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pediatrics congenital heart disease

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pediatrics congenital heart disease

  1. 1. PEDIATRICS CONGENITAL HEART DISEASE Jimma university School of pharmacy Getandale zeleke[B.pharm,clinical pharmacy,MSC,pg2 ] 1 getandale@gmail.com; getandale.zeleke@ju.edu.et
  2. 2. Presentation outline  Introduction  The epidemiology  Classification of congenital heart disease  Pathophysiology  Clinical presentation and physical findings of common CHD  management 2
  3. 3. INTRODUCTION 3
  4. 4. Foetal circulatory system 4
  5. 5. Foetal Circulation Arterial blood leaves the placenta via the umbilical vein This branches and delivers blood to the IVC by way of the ductus venosus Blood then goes into the right atrium, 30% goes across the foramen ovale, the rest to the RV then to PA Instead of going to the lungs, 85% goes through the PDA to the aorta
  6. 6. INTRODUCTION 6  Is a defect in the structure or function of the heart and great vessels which is present at birth.  Congenital heart disease (CHD) is the most common congenital disorder in newborns.  It is not static ,there is always a continuous anatomical or physiological change
  7. 7. Epidemiology and Genetic Basis of Congenital Heart Disease  Congenital heart disease occurs in approximately 0.8% of live births.  The incidence is higher in stillborns (3-4%), spontaneous abortuses (10-25%), and premature infants.  In preterm infants, CHD is two to three times that found in term infants.  about 2-3 in 1,000 newborn infants will be symptomatic with heart disease in the 1st year of life  With advances in both palliative and corrective surgery, the number of children with CHD surviving to adulthood has increased dramatically.  Despite these advances, CHD remains the leading cause of death in children with congenital malformations. 7
  8. 8. Prevalence of Congenital heart Disease (chart review) DevelopedDeveloped countriescountries Ethio-SwedishEthio-Swedish TAHTAH VSDVSD 28.3%28.3% 4141 28.4%28.4% PDAPDA 12%12% 13%13% 16.5%16.5% ASDASD 10.7%10.7% 13.6%13.6% 13%13% COACOA 8.8%8.8% <2%<2% 1.8%1.8% TOFTOF 7%7% 9%9% 5.7%5.7% PSPS 6%6% 9.9%9.9% 8.8%8.8% ASAS 2.3%2.3% 3.5%3.5% 3.4%3.4%
  9. 9. Etiology  The cause of most congenital heart defects is unknown.  thought to be multifactorial and result from a combination of genetic predisposition and environmental stimulus  CHD are related to chromosomal abnormalities, in particular, trisomy and Turner syndrome.  Third Pregnancy 20-30%  The risk of recurrence of CHD increases if a 1st-degree relative (parent or sibling) is affected. 9
  10. 10.  Of all cases of CHD, 2-4% are associated with known environmental or adverse maternal conditions and teratogenic influences, including maternal diabetes mellitus, phenylketonuria, or systemic lupus erythematosus; congenital rubella syndrome;  and maternal ingestion of drugs (lithium, ethanol, warfarin, thalidomide, antimetabolites, vitamin A derivatives, anticonvulsant agents. 10
  11. 11.  Gender differences in the occurrence of specific cardiac lesions have been identified.  Transposition of the great arteries and left-sided obstructive lesions are slightly more common in boys (≈65%),  whereas atrial septal defect, VSD, PDA, and pulmonic stenosis are more common in girls. 11
  12. 12. Evaluation of the newborn
  13. 13. Recognition of Cyanosis Cyanosis = blue color of skin and mucous membranes caused by reduced oxygen content oxygen content of blood depends upon: Hgb level oxygen saturation blood flow cyanosis usually noted when Sat’s <86% cyanosis more difficult to see in anemia 13
  14. 14. Central cyanosis • noted in the trunk, tongue, mucous membranes • due to reduced oxygen saturation Peripheral cyanosis • noted in the hands and feet, around mouth • due to reduced local blood flow Recognition of Cyanosis
  15. 15. Acyanotic Congenital Heart Disease ↑Pulmanary blood flow Left-to-Right Shunt Lesions  Patent Ductus Arteriosus (PDA)  Atrial Septal Defect (ASD)  VentricularSeptal Defect (VSD)  Atrioventricular Septal Defect (AV Canal)  The pathophysiologic common denominator in this group is communication between the systemic and pulmonary sides of the circulation, which results in shunting of fully oxygenated blood back into the lungs
  16. 16.  The direction and magnitude of the shunt across such a communication depend on the size of the defect, the relative pulmonary and systemic pressure and vascular resistances.  The increased volume of blood in the lungs decreases pulmonary compliance and increases the work of breathing.  Fluid leaks into the interstitial space and alveoli and causes pulmonary edema.  The infant acquires the symptoms we refer to as heart failure, such as tachypnea, chest retractions, nasal flaring, and wheezing. 17
  17. 17. Patent Ductus Arteriosus  PDA – Persistence of the normal fetal vessel that joins the Pulmonary artery to the Aorta.  Normally closes in the 1st wk of life.  Female:Male ratio of 2:1  associated with maternal rubella infection during early pregnancy  PDA is a common problem in premature infants.
