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Paediatric Surgery Congenital Heart Diseases Shathar Mahmood Patent Ductus Arteriosus Left-to-right shunt Atrial septal defect Ventricular septal defect Acyanotic Pulmonary Stenosis Outflow obstruction Aortic Stenosis CHD Coarctation of aorta Tetralogy of Fallot Tricuspid atresia Cyanotic Transposition of great vessels Truncus arteriosusPatent DuctusArteriosus Causes Complications • Genetic • Heart failure • Maternal infection • Eisenmenger’s (Rubella) syndrome Features Management • SOB • Conservative – if <1y/o, • Difficulty feeding asymptomatic, and no • Poor weight gain complications • LRTI • Medical – indomethacin • Continuous crescendo- • Surgical - transcatheter decrescendo murmur or surgical closure (LUSB) Investigations • Echocardiogram + doppler studies • CXRAtrial Septal Defect Types • Ostium primum • Ostium secundum Features • Ejection systolic murmur, fixed splitting of S2 Management • Dyspnoea • Conservative – if small and asymptomatic • Can present in • Surgical - transcatheter adulthood with pHTN, or surgical closure RHF, stroke, or AF In adults – add aspirin, Investigations warfarin, and DOACs • Echocardiogram (stroke prophylaxis) • ECGVentricular Septal Defect Causes Complications • Isolated defect • Aortic regurgitation • Part of an underlying • Infective endocarditis genetic condition • RHF • Congenital infections • Eisenmenger’s Features syndrome • Detect on 20wk scan • Failure to thrive Management • HF (hepatomegaly, • Conservative – manage nutrition and heart tachypnoea, failure tachycardia, pallor) • Surgical • Pansystolic murmur Investigations • Echocardiogram • ECG • CXRT etralogy of Fallot Risk factors • Rubella • Increased maternal age >40y/o • Alcohol • Maternal DM Features • Cyanosis • Clubbing • Poor feeding and failure to thrive • Ejection systolic Management murmur • Prostaglandin infusion • ‘tet spells’ • Total surgical repair Investigations • Echocardiogram • CXRTransposition of the GreatArteries Risk factors • Maternal DM Features • Cyanosis • Tachypnoea • Loud single S2 • Prominent right ventricular impulse Investigations • Echocardiogram • CXR Management • Prostaglandins • SurgeryCoarctation of theAorta Associations • Male sex • Turner’s syndrome • Bicuspid aortic valve • Neurofibromatosis Features • HF (infancy), HTN (adult) • Radio-femoral delay • Mid-systolic murmur Management • Underdevelopment of limbs and end-organ • Symptom-dependent hypoperfusion • Surgical repair Investigations Complications • CT angiography • RecurrenceHypoplastic Left Heart Investigations Associations • Diagnosed prenatally • Generic mutations • Patau syndrome • Echocardiogram • Edward’s syndrome Management Features • Prostaglandins • Cyanosis • Surgical repair (usually • Respiratory distress 3-stage and palliative) • Poor feeding • No response to O2 • Weak peripheral pulses • Cardiogenic shock (and death)Atrioventricular Septal Defect TricuspidAtresiaSyndrome Cardiac anomaly Fetal alcohol syndrome ASD, VSD Trisomy 21 AVSD, ASD, VSD Turner syndrome Coarctation of aorta, bicuspid aortic valve Noonan syndrome Pulmonary stenosis, ASD, hypertrophic cardiomyopathy Williams syndrome Supravalvular aortic stenosis, peripheral pulmonary stenosis Marfan syndrome Mitral valve prolapseQ1. A systolic murmur at the upper left sternal edge: a. Is most unlikely to be a PDA in a preterm baby at 2 weeks of age b. Could be a VSD, especially if there is a harsh rasping quality to the murmur c. Could be due to pulmonary valve incompetence in a 3–4 year old child with Noonan's syndrome d. Is most unlikely to be an innocent heart murmur, even if the pulses are normal e. Could be due to a persistent foramen ovale in a newborn babyQ2. The two congenital heart diseases that classically show left axis deviation on ECG are? a. AVSD, VSD b. AVSD, Tricuspid atresia c. VSD, d-TGA d. VSD, Truncus arteriosusQ3. A 1-month-old baby suffers from intermittent episodes of cyanosis and tachypnoea, particularly when distressed. An examination reveals a harsh ejection-systolic murmur. Given the likely diagnosis, what is the best predictor of the clinical severity of this condition? a. The degree of aortic stenosis b. The degree of left ventricular hypertrophy c. The degree of pulmonary stenosis d. The degree of right ventricular hypertrophy e. The size of the ventricular septal defectQ4. Supravalvular aortic stenosis is found in a 3-year old boy with learning difficulties: a. Noonan Syndrome b. Fragile X Syndrome c. Williams Syndrome d. Edwards Syndrome e. Pierre-Robin SyndromeQ5. A 15-year-old girl of short stature presents to the GP as she hasn't started her period yet. On general examination, the GP notices that she has a wide neck. When examining her chest, the GP hears a murmur during systole. Given the most likely diagnosis, what option from the choices below is this condition associated with? a. Coarctation of the aorta b. Mitral regurgitation c. Mitral stenosis d. Mitral valve prolapse e. Tricuspid regurgitationQ6. You are called to the postnatal ward as part of the neonatal team to review an infant born 8 hours ago who has suddenly turned blue and has an increased work of breathing. There is no grunting, and he is not tachypnoeic. There is no murmur on examination. A chest x-ray has been done which shows obvious cardiomegaly, but it has not yet been reported. The ward team have already tried giving oxygen, but this has not improved the breathing. What is the most appropriate initial management? a. Alprostadil b. Furosemide c. Ibuprofen d. Insert naso-gastric tube for feeding e. OxygenQ7. A boy, born at term, via caesarean section is under cardiology review after he was found to have a pansystolic murmur. Cardiology reports a ventricular septal defect (VSD). His mother becomes very anxious when she finds out and is keen to find out more about VSD. She asks about the risks to her son. Which one of the following is he at a high risk of? a. Essential hypertension b. Aortic aneurysm c. Carotid dissection d. Endocarditis e. Papillary muscle atrophyQ8. A 28-year-old pregnant female attends a routine clinic appointment. She is concerned because one of her fetal ultrasound reports shows evidence of a large patent ductus arteriosus in the fetus. She has read on the internet that indomethacin can treat this condition and wants to know more about the drug. What will you tell the mother about the administration of this drug? a. It is given to the mother if repeat fetal ultrasound shows a large patent ductus arteriosus b. It is given to the newborn right after delivery c. It is given to the newborn if the echocardiogram shows patent ductus arteriosus one week after delivery d. It is not used for closing the patent ductus arteriosus as it keeps the ductus arteriosus open e. It is given to the mother between week 26 and 28 of pregnancyQ9. Which of the following statements are true of Coarctation of the aorta? a. It usually presents with cyanosis and can be detected with a drop in oxygen saturations from pre to post ductal measurements b. It typically presents with hypertension in the legs in newborn babies c. It can present with renal failure and lactic acidosis as the duct closes d. It is the commonest form of congenital heart defect e. It is associated with Down's syndrome but not Turner's syndromeQ10. A 4-year-old girl presents to her GP following a productive cough and wheeze. On examination a systolic murmur is heard in the second 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 stenosisThanks to our partners!@supta_uk @SUPTAUK www.supta.uk