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Paediatric Cardiology Dr Sweatha Ananthalingam (FY1) TABLE OF CONTENTS 1 2 3 Case Fetal Circulation Congenital Cardiac diseases 4 5 6 Acyanotic Heart Cyanotic Heart Practice Diseases Diseases Questions Case 1 A 5-week-old female infant was referred to hospital because ofwheezing, poor feeding and poor weight gain duringthe previous 2 weeks. What else do you want to know? Case 1 Before this she was well in herself. Her routine neonatal examination post birth had been normal. Examination: • She was tachypnoeic (50–60 breaths/min) • There was some sternal and intercostal recession • There were scattered wheezes • The pulses were normal • Pansystolic murmur at the lowerleft sternal edge. • The liver was enlarged, palpable at two finger breadths below Case 1 What are the main investigations that need doing? 1. ECG 2. CXR 3. EchocardiogramFetal Circulation ➢ Lungs are not functional in fetuses. The placenta is where fetal blood gets oxygenated. ➢ Fetal shunts allow blood to bypass the lung & liver. ➢ At birth, the alveoli expand, decreasing the pulmonary vascular resistance -> F.O closure ➢ Decreasing prostaglandins, causing the closure of DA Congenital Cardiac Diseases • Heart disease in children is primarily congenital • 8 per 1000 live born infants have significant cardiac malformations, 30% of which require intervention in the 1st year of life. • 1 in 10 still born infants have a cardiac anomaly • The nine most common anomalies account for 80% of all lesions What are some causes/risk factors for Congenital cardiac abnormalities? Maternal Disorders Rubella Diabetes SLE Maternal Drugs Risk factors Chromosomal Abnormalities Fetal Alcohol Syndrome Down syndrome Warfarin Therapy Edward syndrome Patau syndrome Noonans Turner syndrome HOW DO CCD’S PRESENT?• Antenatal Cardiac ultrasound – between 18-20 weeks • Heart murmur • Heart failure – (Usually from left to right shunt) • Shock • Cyanosis – when persistent, nearly always a sign of structural heart disease What next? – ECG, CXR, Echocardiogarm Murmurs - Commonest presentation of CCD - Most are ‘Innocent Murmurs’ – 30% of children - Often heard during Febrile illness (or) Anemia -> examine child after illness! How do you differentiate between Innocent vs Pathological Murmurs? 5s ‘InnoSent’ murmur = Soft, Systolic, aSymptomatic, left Sternal edge + No added heart sounds, thrills,radiation Murmurs Refer if : Murmur louder than 2/6 Diastolic murmurs Louder on standing Other symptoms, such as failure to thrive, feeding difficulty, cyanosis or shortness of breath Types of CCD? CHD Acyanotic Cyanotic Shock – hypotension, peripheral vasoconstriction, tachycardia, Left to Right Outflow tachypnoeaand/orapnoea, hypoxia, Right to Left Heart Failure –Shunt Poor feeding, obstruction ina obstruction ina Shunt Common Mixing Recurrent chest infections, well child metabolicasick neonate disturbance (Blue) (Breathless) (lethargy, irritability), Tachypnoea, Tachycardia, Hepatomegaly, Cool peripheries gastrointestinal disturbance, oliguria. Ventricular Atrial Septal Patent Ductus Pulmonary Coarctation of Tetralogy of Transposition of Septal Defect Defect Arteriosus stenosis Aortic stenosis the Aorta Fallot the Great Eisenmenger’s Arteries Syndrome Often Asymptomatic. If Often asymptomatic Presents sick with heart Presents with cyanosis large or longstanding, with exertional SOB, failure and shock in the (blue, oxygen saturations can present with signs fatigue, dizziness neonatal period ≤94%, or collapsed), of heart failure (VSD).Patent Ductas Arteriosus • Left to Right Shunt-> (Initially Right sided hypertrophy + pulmonary hypertension) • Often Asymptomatic and closes spontaneously. • Continuous crescendo-decrescendo “machinery” murmur, heard lou below the clavicle • Can present with SOB, Feeding difficulties, Reduced weight gain, repeated Chest Infections. Arrythmias-> mid 40s onward • Prostoglandin (from placenta) keeps PDA open • Meds to inhibit prostaglandin : Indomethacin, Ibuprofen • May require surgery (Endovascular/open) ASD - 2 types – septal secundum(80%) or septal primum defect(AVSD) - Soft Ejection systolic best heard across upper left sternal edge - Maybe Asymptomatic in childhood and present in adulthood with dyspnoea, heart-failure or stroke - Childhood symptoms ->SOB, Difficulty feeding, Poor weight gain, LRTI - Link between migraine with aura and patent foramen ovale. - Management : Active monitoring, Percutaneous transvenous catheter closure (via the femoral vein); Open-heart surgery - Complications : Stroke(due to VTE), AF, RHF, Eisenmenger’s syndromeVSD - 30% of all CHD - Usually asymptomatic; - Large VSD can present with HF and breathlessness after 1 week of age and recurrent chest infections. (Needs diuretics) - Pansystolic murmur at lower left sternal edge - Association with Trisomy 21 and 45XO - Often closes