 Interventional cardiac catheterization
 Ventricular septal defect
 Location: subarterial, perimembranous,
muscular
 Size
 Associated lesions
• eg. Coactation of aorta
Acyanotic lesions
Natural history of VSD

 Decrease in size
 Spontaneous closure
 Congestive heart failure
 Infective endocarditis
 Infundibular stenosis
 Eisenmenger syndrome
Management of VSD

 Leave it alone+prophylaxis for infective

endocarditis
 Medical treatment to control heart failure
 Surgical repair
 Interventional catheterization to close the
VSD with a device
Cyanotic lesions
Natural course of TOF

 Depend on the degree and progression

of RV outflow tract obstruction
 Severe: present in neonatal period,
require urgent palliative surgery
 Moderate: present in infancy with
cyanosis +/- cyanotic spells
 Mild: mild cyanosis with complications
of polycythaemia (cerebral thrombosis
etc)
Blalock Taussig shunt
Management of TOF

 Total correction by surgery

 Palliative surgery (Blalock Taussig shunt)
 Prophylaxis for infective endocarditis
Other symptoms possibly related to heart
problems in children
 Dizziness/syncope
• More significant if happens during exercise
 Chest pain
• Usually musculoskeletal origin
• Coronary heart disease is rare in children
 Kawasaki disease
 Palpitation
• Supraventricular tachycardia
• Atrial/ventricular ectopics
Acquired Heart Disease in
Children
Kawasaki Disease
川崎病
 In 1961, Dr.Kawasaki noticed a 4 years
old boy having

 fever for 7 days

 swollen cervical lymph nodes
 injected conjunctivae
 fissuring lips
 body rash
 erythematous palms & soles with indurative
edema, desquamating later
Diagnostic Guidelines

 Fever for 5 days or more

 Changes of the extremities
Initial: reddening of palm & sole, indurative edema
Convalescent: desquamation from fingertips
 Polymorphous exanthema
 Bilateral conjunctival congestion
 Changes of lips & oral cavity
 Acute nonpurulent cervical lymphadenopathy
Incidence
 184.6/100,000 for children <5 yr in Japan
(2007)
 6 to 9 / 100,000 in USA
 53/100,000 in Hong Kong (2007)
Pathology
 Acute non-specific systemic vasculitis
 Small & medium sized arteries
 Transient coronary artery dilatation in 40%
of children in the acute stage
 Giant coronary aneurysm in 1%
 Aetiology unknown
Treatment
Newburger JW et al. The treatment of
Kawasaki syndrome with intravenous
gamma globulin.(N Engl J Med
1986;315:341-7)
 Aspirin 100mg/kg/day for 14 days and
IVGG 400mg/kg/day for 4 days (N=84)
 Aspirin 100mg/kg/day for 14 days only
(N=84)
Treatment
25

20

15
ASA
10 IVIG

0
0 2 7

% prevalence of coronary abnormalities

Treatment
Newburger JW. A single intravenous
infusion of gammaglobulin as compared with
four infusions in the treatment of acute
Kawasaki syndrome. (N Engl J Med
1991;324 ;1633-9)
 Aspirin +IVGG 400mg/kg/D for 4 days (N=276)
 Aspirin + IVGG 2g/kg/D for 1 dose (N=273)
Arrhythmia in children
Supraventricular tachycardia

 All forms of re-entrant tachycardia that require

participation of the AV junction to maintain the
tachycardia.
 The most common form of arrhythmia in child
 Incidence unknown from 1/25,000 to 1/250
 Incidence of WPW 3/1000 (Sano et al.
Circulation,1995)
Management of acute PSVT

 Valsalva maneuver
• blow against closed mouth & nose
 Vagal stimulation
• gap reflex
• ice in some form to the face (success rate 33-62%)
 Carotid massage ( one at a time)
 Eyeball pressure contraindicated ( retinal detachment)
Drugs used in PSVT

 Adenosine
 Digoxin
 Verapramil (avoid in infant <1 year old)
 Propanolol
 Amiodarone
 Cardioversion