  18. 18. Pathophysiology  As a result of the higher aortic pressure postnatally, blood shunts left to right through the ductus, from the aorta to the pulmonary artery.  The extent of the shunt depends on the size of the ductus and on the ratio of pulmonary to systemic vascular resistance.  If the PDA is small, pressures within the pulmonary artery, the right ventricle, and the right atrium are normal.  If the PDA is large, pulmonary artery pressure may be elevated to systemic levels during both systole and diastole 19
  19. 19. Patent Ductus Arteriosus Hemodynamics  As a result of higher aortic pressure, blood shunts L to R through the ductus from Aorta to PA.
  20. 20. Clinical Manifestations  A small PDA is usually asymptomatic.  tachycardia  Shortness of breath  Retardation of physical growth.  Characteristic systolic-diastolic murmur at the base of the heart with maximum in the PA 21
  21. 21. Patent Ductus Arteriosus  Classic continuous machine-like murmur  It begins soon after onset of the 1st sound, reaches maximal intensity at the end of systole, and wanes in late diastole.  prominent apical impulse enlarged heart,  Left subclavicular thrill  Bounding pulse Clinical Signs & SymptomsClinical Signs & Symptoms
  22. 22. • pulmonary vascularity is increased; • enlargement of left sided heart Chest X-ray
  23. 23. THERAPEUTIC INTERVENTIONS Interventions for PDA closure include: Pharmacologic therapy, which is used exclusively in premature infants Surgical ligation Percutaneous catheter occlusion Mortality is < 1%
  24. 24. Pharmacologic therapy Inhibitors of prostaglandin synthesis, such as indomethacin and ibuprofen , are used as the initial interventions for PDA closure in preterm infants. Indomethacin -0.2 mg/kg Ibuprofen -10-5-5 mgkg ibuprofen is as effective as indomethacin in closing a PDA and currently appears to be the drug of choice. Ibuprofen reduces the risk of NEC and transient renal insufficiency. Oro-gastric administration of ibuprofen appears as effective as iv administration 25 Cochrane Database Syst Rev. 2015 Feb 18;(2)
  25. 25.  Paracetamol is as effective as indomethacin and ibuprofen in closure of PDA in preterm neonates and has less side effects mainly on renal function, platelet count, and GIT bleeding. 26 Eur J Pediatr. 2017 Feb;176(2):233- 240 Trials. 2016 Apr 2;17:182
  26. 26. Surgery  Irrespective of age, patients with PDA require surgical or catheter closure  The surgical approach and technique are dependent upon the size and age of the patient  The following complications are associated with surgical closure Recurrent laryngeal nerve paralysis Respiratory compromise Infection Intracranial hemorrhage in preterm infants 27
  27. 27. Percutaneous closure    provides an alternative to surgical ligation in patients beyond infancy  Access is generally achieved through the femoral artery or vein.  Closure of the PDA with coil or device without thoracotomy 28
  28. 28. Indications for PDA closure  PDA closure for patients with a significant left-to-right shunt who are symptomatic, have evidence of left cardiac overload (ie, left atrial or ventricular enlargement), or have mild to moderate pulmonary arterial hypertension (PAH) .  for patients with a previous episode of endocarditis regardless of the size of PDA in the absence of severe PAH 29
  29. 29. Atrial Septal Defect ASD is an opening in the atrial septum permitting free communication of blood between the atria  Atrial septal defects (ASDs) can occur in any portion of the atrial septum (secundum, primum, or sinus venosus), depending on which embryonic septal structure has failed to develop normally 30
  30. 30. There are 3 majortypes: Se cundum ASD – at the Fossa Ovalis, accounts for 70 to 75 percent of all ASDs,most common. • Prim um ASD – lower in position accounts for 15 to 20 percent of ASDs. • Sinus Ve no sus ASD – high in the atrial septum, the least common. 31
