Paediatrics – Cardiology and Vascular

ASD/AVSD
Definition
Acyanotic congenital heart condition characterized by malformation of A/AV septum.

Aetiology
 Secundum ASD: PFO (abnormal resopriton of septum primum in formation of
secundum)
 Primum ASD (partial AVSD): defect in lower atrial septum, due to incomplete
fusion of septum primum with endocardial cushion. Immediately adjacent to AV
valves (tricuspid/mitral).
 Sinus venosus ASD: defect high in atrial septum near to SVC entry
 Coronary sinus defect: unroofed coronary sinus and persistent left SVC into left
atrium
 Complete AVSD: large central defect due to ASD/VSD with single large AV valve

Epidemiology
1/100k. Associated with genetic/chromosomal anomalies, i.e. trisomy 21.

History
Depends on defect size and type. May manifest at neonatal (recurrent RTI, CHF,
breathless, fail to thrive), childhood (routine examination, murmur) or later (pulmonary
HTN, arrhythmia, MV disease).

Examination
ASD: left to right shunt  pink and breathless. Murmurs due to high volume of flow
(blood from left enters right) across small right-sided valves at high pressure. Not due to
the shunt itself. Can hear both an ejection systolic murmur (pulmonary valve) and a mid
diastolic murmur (tricuspid valve).
Paediatrics – Cardiology and Vascular

ASVD: Mixed shunt  blue and breathless. Atrial + ventricular components, therefore
presents with pulmonary HTN and a large VSD. May be cyanosed at birth, murmur in the
first few weeks.

Investigations
CXR: RAH, RVH, prominent PA and increase in vascular markings
Echo is gold standard, with or without Doppler.
ECG can shor RAD, IDHB, RBBB, RAH/RVH.

Management
Supportive: antibiotic prophylaxis if dental surfery, medical treatment for CHF.
Surgical: closure prevents RVH and arrhythmias. Repair of associated anomalies at the
same time. This usually occurs at 2-5y unless severe MR.
AVSD/ASD: closure is via sternotomy approach, fixing of the defect with pericardial
autologoisly or with Dacron/PTEF patches. Needs extracorporeal support. Percutaneous
transcatheter can be done too.
Complete AVSD: 3/12, requires tx of pulmonary vascular resistance if present at birth.

Complications / Prognosis
Infective endocarditis, CHF, AF, arrhythmias, cyanosis, pHTN. 95% remain open, 5%
close spontaneously. Small defects cause little morbidity.
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Paediatrics – Cardiology and Vascular

Coarctation of the aorta (CoA)

Definition
Narrowing of the limen of the aorta, producing obstruction. Defined by its relation to the
ductus arterious (DA, ligamentum arteriosum after regression). Preductal, ductal or
potductal.

Aetiology
Failure of normal development of the left 4th and 6th aortic arches (6/40).
Related to Turner, rubella, VSD, PDA, hypoplastic left heart (HLH), interruption of AA.

Epidemiology
1/10.000

History
Neonatal: in ductal/postductal forms, when ductus arteriosus closes, the sudden
decrease in outflow leads to pressure build in the LV, leading to sudden LVF (dyspnea
etc).
Infant: symptoms depend on severity and collaterals. Preductal has good collaterals,
postductal less. May be asymptomatic until adolescence if good collaterals.

Examination
Nodding type head movements with HR, delayed or absent femoral pulse, RR delay,
difference in BP in both arms, (>20mmHg).
Loud S1 and narrow split S2, collateral flow/coarctation murmur.

Investigations
CXR: cardiomegaly, increased pulmonary markings in LVF. Hypoplastic aortic knob with
dilated poststenotic segment. ECg may show LVH.
Doppler echocardiogram is diagnostic.

Management
Medical: infusion of PGE1 in neonates to maintain open DA. Palliate symptoms of LVF
with fluid restriction and diuretics.
Surgical: resection of the coarctation, end to end anastomosis or subclavian artery flap
repair. 10% recoarctation.
Baloon angioplasty: alternative to surgery.