spontaneously, but if large surgery between 3-6 months. - Increased risk of Infective Endocarditis - Abx prophylaxis with surgery for IE CHD Acyanotic Cyanotic Left to Right Outflow Outflow Right to Left Shunt obstruction ina obstruction ina Shunt Common Mixing well child sick neonate (Blue) (Breathless) Transposition of Ventricular Atrial Septal Patent Ductus Pulmonary Aortic stenosis Coarctation of Tetralogy of the Great Septal Defect Defect Arteriosus stenosis the Aorta Fallot Arteries Eisenmenger’s Syndrome Eisenmenger’s syndrome - Develops within 1-2 years in large shunts and adulthood smaller shunts. - Develops quicker in pregnancy(mortality– 50%). Maternal history of CCD -> Needs ECHO - ‘Cyanosis’ – blue discoloration of the skin due to low o2 levels. Eisenmenger’s syndrome - Cyanosis, Clubbing, Dyspnea, Plethoric(Ruddy) Complexion due to polycythemia - Examination : Raised JVP, Peripheral Oedma (RHF), R Ventricular Heave, Loud S2 - Ideally prevention with early intervention - Once syndrome developed, not medically reversible - Hear—Lung transplant is definitive but has high mortality Medical management: •Oxygen -> does not affect overall outcomes •Treatment of pulmonary hypertension(sildenafil) •Treatment of arrhythmias •Treatment of polycythaemia with venesection •Prevention and treatment of thrombosis with anticoagulation •Prevention of infective endocarditis using prophylactic antibiotics CHD Acyanotic Cyanotic Left to Right Outflow Outflow Right to Left Shunt obstruction ina obstruction ina Shunt Common Mixing well child sick neonate (Blue) (Breathless) Transposition of Ventricular Atrial Septal Patent Ductus Pulmonary Aortic stenosis Coarctation of Tetralogy of the Great Septal Defect Defect Arteriosus stenosis the Aorta Fallot Arteries Eisenmenger’s Syndrome Neonatal Cyanosis - Presents with Cyanosis and reduced o2 saturations, usually in 1 week of life - Nitrogen washout test : The infant placed in 100% oxygen for 10 minutes. If the right radial arterial partial pressure of oxygen (PaO2) <15 kPa ‘cyanotic’. *> 20 kPa not cyanotic - Blood gas analysis must be performed as oxygen saturations are not reliable enough in this range of values. Management of the cyanosed neonate: -Stabilize the airway, breathing, and circulation (ABC) -Start prostaglandin infusion -Most infants with cyanosis in the first few days of life are ‘duct dependent’. -Maintenance of ductal patency is the key to early survival of these children -Observe for side-effects of prostaglandin – apnoea, jitteriness and seizures, flushing, vasodilatation and hypotension. *lung disease and persistent pulmonary hypertension of the newborn to be excludedTeratology of Fallot - pulmonary stenosis depends on the - Right-to-left shunt - Associated with ‘Rubella’ - Maternal age, alcohol, diabetes are also risks - Echo(+doppler studies for direction of flow) - Usually picked up antenatally - Ejection systolic murmur loudest in the pulmonary area Teratology of Fallot Symptoms: • Cyanosis • Clubbing • Poor feeding • Poor weight gain *Tet-spells - Intermittent symptomatic periods due to physical exertion, crying, waking( Increased PVR or decreased SVR) - Severe spells can lead to reduced consciousness, seizures and potentially death - Squatting(older child) and knees to chest(younger child) helps Teratology of Fallot Management: - In neonates -> prostaglandin infusion to maintain the ductus arteriosus (allows blood to flow from the aorta back to the pulmonary arteries) - Open heart surgery is the definitive treatment(mortality 5%) - Prognosis depends on the severity - With corrective surgery, 90% of patients will live into adulthood.Transposition of great arteries - Associated with VSD, Coarctation of Aorta, pulmonary stenosis - Immediate survival depends on a shunt between the systemic circulation and pulmonary circulation(PDA,ASD,VSD) - Picked up antenatally or within few days of life with cyanosis and respiratory distress and poor feeding. Management: - Prostaglandin infusion to keep PDA open till definitive. - Balloon septostomy : catheter inserted into the foramen ovale via balloon inflated to create large ASD - Open heart surgery CHD Acyanotic Cyanotic Left to Right Outflow Outflow Right to Left Shunt obstruction ina obstruction ina Shunt Common Mixing well child sick neonate (Blue) (Breathless) Transposition of Ventricular Atrial Septal Patent Ductus Pulmonary Aortic stenosis Coarctation of Tetralogy of the Great Septal Defect Defect Arteriosus stenosis the Aorta Fallot Arteries Eisenmenger’s Syndrome Question 1 A 2-day-old baby had been discharged home the day after delivery following a normal routine examination. He suddenly collapsed and was rushed to hospital. He was pale, with grey lips. The right brachial pulse could just be felt, the femoral pulses were impalpable and his liver was significantly enlarged. Blood gases showed a severe metabolic acidosis. He was ventilated. Blood cultures were taken and antibiotics started for possible sepsis. Blood and urine samples were taken for an amino acid screen and urine for organic acids. As the femoral pulses remained impalpable, a prostaglandin infusion was started. Within 2 hours, he was pink and well perfused and the acidosis was resolving. What is the most likely diagnosis? 