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Cardiac Problems in Children M Rajimwale Cardiac Problems in Children Congenital heart disease Myocardial/pericardial, endocardial Arrhythmias 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) Edwards (Tris.18) Patau (Tris.13) Turner (XO) de-George (22q11deletion) Williams (7q del) AVSD,VSD,TOF VSD, various defects VSD, various defects Coarct.,AS Truncus,IAA,TOF Supravalvar AS More associations Maternal Disease Diabetes Mellitus – TGA,VSD, HOCM SLE - Heart block Associations Oesophageal Atresia Anorectal malformationDiag. Hernia Exomphalos Pierre Robin - VSD, TOF Any Any Any VSD Teratogens Teratogenic Exposure Rubella Alcohol Phenytoin Lithium Warfarin Coarct, VSD, PDA VSD ASD Ebsteins anomaly VSD, TOF FOETAL CIRCULATION Two intracardiac communications Ventricles working in parallel Right heart Left heart 75% LA RA 75% 3mm RV 25/3 25/10 >95% >95% 8 LV 75% 75% PA >95% >95% Aorta 100/8 100/60 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 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 cyanosis5 Ts – TOF TGA Tricuspid atresia TAPVD Truncus Arteriosus Pulm atresia Clinical Manifestations... • Incidental detection of murmur on routine examination MURMUR OFTEN ABSENT IN CYANOTIC CONGENITAL HEART DISEASE Clinical manifestations ... • Palpitation, dizziness, fainting - arrhythmia, long QT syndrome • Infective endocarditis - rare < 2 years • Chest pain - rare, ischaemia - aortic stenosis, anomalous origin of coronary artery pericarditis • Sudden death - rare, HOCM, severe AS, long QT Examination • General exam – growth, dysmorhism, well/unwell – colour, perfusion, pulse (including femorals) , BP, post-ductal SaO2 • CVS inspection palpation auscultation (supine and standing) • 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 Thrill +sternal edge ASD Soft, ejection systolic PS Ejection systolic Lt upper Wide, fixed sternal edge splitting of 2nd sound Lt upper Ejection click sternal edge AS Rt upper Thrill in sternal edge suprasternal notch, Ejection radiation to neck click at apex Ejection systolic 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) 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