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Congenital heart disease, by dr Shaymaa Fayad, El Nasr Hospital Port said

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25 Ferbruary talk by El Nasr hospital pediatricians:
By Dr Shaymaa Fayad Congenital heart disease

Published in: Health & Medicine
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Congenital heart disease, by dr Shaymaa Fayad, El Nasr Hospital Port said

  1. 1.  Blood from the placenta is carried to the fetus by the umbilical vein. Less than 50% of this enters the fetal ductus venosus and is carried to the inferior vena cava, while the rest enters the liver proper from the inferior border of the liver.  The blood then moves to the right atrium of the heart.
  2. 2.  In the fetus, there is an opening between the right and left atrium (the foramen ovale), and most of the blood flows through this hole directly into the left atrium from the right atrium, thus by passing pulmonary circulation.
  3. 3.  The continuation of this blood flow is into the left ventricle, and from there it is pumped through the aorta into the body.  blood from SVC entering the right atrium but does not pass directly to the left atrium through the foramen ovale, enters the right ventricle and is pumped into the pulmonary artery.
  4. 4.  Other special connection between the pulmonary artery and the aorta found, called the ductus arteriosus, which directs most of this blood away from the lungs (which are not being used for respiration at this point as the fetus is suspended in amniotic fluid).  Because the pulmonary arterial circulation is vasoconstricted, only about 5% of ventricular outflow enters the lungs.
  5. 5.  The placenta is not as efficient an oxygen exchange organ as the lungs, so that umbilical venous Po2 (the highest level of oxygen provided to the fetus) is only about 30-35 mm Hg.  Intracardiac pressure remains identical between the right and left ventricles of the human fetus.
  6. 6.  during fetal life the Rt ventricle is not only pumping against systemic blood pressure but is also performing a greater volume of work than the left ventricle.  Thickening of Rt ventricular wall.  Rt axis deviation in fetal and neonatal period.
  7. 7. Foramen ovale : � Closes at birth due to 1. decreased flow from placenta and IVC to hold open foramen 2.increased pulmonary blood flow and pulmonary VR to left heart causing the pressure in the left atrium to be higher than in the right atrium. 3.Some times foramen may remain probe patent for several years. �
  8. 8. Other changes in the heart:  The output from the right ventricle now flows entirely into the pulmonary circulation.  By the end of the first month, the left ventricular wall is thicker and the right ventricular wall becomes thinner
  9. 9. Ductus Arteriosus �  The DA constricts at birth, but there is often a small shunt of blood from the aorta to the left pulmonary artery for a few days in a healthy, full-term infant . �  In premature infants and in those with persistent hypoxia the DA may remain open for much longer. �  Oxygen is the most important factor in controlling closure of the DA in full-term infants.
  10. 10.  When the PO2 of blood passing through the DA reaches about 50 mm Hg, the wall of the DA constricts.  Closure of the DA appears to be mediated by bradykinin( a substance released by the lungs upon initial inflation), and by Oxygen� effect on decreasing PG E2 and prostacylcin secretion  As a result of reduced pulmonary vascular resistance, the pulmonary arterial pressure falls below the systemic level and the blood flow thrugh the ductus arteriosis is diminished.
  11. 11.  The largest decline in pulmonary resistance level usually occurs within the 1st 2-3 days but may be prolonged for 7 days or more.  Over the next several weeks of life, pulmonary vascular resistance decreases even further.  This decrease in pulmonary vascular resistance significantly influences the timing of the clinical appearance of many congenital heart lesions that are dependent on the relative levels of systemic and pulmonary vascular resistances.
  12. 12. Fetal Structure  Foramen Ovale  Umbilical Vein (intra-abdominal part)  Ductus Venosus  Umbilical Arteries and ligaments abdominal  Ductus Arteriosum Adult Structure  Fossa Ovalis  Ligamentum teres  Ligamentum venosum  Medial umbilical ligaments,superior vesicular artery (supplies bladder)  Ligamentum arteriosum
  13. 13. Congenital Heart Disease
  14. 14.  Congenital heart disease is a category of heart disease that includes abnormalities in cardiovascular structures that occur before birth.  May affect approximately 8 in 1000 live births, 2% in preterm.  Congenital heart defects may produce symptoms at birth, during childhood, or not until adulthood. Other congenital defects may cause no symptoms.
