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Congenital heart diseases
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Congenital heart disease

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paeds, congenital heart disease

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Congenital heart disease

  1. 1. Congenital Heart Disease By: Dr Ismah, Pediatric Department 1
  2. 2. OUTLINE • Epidemiology • Anatomy • Types • Clinical approach - History, physical exams - Investigation - General management 2
  3. 3. EPIDEMIOLOGY • CHDs affect nearly 1% of or about 40,000 births per year in the United States • The most common type of heart defect is a ventricular septal defect (VSD) • About 95% of babies born with a non-critical CHD are expected to survive to 18 years of age [2012] • About 69% of babies born with critical CHDs are expected to survive to 18 years of age [2012] http://www.cdc.gov • A study on under five deaths in Malaysia in the year 2006 showed that 10% of mortality was directly related to CHD - http://mjpch.com 3
  4. 4. ANATOMY http://www.stanfordchildrens.org 4
  5. 5. www.rch.org.au 5
  6. 6. TYPES Acyanotic Cyanotic • Atrial septal defects (ASD) • Ventricular septal defects (VSD) • Patent ductus arteriosus (PDA) • Tetralogy of Fallot (TOF) • Tricuspid atresia (TA) • Transposition of the great vessels 6
  7. 7. 7
  8. 8. Atrial Septal Defect • Most commonly asymptomatic • Features: - Right ventricular heave - S2 widely split and usually fixed - Grade I-III/VI systolic murmur at the upper left sternal border - Cardiac enlargement on CXR 8 http://www.merckmanuals.com
  9. 9. Treatment Small defects: • No treatment Large defects: • Elective closure at 4-5 years age 9 Paeds Protocol 3rd Ed
  10. 10. Ventricular Septal Defect Clinical findings • Grade II-IV/VI, medium- to high- pitched, harsh pansystolic murmur heard best at the lower left sternal border with radiation over the entire precordium 10 http://www.merckmanuals.com
  11. 11. Treatment Small defects: Moderate defects: Large defects: No treatment; high rate of spontaneous closure. • SBE prophylaxis. • Yearly follow up for aortic valve prolapse, regurgitation. • Surgical closure indicated if prolapsed aortic valve. - Anti-failure therapy if heart failure. - Surgical closure if: • Heart failure not controlled by medical therapy. • Persistent cardiomegaly on chest X-ray. • Elevated pulmonary arterial pressure. • Aortic valve prolapse or regurgitation. • One episode of infective endocarditis. Early primary surgical closure. • Pulmonary artery banding followed by VSD closure in multiple VSDs. 11 Paeds Protocol 3rd Ed
  12. 12. Patent Ductus Arteriosus • Pulses are bounding and pulse pressure is widened • Characteristically has continuous murmur is heard best in the upper left sternal border, machinery murmur 12 http://www.merckmanuals.com
  13. 13. Treatment Small PDA: • No treatment if there is no murmur • If murmur present: elective closure as risk of endarteritis. Moderate to large PDA: • Anti-failure therapy if heart failure • Timing, method of closure (surgical vs transcatheter) depends on symptom severity, size of PDA and body weight. 13 Paeds Protocol 3rd Ed
  14. 14. Tetralogy of Fallot 14 http://my.clevelandclinic.org
  15. 15. Typical features • Symptoms include cyanosis, dyspnea with feeding, poor growth, and • Hypercyanotic "tet" spells (sudden, potentially lethal episodes of severe cyanosis) • A harsh systolic murmur at the left upper sternal border with a single 2nd heart sound (S2) is common 15 http://www.merckmanuals.com
  16. 16. Transposition of great arteries 16 http://mvpresource.com
  17. 17. Tricuspid atresia 17 www.riversideonline.com
  18. 18. HISTORY • Antenatal scans (cardiac malformation, fetal arrhythmias, hydrops). • Family history of congenital heart disease. • Maternal illness: diabetes, rubella, teratogenic medications. • Perinatal problems: prematurity, meconium aspiration, perinatal asphyxia. 18 Paeds Protocol 3rd Ed
  19. 19. PHYSICAL EXAMINATIONS • Dysmorphism: Trisomy 21, 18, 13; Turner syndrome • Central cyanosis. • Tachypnoea. • Weak or unequal pulses. • Heart murmur. • Hepatomegaly. 19 Paeds Protocol 3rd Ed
  20. 20. INVESTIGATIONS - CXR - Hyperoxia test: • Administer 100% oxygen via headbox at 15 L/min for 15 mins. • ABG taken from right radial artery. • Cyanotic heart diseases: pO₂ < 100 mmHg; rise in pO₂ is < 20 mmHg. - Echocardiography 20 Paeds Protocol 3rd Ed
  21. 21. GENERAL MANAGEMENT • Correct metabolic acidosis, electrolyte derangements, hypoglycaemia; prevent hypothermia. • Empirical treatment with IV antibiotics. • Early cardiology consultation. 21 Paeds Protocol 3rd Ed
  22. 22. • IV Prostaglandin E infusion if duct- dependent lesions suspected: - Starting dose: 10 – 40 ng/kg/min; maintenance: 2 – 10 ng/kg/min. - Adverse effects: apnoea, fever, hypotension. 22
  23. 23. • If unresponsive to IV prostaglandin E, consider: - Transposition of great arteries, obstructed total anomalous pulmonary. - Blocked IV line. - Non-cardiac diagnosis. • Arrangement to transfer to regional cardiac center once stabilized. 23
  24. 24. THANK YOU 24
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paeds, congenital heart disease

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