Cardiac Problems in Children

M Rajimwale

Arrhythmias

Cardiac Problems in Children

Congenital heart disease

Myocardial/pericardial,endocardial

Congenital heart disease

Incidence - 0.8% live births

10% in still born/ abortus

< 10% chromosomal abnormality/genetic mutations

25% have extracardiac abnormality

Syndromes

Chromosomes

Downs (Trisomy 21) AVSD,VSD,TOF

Edwards (Tris.18) VSD, various defects

Patau (Tris.13) VSD, various defects

Turner (XO) Coarct.,AS

de-George (22q11deletion) Truncus,IAA,TOF

Williams (7q del) Supravalvar AS

 

More associations

Maternal Disease

Diabetes Mellitus – TGA,VSD, HOCM

SLE - Heart block

Associations

Oesophageal Atresia - VSD, TOF

Anorectal malformation-Any

Diag. Hernia - Any

Exomphalos - Any

Pierre Robin - VSD

Teratogens

Teratogenic Exposure

Rubella Coarct, VSD, PDA

Alcohol VSD

Phenytoin ASD

Lithium Ebsteins anomaly

Warfarin VSD, TOF

FOETAL CIRCULATION

Two intracardiac communications

Ventricles working in

parallel

>95%

>95%

>95%

>95%

75%

75%

75%

75%

3mm

25/3

8

100/8

25/10 100/60

Left heart

Right heart

LA

LV

RA

RV

AortaPA

VSD 30.5%

ASD 9.8%

PDA 9.7%

PS 6.9%

Coarctation of aorta 6.8%

AS 6.9%

TOF 5.8%

TGA 4.2%

Truncus 2.2%

TA 1.3%

Clinical Manifestations

• Cardiac failure – (Lt to Rt shunt – first few monthsLV outflow obstruction – few

days/weeksFunctional failure-cardiomyopathy)

– tachypnoea– tachycardia– poor feeding, sweating– failure to thrive– hepatomegaly

• Central Cyanosis -– duct dependant -

acutely unwell neonate

– cyanotic spells - TOF

CHD causing cyanosis-5 Ts –TOFTGATricuspid atresiaTAPVDTruncus ArteriosusPulm atresia

Clinical Manifestations...

• Incidental detection of murmur on routine examination

MURMUR OFTEN ABSENT IN CYANOTIC CONGENITAL HEART DISEASE

Clinical manifestations ...

• Infective endocarditis - rare < 2 years

• Sudden death - rare, HOCM, severe AS, long QT

• Palpitation, dizziness, fainting - arrhythmia, long QT syndrome

• Chest pain - rare, ischaemia - aortic stenosis, anomalous origin of coronary artery

pericarditis

Examination

• General exam – growth, dysmorhism, well/unwell– colour, perfusion, pulse (including femorals) , BP,

post-ductal SaO2

• CVS

inspection auscultation (supine and standing)

palpation

• Auscultation – heart sounds (intensity, splitting of 2nd sound)– systolic murmurs - intensity I - VI, phase of

cardiac cycle, area best heard, radiation (listen to neck, axilla, back), change with posture,

– diastolic murmurs - I - IV

• Other systems - respiratory, abdomen

Murmur Best heard Other features

VSD Harsh pansystolic

Lt lower sternal edge

Thrill +-

ASD Soft, ejection systolic

Lt upper sternal edge

Wide, fixed splitting of 2nd sound

PS Ejection systolic

Lt upper sternal edge

Ejection click

AS Ejection systolic

Rt upper sternal edge Ejection click at apex

Thrill in suprasternal notch, radiation to neck

Commonest cardiac problem a general paediatrician will see?

Innocent murmurs

Innocent murmurs• 30% of all children on routine auscultation may have

one. • ‘Still’s murmur’- commonest age group 3-7yr –

vibratory/musical in quality • ‘pulmonary flow’, ‘venous hum’, ‘peripheral

pulmonary stenosis’• Change in intensity with posture • Always systolic (except venous hum – continuous)• ASYMPTOMATIC

Investigations

• Chest X-ray – cardiac size, lung vascularity,

• ECG – chamber enlargement

• Hyperoxia test - to differentiate between cardiac and pulmonary cause of cyanosis in neonate

• Echocardiography - definitive diagnosis • Consider chromosomal analysis ( T21, 22q11)

Acyanotic

• Normal pulmonary vascularity

– PS (mild/moderate)

– AS

– Coarctation of aorta

• Pulmonary plethora

– VSD

– ASD

– PDA

– Severe LV outflow obstruction/ hypoplastic left heart

Cyanotic

Pulmonary oligaemia

– severe PS/atresia– TOF– TA– complex lesion with

PS

Pulmonary plethora

- TGA with VSD- Truncus Arteriosus- Total anomalous

pulmonary venous drainage (TAPVD)

Conduction disorders

• Heart block – maternal SLE

– complex congenital defect

• Tachy-arrhythmias– supraventricular

tachcardia

– long QT syndrome - prone to ventricular tachycardia

Other cardiac problems

• Myocardial - cardiomyopathies (genetic, metabolic), myocarditis - viral

• Endocardial - infective (bacterial) endocarditis

• Pericardial - pericarditis, pericardial effusion

Management strategies

MEDICAL

• Cardiac failure - rest, may need O2– afterload reduction - arteriolar dilators (Captopril), diuretics– Inotropes - Digitalis, Dopamine/Dobutamine– arrhythmia - treat– Supportive - nutrition, avoid fluid overload

• Antibiotic prophylaxis – all heart defects causing high velocity

turbulence, prosthetic material– NOT REQUIRED IN ASD

• Dental, surgical/endocsopic, ENT procedures

• Cyanosis -

– acute presentation in neonate - likely to be a duct dependant lesion

– KEEP DUCT OPEN WITH PGE1 INFUSION

– may need urgent surgical intervention (atrial septostomy in TGA, balloon dilatation of pulm/aortic valve, TAPVD)

• Cyanotic spells in TOF (pulmonary stenosis, large VSD, overriding aorta, RVH)– calm the baby

– knee chest position

– O2, Morphine

• Conduction disorders - permanent pacing for congenital complete heart block

• Medication for tachyrrhythmias

Repair of defect

• Interventional cardiac catheterisation –– PDA, ASD, VSD – occlusion with device placement

– PS, AS – balloon dilatation

• Definitive surgical repair• Palliative surgical repair in some complex lesions

• Long term cardiology follow-up