  15. 15. ETIOLOGY Usually the cause of congenital heart disease is unknown. Risk factors include: 1.Genetic or chromosomal abnormalities in the child, such as Down syndrome. 2.Taking certain medications or alcohol or drug abuse during pregnancy.
  16. 16. 3.Maternal viral infection, such as rubella (German measles) in the first trimester of pregnancy. 4.The risk of having a child with congenital heart disease may double if a parent or a sibling has a congenital heart defect.
  17. 17. Classification  a cyanotic CHD that subdivided into: a-with increased pulmonary blood flow. b-with normal pulmonary blood flow(stenotic lesion).  cyanotic CHD that subdivided into: a-with increased pulmonary blood flow. b-with decreased pulmonary blood flow.
  18. 18. 1-VSD 2-ASD 3-COMPLETE A-V CANAL DEFECT 4-PDA 5-Partial anomaly pulmonary venous return 6-Aorticopulmonary window defect
  19. 19. 1.Aortic stenosis 2.Pulmonary stenosis 3.Coarctation of the aorta 4.Congenital mitral stenosis
  20. 20. Abnormal communication in ventricular septum dividing RV and LV. The most common cardiac anomaly about 15-25% of cases of CHD.
  21. 21. According to size of the defect divided into  Restrictive VSD: small defect <0.5cm2. Lt to Rt shunt occur due to higher Lt ventricular pressure, Rt ventricular pressure usually normal → increased pulmonary blood flow, pulmonary congestion and CHF.  Non restrictive VSD: large defect >1cm2. The pressure in both ventricles is equalized and the direction and magnitude of the shunt dependent on the ratio between pulmonary and systemic circulation.
  22. 22.  According to size of the defect and pulmonary blood flow and pressure. 1. asymptomatic and discovered accidently during routine examination: small defect or early in first few days of life where pulmonary pressure and resistance still high. 2. congestive lung symptoms: dyspnea, cough, and repeated chest infection. 3. low cardiac output symptoms (interrupted feeding, syncope). 4. if not corrected can lead to Eisenmenger syndrome.
  23. 23. 5.Low cardiac output signs(small pulse volume, pallor, cold extremities and excessive sweating when heart failure occur, duskiness may seen during infection or crying but cyanosis is usually absent. 6. precordial bulging. 7. biventricular hypertrophy. 8. pulmonary artery diltation (pulsations seen and felt with palpable S2). 9. increase ps2(pulmonary area) 10. Lt parasternal area: Harsh pansystolic murmur. Mid diastolic murmur (functional MS) due to increase blood flow across mitral valve.
  24. 24. 1)X-ray chest:  Lung congested.  Heart : biventricular hypertrophy , pulmonary artery dilatation.
  25. 25. 2) ECG:  Early and small VSD :mainly LT ventricular hypertrophy.  Large VSD :biventricular hypertrophy.
  26. 26. 3) Echo (2 dimensional and doppler)  Shows position and size of the defect.  Examining the degree of volume overload in Lt atrium and ventricle to estimate size of the defect.  AR.  Pressure gradient across the defect(restrictive or non restrictive type). 4) Catheterization:  hemodynamics of VSD.
  27. 27. 1. Repeated chest infection. 2.HF. 3. Infective endocarditis. 4. Acquired infundibular PS 5. Eisenmenger’s syndrome: increase pulmonary blood flow with pulmonary congestion →p arteriolar V.C → increase pulmonary artery pressure → increase Rt sided pressure up to reversal of the shunt → cyanosis First reversible V.C then permanent sclerotic changes occur and permanent V.C and reversal of the shunt.
  28. 28.  A significant number (30-50%) of small defects close spontaneously, most frequently during the 1st 2 yr of life.  Small muscular VSDs are more likely to close (up to 80%) than membranous VSDs (up to 35%).  Surgical correction for infants with large defects have repeated episodes of respiratory infection and heart failure despite optimal medical management.
