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Paediatric Cardiology NUCCS AP Revision Series Session 1 Ellie Pritchard – AP2 Medical StudentOutline 1. Fetal circulation 2. Transition to postnatal circulation 3. Innocent murmurs 4. PDA, ASD and VSD 5. Cyanotic Heart Disease 1. Tetralogy of Fallot 2. Transposition of the GreatArteries 3. Tricuspid atresia 6. 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 venosumInnocent Murmurs Aka flow murmurs → caused by 5 S’s fast blood flow in systole Soft Short Very common in children Systolic Situation dependent → often get May not require further quieter on standing, or only investigation unless concerning appears when child is unwell features Small → defined area of the chestPDA, ASD & VSD A quick look… (covered in more detail in session 2)Atrial Septal Defect Ventricular Septal (ASD) Defect (VSD)Patent Ductus Arteriosus (PDA)Cyanotic Heart DiseaseCyanotic vs Acyanotic Heart Disease • If a heart defect lowers blood oxygen → cyanotic • If a heart defect doesn’t affect oxygenation → acyanoticCyanotic Heart Disease • Cyanosis occurs when deoxygenated blood enters the systemic circulation • Causes blue-purple discolouration of the skin • Occurs when blood bypasses the pulmonary circulation and lungs • Right-to-left shunt • Deoxygenated blood from the right side of the heart shunts straight to the left side and into the body (without passing through the lungs)Cyanotic heart disease 5 T’s - Tetralogy of Fallot - Transposition of the great arteries - Tricuspid atresia - (Truncus arteriosus) - (Total anomalous pulmonary venous return)T etralogy of Fallot 4 co-existing heart defects: 1. VSD 2. Overriding aorta 3. Pulmonary valve stenosis 4. Right ventricular hypertrophy Right-to-left cardiac shunt Most often diagnosed in the first few weeks of lifeOverriding Aorta Pulmonary Valve Stenosis • Entrance to aorta further to right • Encourages blood to flow than usual, above VSD through the VSD into the aorta • Causes deoxygenated blood to rather than into pulmonary enter the aorta vessels RV Hypertrophy VSD • Increased strain on RV as it • Hole in septum between the pumps against LV and ventricles pulmonary stenosisT etralogy of Fallot Risk Factors: Presentation: - Maternal age >40 years - Usually on antenatal scans - Alcohol consumption in - If symptomatic: pregnancy - SoB - Maternal diabetes - Cyanosis - Genetic conditions - Poor feedinging - Congenital rubella syndrome - Poor weight gain - Tetspells - Ejection systolic murmur → loudest in pulmonary areaT etralogy of Fallot Investigations: Management: - Echocardiogram - Prostaglandin infusion → to - Doppler to show direction of maintain ductus arteriosus blood flow - Blalock-Thomas-Taussig shunt → - CXR temporary fix, connection between - “boot-shaped” heart a systemic artery and pulmonary artery - Mxen surgical repair for definitiveT et Spells Management of tet spells: - Older children may squat • Intermittent cyanotic episodes - Younger children placed knee-to- • Due to temporary worsening of chest right-to-left shunt - Both of these methods increase • Pulmonary resistance increases / systemic vascular resistance systemic resistance decreases • Waking, physical exertion or crying - O2 • Child becomes irritable, cyanotic - IV morphine and SoB - IV fluids • Can lead to reduced consciousness, - IV beta blockers seizures and death - Phenylephrine infusionT ransposition of the Great Arteries • Swapped attachments of the aorta and pulmonary trunk • RV pumps blood into aorta • vesselss blood into pulmonary • This creates two separate pulmonary → blood doesn’t mixT ransposition of the Great Arteries • Fetal development normal → gas exchange occurs in the placenta Management: • Life-threatening immediately after birth • Prostaglandin E2 infusion to maintain PDA • Newborn will be cyanotic • Balloon septostomy → balloon between atria to allow mixing of blood • Depends on a shunt connecting systemic • Arterial switch operation → definitive and pulmonary circulation • PDA, ASD or VSD • Often diagnosed on antenatal scans • after birth present as cyanosis immediately • Shunt can compensate by allowing mixed blood initiallyPDAT ricuspid Atresia • Complete absence of the tricuspid valve → hypoplastic RV • There must be an ASD to fill the LA and LV with blood • There must also be either a VSD or PDA to pump blood into the pulmonary arteryT ricuspid Atresia • Majority diagnosed antenatally Management: • Presents with cyanosis and - Prostaglandins to maintain PDA murmur after birth - Definitive management Investigations: surgically • Echocardiogram • ECG → left axis deviationPractice QuestionsWhat 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 are the 5 S’s of an innocent murmur?What are the 5 S’s of an innocent murmur? Soft Short Systolic Situation dependent → often get quieter on standing, or only appears when child is unwell Small → defined area of the chestQuestion 1 and pyrexia. On examination of the precordium a murmur is heard.rache Which one of the following characteristics is not consistent with an innocent murmur? A) Short buzzing murmur in the aortic area B) Soft-blowing murmur in the pulmonary area C) Varies with posture D) Diastolic murmur E) Continuous blowing noise just below the claviclesQuestion 1 and pyrexia. On examination of the precordium a murmur is heard.rache Which one of the following characteristics is not consistent with an innocent murmur? A) Short buzzing murmur in the aortic area B) Soft-blowing murmur in the pulmonary area C) Varies with posture D) Diastolic murmur E) Continuous blowing noise just below the claviclesQuestion 2 wheeze. On examination a systolic murmur is heard in the secondgh and intercostal space lateral to the left sternal edge. It has an intensity of 1/6 and is not audible when she lies flat. Which of the following is the most likely diagnosis? A) Coarctation of the aorta B) Ventricular septal defect C) Innocent murmur D) Atrial septal defect E) Pulmonary stenosisQuestion 2 wheeze. On examination a systolic murmur is heard in the secondgh and intercostal space lateral to the left sternal edge. It has an intensity of 1/6 and is not audible when she lies flat. Which of the following is the most likely diagnosis? A) Coarctation of the aorta B) Ventricular septal defect C) Innocent murmur D) Atrial septal defect E) Pulmonary stenosisQuestion 3 A neonate begins to turn blue and becomes tachypnoeic 5 minutes after birth.They are administered 100% oxygen for 15minutes and an arterial blood gas is performed. On auscultation, the neonate has no murmur but aloud single S2. On palpation,there is a prominent ventricular pulse. What is the most likely diagnosis? A) Coarctation of the aorta B) Pulmonary valve stenosis C) Tetralogy of Fallot D) Transposition of the great arteries E) Tricuspid atresiaQuestion 3 A neonate begins to turn blue and becomes tachypnoeic 5 minutes after birth.They are administered 100% oxygen for 15minutes and an arterial blood gas is performed. On auscultation, the neonate has no murmur but aloud single S2. On palpation,there is a prominent ventricular pulse. What is the most likely diagnosis? A) Coarctation of the aorta B) Pulmonary valve stenosis C) Tetralogy of Fallot D) Transposition of the great arteries E) Tricuspid atresiaQuestion 4 A 17-hour-old baby on the maternity ward has become cyanotic. This heard on auscultation. You suspect the child has transposition of the greate arteries. What is the initialmanagement for this child? A) Ibuprofen B) Indomethacin C) Intubation and ventilation D) Surgery E) Prostaglandin E1Question 4 A 17-hour-old baby on the maternity ward has become cyanotic. This heard on auscultation. You suspect the child has transposition of the greate arteries. What is the initialmanagement for this child? A) Ibuprofen B) Indomethacin C) Intubation and ventilation D) Surgery E) Prostaglandin E1Thank you! Same time nextTuesday for Session 2 ☺