Paediatric Cardiology Abhishek Oswal

Syllabus Age related changes in heart rate and blood pressure: know approximate values for infants and toddlers Innocent murmurs: definition and how to distinguish from pathological murmurs Heart failure: symptoms and signs in infants and children, possible causes Common forms of congenital heart disease: natural history and management of  Acyanotic: ASD, VSD (small, medium and large), PDA, coarctation Cyanotic: Fallot's, transposition Infective endocarditis: which children are at risk, preventative measures

Vital signs

Innocent Murmurs Characteristics Examples Soft Systolic aSymptomatic left Sternal edge No thrills No radiation Otherwise normal heart sounds, no S3/S4 Examples Still’s murmur – musical, vibratory Venous hum – through cardiac cycle Carotid flow murmur – in the neck

Heart Failure Varied presentation Dependent on age Clinical signs: Newborn – cyanosis, circulatory collapse, may need NICU Infant – FTT, sweating, SOB (particularly on feeding) Child – recurrent chest infections, palpitations, syncope, exercise limitation Clinical signs: Tachypnoea, Tachycardia Cool peripheries Cardiomegaly Heart murmur, gallop rhythm Hepatomegaly

Causes of HF Newborn – obstructed outflow tract e.g. Preductal CoA Critical aortic stenosis Infant – high pulmonary flow e.g. VSD Large PDA Older child – right or left or congestive cardiac failure e.g. Eisenmenger Cardiomyopathy

Normal (adult) circulation From body From lungs RA RV LA LV To lungs To body

Defects Acyanotic Cyanotic Atrial septal defect (ASD) Ventricular septal defect (VSD) Persistent ductus arteriosus (PDA) Coarctation of the aorta (CoA) Cyanotic Transposition of the great arteries (TGA) Tetralogy of Fallot (ToF)

Things to know for each one Pink or blue? Presentation Murmur Other clinical signs ECG CXR Management

Atrial septal defect From body From lungs LA LV RA RV To lungs To body

Atrial septal defect Pink Presentation (often in adulthood): asymptomatic, syncope palpitations, decreased exercise tolerance 20% of the population Murmur Soft systolic murmur ULSE + (pan systolic murmur if AVSD affecting AV valves) Fixed split S2 ECG Partial RBBB (RSR’ in V1) in 90% of ASD RAD/RVH Right atrial enlargement - Tall P waves CXR Increased pulmonary vascular markings Rx Conservative if asymptomatic Closure age 3-5 (before starting school) Cath lab device closure for 80% Some may require surgical closure if associated with other defects or too large for device

Ventricular Septal Defect From body From lungs RA RV LA LV To lungs To body

Ventricular septal defect Pink Presentation: Asymptomatic adult  heart failure in an infant Murmur Small: blowing pan systolic murmur LLSE, may be associated with a thrill Large: ?above + rumbling mid diastolic murmur, loud P2, RV heave ECG Large: biventricular hypertrophy by age 2/12, peaked P waves (LA hypertrophy) CXR Large: increased pulmonary vascular markings, cardiomegaly, ?pulmonary oedema Rx Conservative if asymptomatic. Many close spontaneously before 2 years of age Large: diuretics, captopril, calories so ready for surgical closure at 3-5/12

(Eisenmenger syndrome) From body From lungs RA RV LA LV To lungs To body

(Eisenmenger syndrome) Irreversible conversion of a LR shunt disease into a RL shunt, cyanotic disease due to pulmonary hypertension and RV hypertrophy Blue Presentation: cyanosed teen/early 20s, SOB, reduced exercise tolerance, fatigue, HF, erythrocytosis CV exam Clubbing RV heave, Raised JVP PSM of VSD disappears as the shunt reverses. May have early diastolic murmur of pulmonary regurgitation ECG Right heart hypertrophy (right axis deviation) CXR Dilated pulmonary arteries, normal pulmonary vascular markings, normal heart size Rx Supportive therapy, night time O2 Pulmonary vasodilators e.g. prostaglandin analogues, endothelin antagonists, sildenafil Only definitive is heart lung transplant

Persistent ductus arteriosus From body From lungs RA RV LA LV To lungs To body

Persistent ductus arteriosus Pink Presentation: asymptomatic, recurrent LRTI, FTT (rarely HF) CV exam Continuous machinery murmur left infraclavicular region Bounding pulses ECG Normal, LVH CXR Normal, increased pulmonary markings Rx Conservative – wait and let it close Medical – NSAIDs e.g. indomethacin, aspirin Small – cath lab closure with coil/plug at 1 year Large – surgical ligation age 1-3mo

