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Paediatric Cardiology NUCCS AP Revision Series Session 2 Ellie Pritchard – AP2 Medical StudentOutline 1. Fetal circulation (recap) 2. Acyanotic heart disease 1. Atrial Septal Defect 2. Ventricular Septal Defect 3. Patent Ductus Arteriosus 4. Coarctation of the aorta 3. Acquired heart conditions 1. Kawasaki disease 2. Rheumatic Heart Disease 4. Practice questionsFetal CirculationFetal Circulation • In a fetus, the lungs are non-functional • The placenta is responsible for oxygenation of fetal blood and removal of carbon dioxide • Fetal blood travels to the placenta via the two umbilical arteries • Originate from the internal iliac arteries • Fetal blood travels from placenta back to fetus via the one umbilical veinFetal shunts 3 fetal shunts 1. Ductus venosus 2. Foramen ovale 3. Ductus arteriosus Ductus venosus: connects umbilical vein to inferior vena cava bypasses the liverFetal shunts Foramen ovale: connects the right atrium with the left atrium bypasses the right ventricle and lungs Ductus arteriosus: connects pulmonary artery and aorta bypasses the lungsT ransition to postnatal circulation • First breaths expand the alveoli → decreases pulmonary vascular resistance → fall in pressure in RA and RV • LA pressure > RA pressure • Atrial septum becomes squashed → functional closure of the foramen ovale • Foramen ovale → fossa ovalis • Takes a few weeks to sealT ransition to postnatal circulation • Prostaglandins keep ductus arteriosus open • Increased blood oxygenation causes a drop in circulating prostaglandins • Closes ductus arteriosus → ligamentum arteriosum • When blood stops circulating through the umbilical vein, the ductus venosus stops functioning • Ductus venosus → ligamentum venosumAcyanotic Heart DiseaseAtrial Septal Defect • Hole in septumbetween the two atria • Initially a left-to-right shunt due to higher pressure on left side of heart • Eventually this causes pulmonary hypertension and right sided heart failure • Pulmonary pressure > systemic pressure • Becomes a right-to-left shunt → cyanosis → Eisenmenger syndromeAtrial Septal Defect Types Complications Most to least common - Stroke (VTE) 1. Patent foramen ovale - Atrial fibrillation / atrial flutter 2. Ostium secundum - Pulmonary HTN and right sided 3. Ostium primum (tends to lead HF to AVSD) - Eisenmenger syndrome DVT → stroke; think lifelong asymptomatic ASD?Atrial Septal Defect • Mid-systolic, crescendo- Presentation decrescendo murmur - Antenatal scans • Loudest at upper leftsternal border - Newborn examination • Fixed split second heart sound - Asymptomatic in childhood (i.e. doesn’t change with - May present in adulthood with inspiration or expiration) dyspnoea, heart failure or stroke - If symptoms in childhood: SoB, feeding difficulty, poor weight gain, LRTIsAtrial Septal Defect Investigations Management - Echocardiogram - Referral to paediatric cardiologist - Active monitoring → small defects may close by themselves - Percutaneous transvenous catheter closure - Open heart surgeryVentricular Septal Defect • Hole in the septum between right and left ventricles • Can be associated with Down’s syndrome or Turner’s syndrome • Similar mechanism to ASD: initially a left-to-right shunt, causes pulmonary hypertension, pulmonary pressure > systemic pressure, becomes a right-to-left shunt → cyanosis, Eisenmenger syndromeVentricular Septal Defect Presentation Examination Findings - Often initially asymptomatic → - Pansystolic murmur can present as late as adulthood - Loudest over left lower sternal - May be detected during border, 3 and 4 ICS antenatal scans or newborn - May be a systolic thrill on baby check palpation - If symptomatic: poor feeding, dyspnoea, failure to thrive, tachypnoeaVentricular Septal Defect Investigations Management - Echocardiogram - Referral to paediatric cardiologist - Active monitoring – small defects may close themselves VSDs increase risk of infective endocarditis - Percutaneous transvenous catheter closure - Open heart surgeryPatent Ductus Arteriosus • Ductus arteriosus connects the pulmonary artery & aorta → allows blood to bypass the lungs • Usually stops functioning within 3 days and is completely closed within the first three weeks • PDA occurs when this fails to close • Prostaglandin E2 keeps the ductus arteriosus open during pregnancy • PGE2 produced by the placenta → falls after birth → ductus arteriosus closes • Left to right shuntPatent Ductus Arteriosus Presentation Management - May not have any abnormal HS if - Echocardiogram to confirm small diagnosis - Larger PDAs → continuous - Medical closure: indomethacin, crescendo-decrescendo ibuprofen, paracetamol ‘machinery’ murmur - Inhibit prostaglandin synthesis - Loudest below the clavicle Symptoms - Endovascular procedures - SoB - Open surgery - Difficulty feeding - Poor weight gain - LRTIsCoarctation of the Aorta • Narrow point in the aortic arch (usually near the ductus arteriosus) • Often associated with Turner’s syndrome • Increased pressure proximal to narrowing, decreased pressure distal to narrowingCoarctation of the aorta Presentation Other signs: - Weak femoral pulses - Raised resp rate - Increased BPin arms, decreased BP - Increased WoB in legs - Systolic murmur belowleft clavicle - Poor feeding & left scapula - Heart failure - Shock - LV heave Management - Underdeveloped left arm - Mild cases might not need intervention, more severe cases may - Underdeveloped legs need surgery in early days of lifeAcquired Heart DiseaseKawasaki Disease • Systemic, medium sized vasculitis • Typically children <5 yrs • More common in Asian children, particularly Japanese & Korean children • More common in boysKawasaki Disease Presentation - Strawberry Tongue → red - Persistent high fever >39 C for tongue