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A cyanotic congenital heart diseases

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A cyanotic congenital heart diseases

  1. 1. A Cyanotic Congenital Heart Diseases Prepared by Ismail Fadhil Abbas 6th Stage – A2 2019-2020 Undersupervision of Dr. Zeyad Mohammed
  2. 2. 1. Epidemiology
  3. 3. ✗ CHD incidence 8 per 1000 births. ✗ CHD may be asymptomatic. ✗ CHD multifactorial (chromosomal disorders, single gene defects, teratogens, or maternal metabolicdisease). 3
  4. 4. 2. Presenting Problems
  5. 5. Depend on Volume of shunt & severity of obstruction: 1. Feeding difficulties 2. Tachypnea 3. Sweating 4. Sub-costalrecession 5. Recurrent respiratoryinfection 6. Growth impairment in infants 7. Exercise intolerance 8. Easy Fatigue 9. Murmur in olderchildren 5
  6. 6. 3. Classification
  7. 7. 7
  8. 8. 3.1 Atrial Septal Defect (ASD)
  9. 9. 9
  10. 10. ASD: occur due to failure of septal growth or excessive reabsorption of tissue. F:M ratio 3:1 Classification: 1- Secundum ASD (80%): the most common type, it is caused by defect in the floor of fossa ovalis, resulting in failure of the septum secondum to develop completely. 2- Primum ASD or partial atrioventricular septal defect: Ostium primum ASDs may occur in isolation but most commonly present with a cleft in the anterior leaflet of the mitral valve (partial atrioventricular septal defect) 3- Sinus venosus defect: least common, occurs in the upper atrial septum & associated with anomalous pulmonary venous return 10
  11. 11. 11
  12. 12. Pathophysiology:Shuntingleft to right. The degree of this shuntingis dependenton: 1. Size of the defect 2. Relative vascular resistance in the pulmonary and systemic circulations. Resistance in the pulmonaryvascular is commonly normal in children with ASD, and increase in volume load is usually well tolerated. The chronic significant left-to-right shunt can alter the pulmonaryvascular resistance leading to pulmonaryarterial hypertension, even reversal of shunt and Eisenmenger syndrome (if not treated) 12
  13. 13. Symptoms 1. Asymptomatic (commonly) 2. Breathlessness, tiredness on exertion 3. Recurrent chest infections with wheezing 4. Heart failure 5. Arrhythmias Physical Signs 1. A fixed and widely split 2nd heart sound 2. An ejection systolic murmur (soft), upper left sternal edge 13
  14. 14. Investigatons: A- Chest X-ray 1. Usually normal 2. Cardiomegaly 3. Enlarged pulmonary arteries 4. Increased pulmonary vascular markings B- Echocardiography Documents type, size and direction of shunt. It’s the mainstay of diagnostic investigations C- ECG: Provide strong diagnostic clue: 1. Both: right bundle branch block 2. Secundum ASD – shows right axis deviation 3. Partial AVSD – shows left axis deviation (superior axis) 14
  15. 15. 15 Chest X-ray Findings: 1. Cardiomegaly 2. Enlarged pulmonary arteries 3. Increased pulmonary vascular markings
  16. 16. 16 Echocardiography
  17. 17. 17 ECG Findings: 1. Secundum ASD – shows right axis deviation 2. Right bundle branch block
  18. 18. Management A- Secundum ASDs: If significant shunt is present at around 3 y/o, closureis recommended. Cardiac catheterizationwith insertion of an occlusion device (closuredevice) B- Primum ASD: Prophylaxisfor subacute bacterial endocarditis. Surgical correctionat 3-5 y/o to prevent right heart failure and arrhythmias in later life. 18
  19. 19. Closure Device 19
  20. 20. Prognosis: ASDs detected in term infants may close spontaneously. SecundumASDs are well toleratedduring childhood, and symptoms do not usuallyappear until the 3rd decadeor later. 20
  21. 21. Complications: 1. Congestive heart failure 2. Arrhythmias 3. Pulmonaryhypertension 4. Infective endocarditis 5. Surgery may be associated with a long-termrisk of atrial fibrillation or flutter. The risk of infective endocarditisexists during the first 6 months after surgery. 21