Coarctation of the aorta From body From lungs From body From lungs RA RV LA LV RA RV LA LV To lungs To lungs To body To body

Coarctation of the aorta (post ductal) Pink Adult type (rare) Presentation: asymptomatic  progressive hypertension, resistant to drugs CV exam Ejection systolic murmur, upper sternal edge Continuous rumbling murmur on auscultation of the back Radiofemoral delay Upper limb hypertension, lower limb hypotension (difference >20mmHg) ECG LVH CXR rib notching 3 sign – visible notch of coarctation in descending aorta Rx Cath lab angioplasty/stenting age 3-5 if detected early and no HF as reduces long term risk of HTN Surgical repair in adulthood if detected late

Coarctation of the aorta (pre ductal) Emergency! Blue  grey Presentation May be detected antenatally Collapsed, cyanosed, shocked within the first 2 weeks of life Absent femoral pulses O2 sat of right arm >> left arm >> feet Murmur None ECG + CXR: likely normal, ?cardiomegaly + HF Rx Admit to NICU for ?ventilation, PGE1 infusion, transfer to cardiac centre Surgery within 24hr to resect coarcted segment

Any questions so far?

Transposition of the great arteries From body From lungs RA RV LA LV To body To lungs

Transposition of the great arteries Emergency! Blue Presentation Should be diagnosed antenatally Collapsed, shocked, very blue Murmur None Single loud S2 ECG Normal CXR Egg on side Increased pulmonary vascular markings Rx Antibiotics + PGE2 infusion Catheter atrial septostomy Corrective surgery at 1-2/52

Tetralogy of Fallot From body From lungs RA RV LA LV From body To lungs To body From lungs RA RV LA LV To lungs To body

Tetralogy of Fallot Malformation of the outflow tract septum leading to VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy Blue Presentation Antenatal detection Severe cyanosis at birth Older children with reduced exercise tolerance, squatting on exercise Tet (hypercyanotic) spells

Tetralogy of Fallot Murmur ECG CXR Rx Harsh ejection systolic murmur ULSE ECG Normal at birth RVH when older CXR Small, boot shaped heart Pulmonary bay (reduced flow through pulmonary artery) Reduced pulmonary vasculature Rx Surgical (BT) shunt in neonate if severely cyanosed Aim for elective repair at 6-9/12 Tet spells – if >15min need: intubation + ventilation, fluids, IV pain relief, beta blocker

Summary flow diagram Cyanotic? Yes Murmur? ToF No Femoral pulses? TGA CoA (pre-ductal) PSM LLSE (± thrill) VSD ESM ULSE + fixed split S2 ASD Continuous machinery PDA (UL vs LL BP) ESM  back CoA (post-ductal)

Syndromic associations Down’s VSD/AVSD (30%) Turner’s Bicuspid aortic valve, Coarctation Fragile X Mitral valve prolapse, mitral regurgitation, aortic regurgitation Williams’ Supravalvular aortic/pulmonary stenosis

Infective Endocarditis At risk? Any congenital heart defects, particularly CoA, VSD, PDA Any surgical implants, coils, devices, replacement valves Consider in anyone with a persistent fever/ESR Preventative measures Good dental hygiene Avoid piercings/tattoos/IVDU No need for prophylactic Abx in dental/any other surgical procedures

MCQ 1 An uncomplicated VSD in a 5-year-old boy may be associated with which one of the following? A. A collapsing pulse B. Wide and fixed splitting of the second heart sound C. Clubbing of the fingers D. A pansystolic murmur of grade 4/6 in intensity E. Splenomegaly

MCQ 2 A four year old child is found to have a continuous machinery murmur heard loudest below the left clavicle. He is underweight for age but otherwise well. Considering the likely diagnosis, which of the following would you recommend for this patient? A. Recommend early operative closure B. Review the child constantly, expecting spontaneous closure within the next five years C. Recommend prophylactic penicillin until operation is performed D. Delay operation until the child has reached its expected weight for age E. Explain to the parents that this is of little significance and can be ignored

MCQ 3 A previously well 3-day-old becomes unresponsive and dusky on the neonatal unit. A CXR shows an “egg on side” appearance of the heart. Which of the following 3 are correct? A. Femoral pulses may be palpable B. ECG will show RV hypertrophy C. There is a loud, single second heart sound D. There is a ejection systolic murmur at the ULSE E. Give an immediate prostaglandin infusion

Any questions?

References RCH, Melbourne heart defects: http://www.rch.org.au/cardiology/heart_defects/ Lissauer’s Illustrated textbook of Paediatrics Beattie, Champion. Essential revision notes in Paediatrics for the MRCPCH Medscape