with large papillae more than 5 days - Cracked lips - Children will be unhappy & - Cervical lymphadenopathy unwell - Bilateral conjunctivitis - Widespread erythematous maculopapular rash & desquamation on palms & solesKawasaki DiseaseKawasaki Disease Investigations Management - FBC, LFT, inflammatory markers, - High dose aspirin → reduce urinalysis, echocardiogram thrombosis risk Disease course - aneurysmsduce risk of coronary artery - Acute → most unwell; fever, rash & lymphadenopathy, 1-2 weeks - Subacute → acute symptoms settle, - + follow up echocardiograms coronary artery aneurysms, risk of - symptoms settle, coronary aneurysms may regressRheumatic Fever - Autoimmune condition triggered by Group A strep - Typically streptococcus pyogenes (tonsilitis) - Multi-system disorder → affects heart, joints, skin & nervous system - Rare in UK due to early treatment with Abx - Type II hypersensitivity reaction - 2-4 weeks postinfectionRheumatic Fever Presentation Heart involvement: - 2-4 weeks post infection e.g. - Carditis → inflammation tonsilitis throughout the heart incl. - Fever pericarditis, myocarditis & - Joint pain → migratory arthritis endocarditis - Can cause a murmur from mitral - Rash valve disease - SoBRheumatic Fever Investigations Management - Throat swab for bacteria - Treatment of initial strep infection - ASO antibody titres - penicillin V for 10 daysby strep → - Antibodies against strep infection - Echo, ECG, CXR - Specialist management Jones Criteria - Aspirin - Steroids - Prophylactic Abx continued into Complications adulthood - Recurrence of rheumatic fever - Valvular heart disease → mitral stenosis - Chronic heart failurePractice Questions!What kind of heart defect is this?What kind of heart defect is this? VSDWhat kind of heart defect is this?What kind of heart defect is this? TGAWhat kind of heart defect is this?What kind of heart defect is this? ASDWhat kind of heart defect is this?What kind of heart defect is this? PDAWhat kind of heart defect is this?What kind of heart defect is this? Coarctation of AortaQuestion 1 A 2-year-old boy is brought into the emergency department followinga 1 week history of fever, lethargy and irritability.The symptoms came on suddenly over a matter of hours and have not dissipated despite the GP's recommendation of anti-pyretics. He has had a reduced appetite and diarrhoea during this time. Earlier this morning a widespread red rash appeared on his body.. On basic observations the child appears toxic looking,is tachycardic and has a temperature of 39.2ºC.Examination reveals a widespread maculopapular rash, left-sided cervical lymph node enlargement and a swollen,erythematous tongue. Given the likely diagnosis what is the most important investigation in this child? A) Chest X ray B) Abdominal Ultrasound C) Electrocardiogram D) Lumbar puncture E) EchocardiogramQuestion 1 A 2-year-old boy is brought into the emergency department followinga 1-week history of fever, lethargy and irritability.The symptoms came on suddenly over a matter of hours and have not dissipated despite the GP's recommendation of anti-pyretics. He has had a reduced appetite and diarrhoea during this time. Earlier this morning a widespread red rash appeared on his body.. On basic observations the child appears toxic looking,is tachycardic and has a temperature of 39.2ºC.Examination reveals a widespread maculopapular rash, left-sided cervical lymph node enlargement and a swollen,erythematous tongue. Given the likely diagnosis what is the most important investigation in this child? A) Chest X ray B) Abdominal Ultrasound C) Electrocardiogram D) Lumbar puncture E) EchocardiogramQuestion 2 history of fever. She has no previous medical conditions, has no known allergies, is developingnormally and her immunisations are up-to-date. Her vital signs are asfollows: Resp. rate: 20 A) Chickenpox SpO 2 96% B) Kawasaki disease Heart rate: 90 C) Measles Cap. refill time: 1 sec D) Roseola infantum E) Scarlet fever BP: 110/85 mmHg AVPU: Alert Temp.: 38.8ºC in texture. Hertongue isred, swollen and covered with white papillae and her tonsils are erythematous. Other system examinations are unremarkable. Based on these findings, what is the most likely diagnosis?Question 2 history of fever. She has no previous medical conditions, has no known allergies, is developingnormally and her immunisations are up-to-date. Her vital signs are asfollows: Resp. rate: 20 A) Chickenpox SpO 2 96% B) Kawasaki disease Heart rate: 90 C) Measles Cap. refill time: 1 sec D) Roseola infantum E) Scarlet fever BP: 110/85 mmHg AVPU: Alert Temp.: 38.8ºC in texture. Hertongue isred, swollen and covered with white papillae and her tonsils are erythematous. Other system examinations are unremarkable. Based on these findings, what is the most likely diagnosis?Question 3 A 40-year-old woman who is being treated for refractory hypertension undergoes a coronary angiogram after developingnon-specific chest pains. The cardiologist takes a number of measurements duringthe procedure: The blood pressure in her left arm takingduringthe procedure was 188/74 mmHg.What is the most likely underlying diagnosis? A) Left subclavian artery stenosis B) Renal artery stenosis C) Coarctation of the aorta D) Aortic stenosis E) Results consistent with essential hypertensionQuestion 3 A 40-year-old woman who is being treated for refractory hypertension undergoes a coronary angiogram after developingnon-specific chest pains. The cardiologist takes a number of measurements duringthe procedure: The blood pressure in her left arm takingduringthe procedure was 188/74 mmHg.What is the most likely underlying diagnosis? A) Left subclavian artery stenosis B) Renal artery stenosis C) Coarctation of the aorta D) Aortic stenosis E) Results consistent with essential hypertension