congenitalheartdisease-141220205557-conversion-gate01.pptx

chd

1. Congenital Heart Disease DR. SeHAR ZAHID

2. OUTLINE Epidemiology Anatomy Types Clinical approach History, physical exams Investigation General management

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

4. ANATOMY http://www.stanfordchildrens.org

5. www.rch.org.au

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

7. Atrial Septal Defect Most commonly asymptomatic Features: Right ventricular heave S 2 widely split and usually fixed Grade I-III/VI systolic murmur at the upper left sternal border Cardiac enlargement on CXR http://www.merckmanuals.com

8. Treatment Small defects: • No treatment Large defects: • Elective closure at 4-5 years age Paeds Protocol 3rd Ed

9. 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 http://www.merckmanuals.com

10. 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. Paeds Protocol 3rd Ed

11. 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 http://www.merckmanuals.com

12. 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. Paeds Protocol 3rd Ed

13. Tetralogy of Fallot http://my.clevelandclinic.org

14. TOF The most common cyanotic heart disease 4 components 1. Rt ventricular hypertrophy 2. VSD 3. Pulmonary stenosis 4. Over riding Aorta

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 (S 2 ) is common http://www.merckmanuals.com

16. Chest Xray

17. Transposition of great arteries http://mvpresource.com

18. CXR

19. TAPVR

20. CXR

21. Tricuspid atresia www.riversideonline.com

22. CXR

23. 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. Paeds Protocol 3rd Ed

24. PHYSICAL EXAMINATIONS Dysmorphism : Trisomy 21, 18, 13; Turner syndrome Central cyanosis. Tachypnoea . Weak or unequal pulses. Heart murmur. Hepatomegaly . Paeds Protocol 3rd Ed

25. 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: pO ₂ < 100 mmHg; rise in pO ₂ is < 20 mmHg. - Echocardiography Paeds Protocol 3rd Ed

26. GENERAL MANAGEMENT Initial stabilization: secure airway, adequate ventilation, circulatory support Correct metabolic acidosis, electrolyte derangements, hypoglycaemia ; prevent hypothermia. • Empirical treatment with IV antibiotics. • Early cardiology consultation. Paeds Protocol 3rd Ed

27. • 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.

28. • If unresponsive to IV prostaglandin E, consider: Transposition of great arteries, obstructed total anomalous pulmonary. Non-cardiac diagnosis. • Arrangement to transfer to regional cardiac center once stabilized.

29. Surgical treatments BT shunt Modified BT shunt Total correction

30. THANK YOU