  31. 31. Atrial Septal Defect  Secundum ASD  Sinus Venosus ASD
  32. 32. In normal, the chambers of the left side of the heart are higher pressure than of the right side;
  33. 33.  In the case of a large ASD (>9mm), may result in left-to- right shunt, blood will shunt from the LA to the RA.  This extra blood may cause a volume overload of both the right atrium and the right ventricle.  Ultimately the RV must push out more blood than the LV due to the L-to-R shunt.  This condition can result in eventually RV-failure (dilatation and decreased systolic function) and Pulm Htn. 34
  34. 34. Atrial septal defect with left-to-right shunt  When the pressure in the RA rises to the level in the LA, the left-to-right shunt will diminish or cease.  When the pressure in the RA to be higher than the pressure in the LA and will reverse the shunt → right-to-left shunt will exist (this phenomenon is known as Eisenmenger’s syndrome).
  35. 35. Clinical Signs & Symptoms •Most infants with ASDs are asymptomatic. •They may present at 6 to 8 weeks of age with a soft systolic ejection murmur •Hyperactive precordium, RV heave, fixed widely split S2. •Mid-diastolic murmur heard over LLSB. •Children with large left-to-right shunts are likely to complain of some fatigue and dyspnea. •Growth failure is very uncommon 36
  36. 36. Clinical Signs & Symptoms • Rarely presents with signs of CHF or other cardiovascular symptoms. • Most are asymptomatic but may have easy fatigability or mild growth failure. • Cyanosis does not occur unless pulmonary HTN is present. 37
  37. 37. Radiologic Features Echo-CG: RV is enlarged, defect is visualized The heart is usually enlarged, with a cardiothoracic ratio >0.5 chest X-ray: pulmonary vascularity is increased 38
  38. 38. NATURAL HISTORY  Secundum ASDs can close spontaneously, remain unchanged, or enlarge.  Spontaneous closure, or a decrease in size, is most likely to occur in defects less than 7 to 8 mm in diameter and with younger age at diagnosis 39
  39. 39. TREATMENT  The treatment for isolated secundum ASD is closure of the defect, which can be achieved by a surgical or percutaneous transcatheter approach. Which ASDs should be closed? The majority of isolated secundum ASDs <6 mm diameter in infants close spontaneously by 2 years, and some as late as 5 years of age. Thus, in the absence of associated symptoms, early closure is not indicated for these defects. Defects of moderate size (at least 6 to 8 mm in diameter) and larger are relatively unlikely to close spontaneously. 40
  40. 40.  However, closure of even moderate and large isolated secundum ASDs is not recommended in asymptomatic patients before 2 years of age due to the possibility, however small, of spontaneous closure.  The accepted clinical standard is to repair Surgical or catherization closure for secundum ASD w/ a Qp:Qs ratio >2:1  Surgical correction is done earlier in children w/ CHF or significant Pulm HTN.  prophylactic antibiotics are recommended for dental and respiratory tract procedures during the first six months after the repair. 41
  41. 41. Ventricular Septal Defect  VSD– is an abnormal opening in the ventricular septum, which allows free communication between the Rt & Lt ventricles.  Accounts for 25% of CHD. 42
  42. 42. Ventricular Septal Defect  During systole some of the blood from the LV leaks into the RV, passes through the lungs and reenters the LV via the pulmonary veins and LA.  Such circuitous route of blood causes volume overload on the LV.  The LV normally has a much higher systolic pressure (~100 mm Hg) than the RV (~85 mm Hg) and through VSD blood leaks into the RV and elevates RV pressure and volume, causing PulmHTN.