  22. 22. 3.2 Ventricular Septal Defect (VSD)
  23. 23. 23
  24. 24. VSD: Most Common CHD. Three important things with VSD: ✗ Location ✗ Size ✗ Pulmonaryvascular resistance The amount of flow crossing a VSD dependson the size of defect and the pulmonaryvascular resistance. 24
  25. 25. A- Locationof the VSD (prognostic and repair approach): The VSDs are subdivided according to the part of the septum they occur in : ✗ Muscular, ✗ Perimembranous (adjacent to the tricuspid valve), ✗ Inlet, ✗ Outlet 25
  26. 26. Locations of the VSD 26
  27. 27. B- Pulmonaryvascularresistance: At birth, the pulmonaryvascular resistance is normally elevated, thus, even large VSDs are not symptomatic at birth. Over the first 6-8 weeks of life, pulmonaryvascular resistance normally decreases. More blood flows through the lung and into the left atrium. However, in VSD, the amount of shunt increases, and symptoms may start to develop. 27
  28. 28. C- Size of VSD 28
  29. 29. Pathophysiology: VSD permits a left-to-right shunt to occur at the ventricular level with 3 adverse hemodynamic consequences: ✗ Left ventricular (LV) volume overload ✗ Increased pulmonarybloodflow ✗ Compromise of systemic cardiac output 29
  30. 30. Symptoms A- Small VSD: Asymptomatic B- Large VSD: 1. Heart failure with breathlessness 2. Failure to thrive. 3. Recurrent chest infections Physical Signs 1. Murmur Grade II-IV/VI 2. Medium- to high-pitched 3. Harsh 4. Pansystolic murmur: heard best at the lower left sternal border with radiation over the entire precordium 30
  31. 31. Investigations: ✗ A-Echocardiography: Demonstrates the anatomy defect, hemodynamic effects and severity of pulmonary HPT. Small VSD: Chest X-ray & ECG – normal Large VSD: 1- Chest X-ray shows ✗ Cardiomegaly ✗ Enlarged pulmonary arteries ✗ ↑ Pulmonary vascular markings ✗ Pulmonary oedema 2- ECG : ✗ Biventricular hypertrophy and signs of pulmonary HPT ✗ Right ventricular enlargement and hypertrophy (if not treated) 31
  32. 32. 32 Echocardiography
  33. 33. 33 Chest X-ray Findings (large VSD)
  34. 34. 34 ECG Findings (large VSD)
  35. 35. Management of small VSD: Most will close spontaneously. Ensureby the disappearanceof the murmur, normal ECG on follow up, normal echocardiogram. While the VSD is present, for prevention of bacterial endocarditis : ✗ Maintain good dental hygiene ✗ Antibioticprophylaxis before dental extraction or any operation where there’ll be bleeding Surgical closure may not be required 35
  36. 36. Complications: 1. PulmonaryHypertension 2. Heart Failure 3. Growth delay 4. Eisenmenger complex (cyanosis, PH & Erythrocytosis) 5. Secondaryaortic insufficiency &Aortic regurgitation 6. Infective endocarditis 36
  37. 37. 3.3 Atrio-ventricular Canal Defects
  38. 38. 38
  39. 39. AVCD: Also referred to as Endocarial cushion defect, may be complete or partial . Pathophysiology: The defect occurs as the result of abnormal development of the Endocardial cushion tissue, resulting in failure of the septum to fuse with the Endocardial cushion; this results in abnormal atrioventricular valves as well. The complete defect results in a primum ASD, Inlet VSD, and cleft in leaflet of mitral and cleft leaflet of the tricuspid valves. In addition to left-to-right shunting at both levels, there may be atrioventricular valvular insufficiency. 39
  40. 40. Clinical Manifestations: The symptoms of CHF usually develop as the pulmonary vascular resistance decreases over the first 6 to 8 weeks of life. A- Complete atrioventricular canal defect: develops in the first several weeks of life: 1. Difficulty breathing or rapid breathing 2. Wheezing 3. Fatigue 4. Lack of appetite 5. Poor weight gain 6. Pale skin color 7. Bluish discoloration of the lips and skin 8. Excessive sweating 9. Irregular or rapid heartbeat 40