Complications / Prognosis
Hypertension: CoA  decreased systemic BP  low blood flow to renal  low
perfusion pressure of tubule cells  high renin production  high aldosterone 
Na/H2o reabsorb  HTN.
May cause aortic dissection, rupture of aneurysm, and CAD.
Untreated do not survive past 50 typically. With correction 20y survival 91%.
Paediatrics – Cardiology and Vascular

Heart failure
Definition
Failure of the heart to meet the metabolic demands of the system. Congestive heart
failure relates to increased venous congestion in the pulmonary vasculature (LHF), or
systemic (RHF) veins. This occurs when the mechanisms regulating CO are no longer
adequate. Common in syndromic children.

Aetiology
Overcirculaiton: normal blood flow disrupted, heart does not keep up
 CHD: HLH, severe AS, interrupted AA/CoA, PDA, TAPVD, large VSD, TGA, truncus
arteriosus.
 Postoperative CHD repair
 AV malformation (i.e. hepatic)
 Severe anaemia (i.e. hydrops fetalis)
Pump failure: damage to heart muscle, fails to contract
 Viral myocarditis/cardiomyopathy
 Metabolic cardiomyopathy (i.e. Pompe disease)
 Arrhythmias: SVT, VT, congenital heart block
 Ischaemia: post Kawasaki disease
 DMD
 Medications i.e. chemotherapy

Epidemiology
Common in syndromic children. Rare in normal.

History
Infant: breathless, cold extremities, wheeze (cardiac asthma) , grunting, feeding
difficulties, sweat, fail to thrive, recurrent chest infetions.
Older: fatigue, exercise intolerance, dizziness, syncope

Examination
General: tachycardia (not in CHB), absence of heart murmur does not exclude this (TGA,
HLH, COA)
Left CHF: resp distress, gallop rhythma, displaced apex, tachypnea
Right CHF: odema, hepatosplenomegaly, JV distension not often in child.
Uncompenated CHF: hypotension, cool peripheries, shock, low urine output, thread
pulse, high capillary refill time, renal and hepatic failure.

Investigations
Bloods: acid base balance, FBC, WCC, viral PCR, culture for I.E, electrolytes (SIADH
screen)
CXR: cardiomegaly, signs of HF (Kerly B, pulmonary odema, increased markings,
effusions, cardiomegaly)
ECG: rate, rhythm, hypertrophy or hypoplasia. Evidence of myocarditis or ischaemia.
Echo is diagnostic of CHD. Measure intracardial pressures,
Paediatrics – Cardiology and Vascular

Management
Emergency requiring admission to specialist unit.
Newborn: Duct dependent CHD is most likely cause – IV PGE2 should be initiated.
Reduce preload (loop diuretics) enhance contractility (ionotropes) reduce afterload
(ACEi), improve O2 delivery and nutrition.
Cardiac transplantation may be required n severe cases.

Complications / Prognosis
Arrhythmias, SVT, VT, CHB, cardiogenic shock.
Poor prognosis in children with uncorrectable CHD.

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Paediatrics – Cardiology and Vascular

Kawasaki disease
Definition
Childhood acute febrile illness with small and medium vessel vasculitis.

Aetiology
Epidmeiological studies suggst infectous agent in genetically susceptible individuals
 Infectious hypothesis – winter spring seasonality, community outbreaks
 Genetic susceptibility: Japanese individuals more prone
 Superantigen hypothesis ?
1% have positive FHx.
DDX: Streptococcal disease (Scarlet fever) viral infection (measles, EBV, enterovirus)
staphylococcal scalded skin syndrome.
?due to cytokine cascade stimulaiton, endothelial activaiton leading to systemic
vasculitis. Coronary artery aneurysm are caused by influx of neutrophils destroying the
inside lamina. Fibroblastic activaton may then lead to coronary artery stenosis.

Epidemiology
KD leading cause of acquired heart disease. Incidence 1/1000 in Japan.

History / Examination
Classic features: high fever >=4days, presence of >=4 of:
 Erythema, odema of hands and feet
 Diffuse maculopapular rash (within 5 days)
 Bilateral non exudative oconjunctivitis
 Peryhtma of lips and oral mucosa, strawberry tongue
 Cervical lymphadenopathy

Investigations
Blood: WCC, CRP, high platelets in subacute phase.
Microbiology: blood culture, nose throat, rectal swab ,ASOT.
ECHO angiography: KD can be diagnosed if coronary artery aneurysm is seen, fever or
classic fatures.

