1. Cardiac Problems in Children M Rajimwale

2. Arrhythmias Cardiac Problems in Children Congenital heart disease Myocardial/pericardial, endocardial

3. Congenital heart disease Incidence - 0.8% live births 10% in still born/ abortus < 10% chromosomal abnormality/genetic mutations 25% have extracardiac abnormality

4. 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

5. 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

6. Teratogens Teratogenic Exposure RubellaCoarct, VSD, PDA AlcoholVSD PhenytoinASD LithiumEbsteins anomaly WarfarinVSD, TOF

7. FOETAL CIRCULATION Two intracardiac communications Ventricles working in parallel

8. >95% 75% 3mm 25/3 8 100/8 25/10100/60 Left heart Right heart LA LV RA RV Aorta PA

9. VSD 30.5% ASD9.8% PDA9.7% PS6.9% Coarctation of aorta6.8% AS6.9% TOF5.8% TGA4.2% Truncus2.2% TA1.3%

10. Clinical Manifestations Cardiac failure – (Lt to Rt shunt – first few months LV outflow obstruction – few days/weeks Functional 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 – TOF TGA Tricuspid atresia TAPVD Truncus Arteriosus Pulm atresia

11. Clinical Manifestations... Incidental detection of murmur on routine examination MURMUR OFTEN ABSENT IN CYANOTIC CONGENITAL HEART DISEASE

12. 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

13. Examination General exam –growth, dysmorhism, well/unwell –colour, perfusion, pulse (including femorals), BP, post-ductal SaO2 CVS inspectionauscultation (supine and standing) palpation

14. Auscultation –heart sounds (intensity, splitting of 2 nd 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

16. Commonest cardiac problem a general paediatrician will see? Innocent murmurs

17. 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

18. 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)

19. Acyanotic Normal pulmonary vascularity –PS (mild/moderate) –AS –Coarctation of aorta Pulmonary plethora –VSD –ASD –PDA –Severe LV outflow obstruction/ hypoplastic left heart

20. 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)

21. Conduction disorders Heart block –maternal SLE –complex congenital defect Tachy-arrhythmias –supraventricular tachcardia –long QT syndrome - prone to ventricular tachycardia

22. Other cardiac problems Myocardial - cardiomyopathies (genetic, metabolic), myocarditis - viral Endocardial - infective (bacterial) endocarditis Pericardial - pericarditis, pericardial effusion

23. 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

24. Antibiotic prophylaxis –all heart defects causing high velocity turbulence, prosthetic material –NOT REQUIRED IN ASD Dental, surgical/endocsopic, ENT procedures

25. 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)

26. Cyanotic spells in TOF (pulmonary stenosis, large VSD, overriding aorta, RVH) –calm the baby –knee chest position –O2, Morphine

27. Conduction disorders - permanent pacing for congenital complete heart block Medication for tachyrrhythmias

28. 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