  41. 41. B- Partial atrioventricular canal defect: not appears until early adulthood and might be related to complications: 1. Abnormal heartbeat(arrhythmia) 2. Shortnessof breath 3. High blood pressure in the lungs (pulmonary hypertension) 4. Heart failure 41
  42. 42. Investigations: The diagnosis usually is made with echocardiography. A- Chest x-ray: shows cardiomegaly with enlargement of all chambers and the presence of increased vascularity. B- ECG: shows left axis deviation and combined ventricular hypertrophy and may show combined atrial enlargement. Treatment: 1. Initial treatment (Medical): diuretics and ACI (captopril) or digoxin diuretics for treatment of CHF. 2. Surgical repair of the entire defect ultimately is required. 42
  43. 43. 43 Echocardiography
  44. 44. 44 Chest X-ray Findings: 1. Cardiomegaly 2. Increased pulmonary vascular markings 3. Enlargement of all chambers of heart
  45. 45. 45 ECG Findings
  46. 46. 3.4 Patent Ductus Arteriosus
  47. 47. 47
  48. 48. DA: allows blood to flow from the pulmonaryartery to the aorta during fetal life. This changes to the oppositeafter birth. In term infants, it normally closes shortly after birth. Failure of the normal closureof it by a month post term is due to a defect in the constrictor mechanism of the duct. In preterminfants, the PDA is not from CHD but due to prematurity. 48
  49. 49. Symptoms: Depend on size of PDA 1. Small: asymptomatic 2. Moderate to larger shunts: symptoms of CHF or even pulmonary HPT Signs: 1. Continuous machinery murmur beneath the left clavicle 2. Widened pulse pressure collapsing or bounding pulse Investigations: 1. Chest X-ray & ECG: Usually normal unless the PDA is large and symptomatic 2. Features seen are indistinguishable from VSD so Duct should be readily identified by echocardiography 49
  50. 50. Management: A- Small PDA: closureis recommended due to the risk of bacterial endocarditis B- Moderateand large PDA: initially diuretics & digoxin, but eventually closure(Closure is with a coil or occlusion device introducedvia cardiac catheterat about 1 y/o) 50
  51. 51. 3.5 Coarctation of the Aorta
  52. 52. 52
  53. 53. COA: It is almost always juxtaductalin position (the part near where the Ductus Arteriosus attaches) During development of the aortic arch, the area near the insertion of the Ductus Arteriosus fails to develop correctly, resultingin a narrowing of the aortic lumen. 53
  54. 54. Clinical Manifestations: Timing of presentation dependsprimarily on the severity of obstruction and associated cardiac defects. Symptoms: 1. Severe: Shock 2. Moderate: CHF 3. Mild : leg discomfort with exercise, headache, or epistaxis. 54
  55. 55. Signs: 1. Femoral pulses are weaker and delayed compared with the radial pulses. 2. The blood pressure in the lower extremities is lower than that in the upperextremities 3. The murmur is typically best heard in the left interscapular area of the back. Continuous murmurs may be heard throughout thechest. An abnormal aortic valve is present approximately 50% of the time, causing a systolic ejection click and systolic ejection murmur of aortic stenosis. 55
  56. 56. Investigations: 1. Echocardiography:shows the site of Coarctation and associated lesions. 2. In olderchildren, the ECG and chest x-ray usuallyshow left ventricular hypertrophyand a mildly enlarged heart with rib notchingmay also be seen in olderchildren (>8 years old)with large collaterals. Management: Balloonangioplasty or surgical repair. 56
  57. 57. 57 Thanks! Any Questions? References: - Nelson Textbook - Stanford Children’s Health
  58. 58. Case: 3 month old female presented with dypnea with recurrent chest infection. On ausculation, wheezing heard with fixed and widely split 2nd heart sound. Chest x-ray was normal but ECG showed right bundlebranch block. What is your diagnosis? How can you treat this case? 58