Management
Abx: until bacterial infection gone.
IVIG: decreases risk of cardiac complications. High dose (2g/kg) for 7-10d.
Aspirin: high dose, 1.5mg/kg until day 14 followed by low dose antiplatelet therapy
until echo. Steroids only considered if two IVIG infusions ineffective.

Complications / Prognosis
CAAs in 25% if untreated, ma lead to MI or SD or IHD.
KD usually self limiting and resolves spontaneously without treatment 4-8wk. Mortality
0.8% Japan 3.7% UK.
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Paediatrics – Cardiology and Vascular

Patent ductis arteriosus

Definition
Fsilure of closure / reopening of the ductus arteriosus postnatally.

Aetiology
Usually used as bypass for the pulmonary circulation when fetus in mother. Enters
distally to the origin of the left subclavian artery on the aorta. Fetal patency ensured by
ductal production of PGE2 but this falls at birth and DA shold close within 2 weeks.
Normal closure at 15h, some have it after first breath (funcitonal closure, contraciton of
SM). True anatomical closure takes weeks (permanent closure, proliferation of
fibroblasts).
Associated with prematurity, perinatal distress, fluid therapy, hypoxia. May have
congenital rubella, chromosomal abnormality, fetal alcohol syndrome, maternal
amphetamines, FHx.

Epidemiology
<40% in <1500g. 1/2000 term deliveries.

History
 Small: asymptomatic
 Medium: low exercise tolerance, breathless, hoarse cry/cough as child, LRTI
comon and alectasis/pneumonia.
 Large: symptoms of compensated HR, dyspnea and failure to thrive. Neonatal
aopnea/bradycardia.

Examination
 Premature: rough systolic murmur in left sternal border. Bounding pulses.
 Small: normal pulses, BP continuous machinery murmur and thrill below left
clavicle (pressure gradient between aorta and pulmonary vessel.
 Medium: wide pulse pressure, bounding peripheral ulse, LRTI.
 Large: absent thrill, murur in systole only as pressures of aorta and arteries are
equal in diastole.
 CHF: tachycardia, tachypnoea, respiratory distress, displaced apex, cool periph.

Investigations
CXR: cardiomegaly, signs of HF
ECG: LVH
Doppler Echo: diagnostic to confirm patency.

Management
Preterm: conservative if asymptomatic, monitor, fluid restrict, PGE2 inhibitor (stop
them holding closed). Surgical ligaiton if symptomatic. In term children, minimal access
cloure with cardiac catheterisation in >1yo.

Complications / Prognosis
High risk of PDA associated endocarditis, preterm (IVH, bronchopulmonary dysplasia,
CHF). pHTN. Eisenmenger syndrome. Emboli. Untreated mortality 20%, With closure
none..
Paediatrics – Cardiology and Vascular

Pulmonary valve stenosis

Definition
Acyanotic heart disease associated with right ventricular obstruction due to a stenotic
malformed pulmonary valve.

Aetiology
General: may be valvular 80% or subvalvular (infindibulum) or uspravalvular. Low
pulmonary orifice size = outflow obstruction.  RVH and RA dilatation.
Graded by peak gradient pressure: mild <50, moderate 50-80, severe >80mmHg.
 Congenital valvular stenosis: common variants include dome shaped valve, fused
leaflets, uncomonly may be bicuspid or tricuspid.
 Acquired valvular stenosis: rheumatic fever, endocarditis, malignant

Epidemiology
Noonan syndrome, ASD, VSD, PDA, PFO, TOF and familial. 10% of congenital heart defect

History
Mild stenosis: incidental murmur detection
Moderate: dyspnea, odema, fatigue
Severe: exercise intolerance, angine or HF. If very severe, presents with cyanosis from
RL shunting through foramen ovale in associated ASD.

Examination
Mild/moderate: child acyanotic, RV heave, systolic thrill. ESM. S1 with ejection click and
s2 widely split. ESM loudest in P area (left sternal border upper). Intensity NOT related
to severity.
Severe stenosis: cyanosis, heart failure with tricuspid insufficiency, giant A waves in JVP,
hepatomegaly and pulsatile liver. S4.

Investigations
CXR: normal heart size, RA dilation, PA dilation post stenosis, low pulmonary blood flow.
Echo is diagnostic, Doppler, determine the amount of stenosis.
ECG may be normal, may show ventricular hypertrophy.