  43. 43. 4 Types Perimembranous (or membranous) – Most common(80%). defect lies in the outflow tract of the left ventricle immediately beneath the aortic valve Muscular VSD – Defects in the muscular septum are frequently multiple and make up 5% to 20% of defects found at surgery Infundibular (subpulmonary VSD) – involves the RV outflow tract. Defects in the outflow tract of the right ventricle beneath the pulmonary valve AVSD – inlet VSD, posterior and inferior to the membranous defect, beneath the septal leaflet of the tricuspid valve almost always involves AV valvular abnormalities 44
  44. 44. Clinical Signs & Symptoms  Small - moderate VSD, 3-6mm, are usually asymptomatic and 50% will close spontaneously by age 2yrs.  Moderate – large VSD, almost always have symptoms and will require surgical repair.  harsh holosystolic murmur heard along the LSB, more prominent with small VSD, may be absent with a very Large VSD  Prominent P2, Diastolic murmur.  CHF, FTT, Respiratory infections, exercise intolerance  hyperactive precordium.  Symptoms develop between 1 – 6months 45
  45. 45.  If the defect is large and pulmonary vascular resistance is not significantly elevated (L-t-R-shunt) – growth failure, CHF, repeated lower respiratory tract infections (begin at 1-2month);  If the defect is large and pulmonary vascular resistance is very high (Eisenmenger’s reaction) – shortness of breath, dyspnea on exertion, chest pain, cyanosis; 46
  46. 46. Chest X-rayChest X-ray:: • increased pulmonaryincreased pulmonary vascularity,vascularity, • enlargement of theenlargement of the LA and LV;LA and LV; Ventricular Septal Defect
  47. 47. CXR: progressive dilatation of heartCXR: progressive dilatation of heart.. • cardiomegalycardiomegaly • increasedincreased pulmonarypulmonary vascularity,vascularity, • enlargementenlargement of the LA andof the LA and LVLV;;
  48. 48. Treatment: Medical Therapy  Children with small VSDs are asymptomatic and have excellent long-term prognosis.  Neither medical therapy nor surgery is indicated.  Bacterial endocarditis prophylaxis is indicated  If children with moderate or large VSDs develop symptomatic congestive heart failure,a trial of medical therapy is indicated.  Furosemide is used in a dosage of 1 to 3 mg/kg/day divided into two or three doses  Systemic afterload reduction with enalapril (initial dosage of 0.1 mg/kg/24 hours divided into twice daily, gradually increasing to 0.5 mg/kg/24hours divided into twice daily dose, maximum of 40 mg/day) 49
  49. 49. Indications forSurgical Closure: Large VSD w/ medically uncontrolled symptomatology & continued FTT(failure to thrive). Ages 6-12 mo w/ large VSD & Pulm. HTN Age > 24 mo w/ Qp:Qs ratio > 2:1. Supracristal VSD of any size, secondary to risk of developing AV insufficiency 50
  50. 50. Acyanotic Congenital Heart Disease Obstruction to blood flow from ventricles  Coarctation of the Aorta  Pulmonary Stenosis  Aortic Stenosis 51
  51. 51. Coarctation of the Aorta  Coarctation-  Coarctation of the aorta is a narrowing of the descending aorta, which is typically located at the insertion of the ductus arteriosus.  occurs in approximately 6% to 8% of patients with congenital heart disease  coarctation occurs more commonly in males than in females.  The genetic component to coarctation has long been recognized in the Turner syndrome, in which about 35% of patients are affected
  52. 52. PATHOGENESIS AND ETIOLOGY Congenital. The vast majority of coarctation cases are congenital. the two main theories for the development of congenital coarctation of the aorta are:  Reduced antegrade intrauterine blood flow causing underdevelopment of the fetal aortic arch.  Migration or extension of ductal tissue into the wall of the fetal thoracic aorta There is also increasing evidence of a vascular wall defect in the ascending aorta of individuals with congenital coarctation of aorta 53
  53. 53. Acquired  In addition to a congenital etiology, aortic narrowing can be an acquired abnormality due to inflammatory diseases of the aorta, such as Takayasu arteritis or, rarely, severe atherosclerosis the former disorder, the midthoracic or abdominal aorta is often the site of involvement 54
  54. 54. Coarctation of the Aorta Hemodynamics  Obstruction of left ventricular outflow ⇒ LV afterload increases ⇒ pressure hypertrophy of the LV.
