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Gavin Burgess R5, PEM. Paediatric Emergency cardiology. General. Review common presentations Uncommon Paediatric ECG Congenital heart disease Rhythm disturbances Long QT HOCM Rheumatic fever Carditis – myo, endo, peri. General. Innocent murmurs Kawasaki disease. Fetal circulation.
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Gavin Burgess R5, PEM Paediatric Emergency cardiology
General • Review common presentations • Uncommon • Paediatric ECG • Congenital heart disease • Rhythm disturbances • Long QT • HOCM • Rheumatic fever • Carditis – myo, endo, peri
General • Innocent murmurs • Kawasaki disease
“Normal” • Ball-park BP? • Neonate? • Older?
“Normal” • Gestational age should equal MAP • Systolic BP = 70 + (2 x age)
“Normal” ECG • Typically have shorter PR, QRS, QT • RV dominance, RAD
RVH • Causes • Tetralogy of Fallot • PS • Coarct • ASD • TAPVD • Large VSD with Pulm HT
LVH • Causes • AS • VSD • PDA • Complete AV block • Cardiomyopathy
Superior or “north west” axis • Endocardial cushion defect • 2% of congenital heart disease • Down syndrome account for 70% • Fatal due to pulm HT • Banding in infancy
Myocardial infarction • AT III • Cardiomyopathy • Congenital heart disease • CAD (ALCAPA) • Drugs (cocaine) • Homocystinuria • Hyperlipidaemia and cholesterolaemia • Kawasaki • Leukaemia • Marfans • Haemoglobinopathies • Tumours (myxoma) • Rheumatic fever • SLE
Which lesions give cyanosis? • Tetralogy of Fallot • Tricuspid atresia • Transposition of the great arteries (IDM) • Truncus arteriosus • Total anomalous pulmonary venous drainage • Hypoplastic left heart • Ebstein’s anomaly (lithium) • Pulmonary atresia/severe stenosis
Pulmonary markings • Decreased: • Pulmonary atresia/stenosis • Tetralogy • Tricuspid atresia • Ebstein’s anomaly • Increased: • TGA • TAPVD • Truncus
What’s the hyperoxia test? • ABG • Give 100% O2 • Repeat ABG after 10 min • If rises by >10%, likely pulmonary lesion
When does the ductus close? • 10-14 days after birth, it is physiologically closed
Neonatal and infant presentations to ED • What are the 4 presentations in and infants neonates? • 1) shock • 2) cyanosis • 3) cardiac failure • 4) murmur
What are the ductal-dependent lesions? • Systemic • Coarct/interrupted arch • Aortic stenosis • HLH • Pulmonary • PS/atresia • Tricuspid atresia
Shock • L ventricular outflow obstruction • Coarct • AS • HLH
Shock • Management: • ABC’s • Start prostin • CXR • ECG
What’s prostin? • Prostaglandin E1 • Rate 0.05-0.2 mcg/kg/min • Side effects? • Apnoea • Fever • Flushing • Hypotension • Prostin has an “all or nothing” action • Should work in 15min
Cyanosis • What is a tetralogy of Fallot? • RVH • Overriding aorta • VSD • RV outflow obstruction
What’s a “tet spell”? • Change in the balance of pulmonary and systemic flow • Hypoxic and cyanotic event • Decreased system vascular resistance or increased RV outflow obstruction • Increasing hypoxia
How do I treat it? • O2 • Chest-knee (why?) • Analgesia • B-blocker (why?)
Cardiac failure • History: • Fussy • Sweating • FTT • Short frequent meals • Physical: • HSM • Murmur • FTT • You will NOT see a JVP • AVM – auscultate the head
Murmurs • Features of an innocent murmur • 80% of children will have a murmur at some time in their lives • All have normal ECG and X-rays • Never diastolic
Arrhythmia • SVT • Very common • Tolerated well, occasional LOC change • Child is fussy • Newborn >220 bpm • <12y often accessory pathway
Arrhythmia • SVT treatment • In shock vs stable • Vagal stim • Adenosine • Amiodarone ,verapamil use extreme caution. Frequently develop profound hypotension and die
Arrhythmia • Long QT • History • Deafness • Single person MVC • Swimming syncope • Exercise syncope • Family history of sudden death • Seizure of unknown etiology • Recurrent syncope/lightheadedness • Sibling with SIDS • Physical • Infant with bradycardia
Arrhythmia • All first degree family members should be screened with ECG
HOCM • 2% under 2 y, 7% under 10y • Variable history • CP • Palpitations • SOB • Syncope • Sudden death • High risk if syncope • Sudden death with strenuous exercise
HOCM • Physical • S4 gallop, mid systolic murmur • Increased PVR decreases murmurs
Rheumatic fever • Who was Jones? • What where his criteria? • What do you need to make a diagnosis? • Which valve? • Then?
Rheumatic fever • What about Sydenham’s chorea? • And the rash?
Rheumatic fever • Treatment • ASA 75-100mg/kg • Prednisone 1-2mg/kg • Benzathine (Pen G) 600 000U (27kg), 1.2 million U (27kg) • Prophylaxis • Age questioned
Myocarditis • Various causes, most notably viral • Coxsackie A,B, ECHO, flu’ • Non-specific viral prodrome • Non-specifc fussiness, lethargy etc • Heart failure • IVIG may be indicated
Infective endocarditis • Rheumatic fever, congenital heart defects, catheters, IVD • S. aureus, viridans are the usual suspects • Fungi in neonates, usually in the NICU
Infective endocarditis • Major • 2 + BC, (viridans, s. bovis, HACEK, S. aureus, enterococci • Persistently + BC (1 hr between multiple, or 12h or 3h +) • + echo mass at typical sites • Intracardiac abscess • Prosthesis failure • New regurgitant murmur
Infective endocarditis • Minor • Fever (38C) • Predisposing condition/IVD • Vascular phenomena • Non-specific echo findings
Prophylaxis -1997 • High risk – amp and gent • Prosthesis • Previous IE • Complex CHD • Surgical systemic-pulmonary shunts • Medium risk - amp • Other congenital heart malformation • Acquired valve dysfunction • HOCM • MVP • Negligible risk – no Rx • Isolated secundum; repaired ASD, VSD, PDA; bypass graft; MVP (no regurge); “innocent” murmurs; KD with normal valves; RF with no valve dysfunction; pacemakers
Prophylaxis • High risk • Prosthesis • Previous IE • Transplants • Complex CHD • Dropped from the list……. • Moderate risk (PDA,VSD,primumASD,coarct,bicuspidAV) • Calcified AS,RF,HOCM,MVP
Pericarditis • Classic chest pain worse when lying flat • Radiation to L shoulder • Friction rub • Most often viral causes • Diffuse ST changes, “saddle”shaped • CXR important • Cefotaxime, ASA, prednisone, colchicine
Kawasaki disease • Etiology unkown, presumed infectious • More common in Asian and Pacific islanders • Peaks around 1-2years, 80% under 4y, 50% under 2y • Slight male preponderance • 3mo-8y is typical range
Kawasaki disease • 3 phases • Acute phase (10 days) • High fever for 5 days • 4 of • rash (ANY rash, no bullae/vesicles), • oedema of extremities/ peeling of extremities • Non-exudative bulbar conjuctivitis • Mucosal changes (cracked lips, strawberry tongue – even on HISTORY) • Cervical LN (1.5cm) • Carditis, other organs (arthritis, pyuria, gallbladder/liver, menigitis, irritable