1. Septicaemia 2. In-born error of metabolism 3. Coarctation of Aorta 4. Complete Atrio-Ventricular Septal defect 5. Patent Ductas Arteriosus Question 1 A 2-day-old baby had been discharged home the day after delivery following a normal routine examination. He suddenly collapsed and was rushed to hospital. He was pale, with grey lips. The right brachial pulse could just be felt, the femoral pulses were impalpable and his liver was significantly enlarged. Blood gases showed a severe metabolic acidosis. He was ventilated. Blood cultures were taken and antibiotics started for possible sepsis. Blood and urine samples were taken for an amino acid screen and urine for organic acids. As the femoral pulses remained impalpable, a prostaglandin infusion was started. Within 2 hours, he was pink and well perfused and the acidosis was resolving. What is the most likely diagnosis? 1. Septicaemia 2. In-born error of metabolism 3. Coarctation of Aorta 4. Complete Atrio-Ventricular Septal defect 5. Patent Ductas Arteriosus Question 2 4 year old boy presents with 3 episodes of shortness of breath, dizziness and fainting after running around with his siblings. He is otherwise well in himself. On examination a ejection systolic murmur can be heard over the left sternal edge 2 intercostal space. What is the most likely diagnosis? 1. Pulmonary stenosis 2. Exacerbation of asthma 3. Aortic Stenosis 4. Hypoglycaemia 5. Atrial Septal Defect Question 2 4 year old boy presents with 3 episodes of shortness of breath, dizziness and fainting after running around with his siblings. He is otherwise well in himself. On examination a ejection systolic murmur can be heard over the left sternal edge 2 intercostal space. What is the most likely diagnosis? 1. Pulmonary stenosis 2. Exacerbation of asthma 3. Aortic Stenosis 4. Hypoglycaemia 5. Atrial Septal Defect Question 3 Cyanosis is produced by deoxygenated haemoglobin of at least: 1. 1-2 gm/dl 2. 3-5 gm/dl 3. 6-8 gm/dl 4. 9-10 gm/dl Question 3 Cyanosis is produced by deoxygenated haemoglobin of at least: 1. 1-2 gm/dl 2. 3-5 gm/dl 3. 6-8 gm/dl 4. 9-10 gm/dl Question 4 Rheumatic heart disease results from of which of the following? 1. Damage to the heart valves from recurrent episodes of acute rheumatic fever 2. Untreated rheumatic arthritis 3. Initial group A streptococcal infection 4. Recurrent episodes of infective endocarditis Question 4 Rheumatic heart disease results from of which of the following? 1. Damage to the heart valves from recurrent episodes of acute rheumatic fever 2. Untreated rheumatic arthritis 3. Initial group A streptococcal infection 4. Recurrent episodes of infective endocarditis Question 5 What is the most common congenital heart defect with left to right shunt causing congestive heart failure in the paediatric age group? 1. Atrial Septal Defect 2. Atrioventricular Canal 3. Ventricular Septal Defect 4. Patent Ductas Arteriosus Question 5 What is the most common congenital heart defect with left to right shunt causing congestive heart failure in the paediatric age group? 1. Atrial Septal Defect 2. Atrioventricular Canal 3. Ventricular Septal Defect 4. Patent Ductas Arteriosus Question 6 What is the most likely age an infant with a large ventricular defect will begin manifesting symptoms of congestive heart failure? 1. 1 day 2. 1 week 3. 1 month 4. 6 months 5. 1 year Question 6 What is the most likely age an infant with a large ventricular defect will begin manifesting symptoms of congestive heart failure? 1. 1 day 2. 1 week 3. 1 month 4. 6 months 5. 1 year Question 7 A 5 year old boy comes in with a fever persisting for 9 days with a strawberry tongue, peeling skin and swollen lymph nodes. He is booked for an echocardiogram to rule out coronary artery aneurysm. What prophylactic medication helps reduce the risk of developing coronary artery aneurysm with the given underlying pathology? 1. Aspirin 2. Labetolol 3. Clopidogrel 4. IV Immunoglobins Question 7 A 5 year old boy comes in with a fever persisting for 9 days with a strawberry tongue, peeling skin and swollen lymph nodes. He is booked for an echocardiogram to rule out coronary artery aneurysm. What prophylactic medication helps reduce the risk of developing coronary artery aneurysm with the given underlying pathology? 1. Aspirin 2. Labetolol 3. Clopidogrel 4. IV Immunoglobins Thank you☺ Feel free to email me on sweathaananth@gmail.comReferences https://zerotofinals.com/paediatrics/ Illustrated book of paediatrics Teach me paediatrics