  29. 29.  Abnormal communication in atrial septum.  can occur in any portion of the atrial septum (secundum, primum, or sinus venosus).  Isolated secundum ASDs account for ≈7% of congenital heart defects
  30. 30.  The majority of cases of ASD are sporadic; autosomal dominant inheritance does occur as part of the Holt- Oram syndrome (hypoplastic or absent radii, 1st-degree heart block, ASD)
  31. 31.  Dependent on the size of the shunt and PVR  In large defects, a considerable shunt of oxygenated blood flows from the left to the right atrium  Shunting of blood from Lt atrium to Rt atrium during systole→ Rt ventricular hypertrophy and dilatation
  32. 32.  Despite the large pulmonary blood flow, pulmonary arterial pressure is usually normal because of the absence of a high-pressure communication between the pulmonary and systemic circulations.  Pulmonary vascular resistance remains low throughout childhood, although it may begin to increase in adulthood and may eventually result in reversal of the shunt and clinical cyanosis.
  33. 33.  May be a symptomatic  Congestive lung symptoms  Complications as HF (rare in early childhood), infective endocarditis and Eisenmenger’s  Mild Lt pericordial pulg (Rt ventricular enlargement)
  34. 34.  Wide fixed split of S2  No murmur because of the shunt  Functional PS → ejection systolic murmur over pulmonary area  Functional tricuspid stenosis →mid diastolic over tricuspid area
  35. 35. X-ray chest  Congested lung  Rt ventricular hypertrophy  Pulmonary artery dilatation
  36. 36. ECG  Rt axis deviation may be present  Rt ventricular hypertrophy
  37. 37. Echo 1.Features of Rt ventricular volume over load e.g., flattening and abnormal motion of ventricular septum 2.The location and size of ASD Catheterization 1.Confirm the presence of the defect 2. Directly measures pulmonary artery pressure and compare pulmonary artery to systemic artery pressure
  38. 38.  Most ASDs <8 mm spontaneously close  Surgical repair of large defect usually after first year of age and before entering school  Mortality rate in childhood <1 %, more in adulthood
  39. 39.  Lt to Rt shunt at both atrial and ventricular level →↑↑ pulmonary blood flow and early onset pulmonary hypertension (↑↑ risk of eisenmenger’s syndrome)  MI and TI→ volume overload on both Lt and Rt venrticle
  40. 40.  The liver is enlarged and the infant shows signs of failure to thrive  Early onset of HF (pulmonary congestion, low CO, systemic congestion)  Transient episodes of cyanosis  Complete endocardial cushion is common in children with Down syndrome.
  41. 41. Auscultatory signs produced by the left-to-right shunt include:  a normal or accentuated 1st heart sound.  wide, fixed splitting of the 2nd sound.  a pulmonary systolic ejection murmur sometimes preceded by a click.
  42. 42.  If there’s large VSD component, S2 will be single.  additional apical holosystolic murmur caused by mitral insufficiency.
  43. 43. x-ray chest  Cardiomegaly with enlargement of all chambers  Lung congestion
  44. 44. ECG 1.Lt axis deviation 2.Combined ventricular hypertrophy 3.May show combined atrial enlargement
  45. 45. Echo 1.is characteristic 2. “gooseneck” deformity of the left ventricular outflow tract. 3.The presence of associated lesions such as patent ductus arteriosus (PDA) or coarctation of the aorta. 4.Doppler echocardiography will demonstrate left-to-right shunting at the atrial and ventricular level.
  46. 46. Catheterization:  is rarely required unless pulmonary vascular disease is suspected, such as in a patient in whom diagnosis has been delayed beyond early infancy, especially with Down syndrome
  47. 47.  Because of the risk of pulmonary vascular disease developing as early as 6-12 mo of age, surgical intervention must be performed during infancy.  Treatment of heart failure if present
  48. 48.  Persistence of fetal connection between pulmonary artery and aorta  F:M is 2:1  Increased incidence in prematurity, trisomy 21 and maternal rubella.