Management
Mild: conservative, follow up with echo
Moderate: if symptomatic, treat as severe.
Severe: surgical intervention, minimally invasive baloon pulmonary valvuloplasty. May
do open aproach, valvotomy with inflow occlusion, hyopthermia and cardiopulmonary
bypass).

Complications / Prognosis
RVH, CHF. Mild usually does not progress, but moderate becomes severe. Px is good with
surgery. 25% neonates require 2 interventions and 10% older children. Life span
normal.
Paediatrics – Cardiology and Vascular

Supraventricular tachycardia (SVT)

Definition
Narrow complex tachycardia due to abnormal mechanism oiginatimg proximally to the
bifurcation for the Bundle of His.

Aetiology
General: 15% of infants <4/12 and 93% of children have predisposing factors (illness,
CHD, pyrexia).
Four categories:
1. Atrial tachycardia, primary
2. SVT using accesory pathway (WPW) or concealed accessory connection. Most
common.
3. Re-entrant tachycardia without accessory pathway, such as AVNRT or atrial
flutter.
4. Juncitonal ectopic tachycardia.
Episodes are life altering but not life threatening. May be due to acid base/electrolyte
disturbance etc.

Epidemiology
4/1000. Peak 1yo.

History / Examination
General: sudden onset and sudden resolution.
Children: HR 180-300 BOM, palpitaitons.
Infants: more obscure diagnosis, HR 200-300. Usually present with HF, acutely ill in
attacks lasting >6h, ashen complexion, irritable.
Neonates: differentiation from sinus tachycardia is difficult but HR is invariable and
with abnormal P wave, >230bpm.
NB pathology of re entry: premature atrial beat (happens in everyone)  through
ventricle through normal AV pathway  ventricular rresponse finds AV pathway
refractory, but accessory tract conducts readily back to atrium back to atrium as echo
beat  retransmitted to ventricle  repeat

Investigations
ECG: (MA tripple criteria) Narrow complex, tachycardia 250+, may have myocaridal
ischaemia i.e. T wave inversion in lateral precordial leads.
WPW: characteristics seen at rest: short PR and slow upstroke of QRS (delta wave)

Management
 Vagotonic manouvre: Valsava, face in iced saline, breath holding, carotidsmassag
 IV/IO adenosine in acute situation, induces IV block terminating re entry circuit
 Synchronised DC cardioversion if adenosine fails or haemodynamic compromise
present.
 Recurrent: accessory pathway ablation.

Complications / Prognosis
Hydrops fetalis, HF, MI, CMP. Symptomatic WPW have a small risk of SD.
Paediatrics – Cardiology and Vascular

Teratology of Fallot
Definition
Cyanotic cardiac malformation encompassing these four:
 Large VSD
 Infundibular and valvular pulmonary stenosis
 Right ventricualr hypertrophy
 Overriding of aorta relative to ventricular septum (superior to VSD)

Aetiology
Complex abnormalities due to abnormal development of right ventricular infindibulum.
Related to fetal hydantoin, CBZ, fetal alcohol, DiGeorige, trisomy21.
Severity is mainly related to degree of pulmonary outflow obstruction. Low blood in
lungs  high RV pressure  RVH  increase reistance to outflow  higher RV
pressure  increase R-L shunting  deoxygenated blood in circulation.
Hypoxic spells occur in times of high RL sunting.

Epidemiology
5/10k

History/Examination
Birth: cyanotic. Low BW
Infant: hypoxic spells, harsh ESM at left sternal edge (PS), loud single second heart
sound, parasternal throust for RVH.
Child: often adopt squatting position in spells to increase systemic vascula resistance
and increase venous return, decreasing R-L shunt. Signs of HF, developmental delay.

Investigations
Echo for diagnosis.
CXR may show RVH, boot shaped heart, low lung markings and vascularity. ECG. (RAD,
RVH, RBBB)

Management
Treat cyanotic spells: soothe, knee chest position, calm increase venous return. If
prolonged, IV morphine for sedation.
Corrective surgical intervention: VSD patch to close, treat other associated anomalies
too. Repair at >1y. Preoperative stabilisaiton with PGE2 for PDA.