  49. 49.  Lt to Rt shunting → pulmonary congestion and increased pulmonary artery pressure.  Increase blood passing to Lt ventricle → Lt ventricular hypertrophy  Increase pulse pressure due to run off of blood into pulmonary artery during diastole
  50. 50.  A small PDA is usually asymptomatic  A large PDA will result in heart failure similar to that in infants with a large VSD.  Retardation of physical growth  Bounding peripheral arterial pulses  a wide pulse pressure  apical impulse is prominent and heaving.  A thrill, maximal in the 2nd left interspace
  51. 51.  Increase PS2 over pulmonary area  murmur is described as being like machinery in quality, starts just after S1 and ends in the late diastole
  52. 52. X-ray chest  prominent pulmonary artery with increased pulmonary vascular markings.  the left atrium and left ventricle enlarged
  53. 53. Echo  left atrial and left ventricular dimensions are increased.  The ductus can easily be visualized directly and its size estimated  Color Doppler examinations demonstrate systolic or diastolic (or both) retrograde turbulent flow in the pulmonary artery, and aortic retrograde flow in diastole
  54. 54. Catheterization  In patients with atypical findings  Demonstrate increased pressure in the right ventricle and pulmonary artery  presence of oxygenated blood shunting into the pulmonary artery confirm diagnosis
  55. 55.  Indomethacin: for uncomplicated PDA in preterm neonates  Surgical ligation : secondary option for uncomplicated PDA in preterm, term, infants, and children; first option for complicated PDA  Catheter device closure: uncomplicated PDA in child
  56. 56.  Narrowing in the aorta causing obstruction to flow usually below origin of Lt subclavian artery at the origin of ductus (juxtaductal)  Infantil type→ coarctation with arch hypoplasia (sever form)  Adult type → isolated juxtaductal (mild form)  M to F ratio is 2:1  It may be a feature of Turner syndrome
  57. 57. aortic obstruction leading to: 1.High pressure in proximal part of the aorta → Lf ventricular hypertrophy and ↑↑ blood pressure in the upper part of the body 2.Low pressure in distal part of the aorta → low blood pressure in the lower part of the body
  58. 58. 3. Collateral circulation development 4. Sever coarctation+ PDA → differential cyanosis
  59. 59.  Usually asymptomatic during infancy and childhood  Heart failure in sever condition  Manifestations of hypertension e.g., headache, epistaxis, cerebral hemorrhage.  Differential cyanosis
  60. 60.  Weak or absent femoral pulsation  Prominent radial pulsation  Radial femoral delay →femoral pulse felt after radial pulse  Blood pressure in LL lower than in UL  Lt ventricular hypertrophy
  61. 61.  Ejection systolic murmur best heard in the left infrascapular area  a systolic ejection click or thrill in the suprasternal notch suggests a bicuspid aortic valve (present in 70% of cases).  Mid diastolic murmur at the apex of MS if present
  62. 62.  murmur of mild aortic stenosis can be heard in the 3rd right intercostal space  In older patients with well-developed collateral blood flow, systolic or continuous murmurs may be heard over the left and right sides of the chest laterally and posteriorly
  63. 63.  Neonates or infants with more severe coarctation: 1.Initially have signs of lower body hypoperfusion 2.Acidosis 3.Severe heart failure. 4. On physical examination, the heart is large, and a systolic murmur is heard along the left sternal border with a loud 2nd heart sound.
  64. 64.  These signs may be delayed days or weeks until after closure of the ductus arteriosus.  If detected before ductal closure, patients may exhibit differential cyanosis, best demonstrated by simultaneous oximetry of the upper and lower extremities
  65. 65. X-ray  Lt ventricular hypertrophy  Rib notching  Enlarged left subclavian artery→ a prominent shadow in the left superior mediastinum.
  66. 66. ECG  Evidence of left ventricular hypertrophy in older patients.  Neonates and young infants display right or biventricular hypertrophy.
  67. 67. Echo  The segment of coarctation can be visualized  Associated anomalies of the mitral and aortic valve can also be demonstrated CT, MRI  valuable noninvasive tools for evaluation of coarctation when the echocardiogram is equivocal. Cardiac catheterization with selective left ventriculography and aortography  is not usually required before surgery
  68. 68.  In neonates with severe coarctation of the aorta 1.prostaglandin E1 infusion 2. surgical repair→ Once a diagnosis has been confirmed and the patient stabilized
  69. 69.  Older infants with heart failure but good perfusion 1.anticongestive measures 2.surgical intervention
  70. 70.  Surgical repair should be as soon as possible because delay lead to less successful operation because of decreased left ventricular function and degenerative changes in the aortic wall.

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