Complications / Prognosis
Hypoxia, MI, ischaemia in brain, secondary polycythaemia (therefore leading to
thrombotic events), infective endocarditis, cerebral abscess, delayed growth and
puberty.
Pre surgery 30% mortality in first year of life, 75% by 10y. Now, 90% survive to be
adults. .
Paediatrics – Cardiology and Vascular

Transposition of the great arteries

Definition
Cyanotic congenital malformation characterised by transposition of the aorta and
pulmonary artery.

Aetiology
Unknown, Embryologically linked to perisstence of the subaortic conus and
reapsorption or underdevelopment of the subpulmonary conus (infundibulum).
Abnormality aligns the oarta superior to the RV during development and is
characterised by AV concordance but ventriculoarterial discordance. Classificaiton
anatomical and depends on relaitonship of great vessels to each other and infundibular
morphoogy. Isolated in 50%, part of other malformations in 10%.

Epidemiology
3/10k.

History / Examination
Depends on the extent of intercircualtory mixing and other anatomical lesons.
 TGA alone, NO VSD: cyanosis ay birth
 TGA with large VSD: may be asymptomatic initially but exhibits cyanosis when
crying. Parasternal heave due to RVH. 3-6 weeks signs of CHF.
 TGA with VSD with PS: Similar to TGA alone.
 TGA with VSD and pulmonary vascular obstructive disease: progressie cyanosis
despite baloon arterial septostomy.
Related to metarnal rubella, poor nutrition, fetal alcohol, exposure to herbicide, high
maternal age and diabetes.

Investigations
Echo: diagnostic
CXR shows narrow mediastinum, high pulmonary markings, egg shaped heary due to
RVH.

Management
Medical: preoperative correction, correct electrolytes and PG infusion to maintain PDA.
Interventional: baloon septostomy with catheter passed into foramen ovale. Baloon
inflated and pulled through, tearing septum and allowing mixing circulaiton.
Surgical: arterial switch procedure: once stablisied and 4/52, pulmonray artery and
aorta transected and switched back. Coronary arteries are also transferred.

Complications / Prognosis
CCF, arrhythmias, PHT, polycythaemia and then clotting, Eisenmenger syndrome.
Untreated mortality 90%, with surgery 90% survive.

Eisenmenger: occurs when a long standing LR cardiac shunt causes enough
pulmonary HTN to precipitate the reversal of the shunt into a cyanotic RL shunt.
Paediatrics – Cardiology and Vascular

VSD ventricular septal defect

Definition
Acyanotic congenital heart condiiton due to a defect in the intraventricular spetum and
therefore mixing of blood.

Aetiology
Can occur primary abnormality or be associated with other disease. Classified due to
position
 Perimembranous : most common, 80%, present in the LV outflow tract inferior
to aortic valve.
 Muscular: trabercular, <20%, presentin the muscular septum, multiple.
 Supracristal: less common, 8%, inferior to pulmonary valve.
Related to trisomy 18, 21, 13, maternal diabetes and phenylkeotnuria, alcohol, FHx.

Epidemiology
4/1000. Most common cardiac defect.

History
Asymptomatic or symptomatic depending on size and where. Large cause fatigue,
sweaty in feeds, recurrent RTIs, CHF and failure to thrive.

Examination
Small defect: blood flowing through VSD results in loud harsh blowing high pitched
pansystolic murmur. May be associated with parasternal thrill.
Large defect: parasternal heave due to RVH, systolic murmur softer. Additional diastolic
murmur due to increased flow through the mitral valve.

Investigations
Doppler with echo diagnostic.
CXR may show cardiomegaly, high pulmonary vasculature, ECG may show RVH/LVH.

Management
Medical: Abx procedure prophylaxis, medical tx of HF.
Surgical indicaiton: if CHF, large defects with high pulmonary pressure, prolapse of
aortic valve etc. Transatrial aproach or pulmonary valve approach. Left ventriculotomy
with patch repair. Minimally invasive also available.

Complications / Prognosis
Infective endocarditis, CHF, pHTN with Eisenmenger.
Spontaneous closure can occur over 2y, uncommon in >4y. Closes in 80% of muscular
but only in 40% of perimembranous
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Paediatrics – Cardiology and Vascular

ASD/VSD
Definition

Aetiology

Epidemiology

History

Examination

Investigations

Management

Complications / Prognosis