  55. 55. Feel the pulses especially brachial and femoral Clinical Signs &Clinical Signs & SymptomsSymptoms • Classic signs of coarctation are diminution or absence of femoral pulses.. • Pulse discrepancyPulse discrepancy between rt & lt arms.between rt & lt arms. Coarctation of the Aorta
  56. 56. Clinical Signs & Symptoms Higher BP in the upper extremities as compared to the lower extremities. 90% have systolic hypertension of the upper extremities. Pulse discrepancy between rt & lt arms. 57
  57. 57. ….  Sings of low cardiac output, poor peripheral perfusion – Lower extremities hypoperfusion, acidosis, HF and shock  Differential cyanosis if ductus is still open  systolic ejection murmur @ LSB.  Cardiomegaly, rib notching on X-ray 58
  58. 58. Coarctation of the Aorta
  59. 59. Treatment  untreated, coarctation of the aorta has a poor natural history.  natural history data for untreated coarctation documented a mean age at death of 34 years,75% of patients died by 46 years of age.  The most common causes of death were congestive heart failure (26%), aortic rupture (21%), bacterial endocarditis (18%), and intracranial hemorrhage (12%) 60
  60. 60. Cont…  Management decisions for patients with coarctation of the aorta depend upon patient age, presentation, and the severity of the lesion.  Critical coarctation in infancy: Infants with severe (“critical”) coarctation are at-risk for developing heart failure and death when the ductus arteriosus closes.  Identification of these patients is essential in order to maintain patency of the ductus prior to surgical repair.  immediate treatment is required to stabilize patients with heart failure. 61
  61. 61. Medical therapy consists of the following: Continuous intravenous infusion of prostaglandin E1 (also known as alprostadil ) to keep the ductus arteriosus open. Dopamine and/or dobutamine to improve contractility in those with heart failure. Supportive care to correct metabolic acidosis, hypoglycemia, respiratory failure, and anemia that may contribute to or be a consequence of heart failure. The introduction of prostaglandin E1 ( alprostadil) has significantly decreased the mortality in neonates with critical coarctation Once the patient is stabilized, surgical repair can be performed. 62 Pediatr Cardiol. 2004 Jan- Feb;25(1):49-52. Pediatr Cardiol. 2006 Nov- Dec;27(6):766-8
  62. 62. Surgical Repair  Surgical repair remains the conventional treatment for most children with coarctation of the aorta.  The types of surgical repair of coarctation include: Resection with end-to-end anastomosis Subclavian flap aortoplasty in infants with long-segment coarctation A bypass graft across the area of coarctation when the distance to be bridged is too long for an end-to-end repair Prosthetic patch aortoplasty, which is avoided whenever possible because of the frequent occurrence of aortic aneurysm or rupture 63
  63. 63. Balloon angioplasty Balloon angioplasty is a noninvasive alternative to surgical repair for older infants (greater than four months of age) and young children with native discrete coarctation.  It remains the preferred intervention for all patients with isolated recoarctation regardless of age 64
  64. 64. Pulmonary Stenosis  Pulmonary Stenosis is obstruction in the region of either the pulmonary valve or the subpulmonary ventricular outflow tract.  Accounts for 7-10% of all CHD.  Most cases are isolated lesions  Can present w/ or w/o an intact ventricular septum  Maybe biscuspid or fusion of 2 or more leaflets. 65
  65. 65. Hemodynamics  RV pressure hypertrophy ⇒ RV failure.  RV pressures maybe > systemic pressure.  Post-stenotic dilation of main PA.  W/intact septum & severe stenosis ⇒ R-L shunt through PFO ⇒ cyanosis.  Cyanosis is indicative of Critical PS. 66
  66. 66. Clinical Signs & Symptoms  Most patients with valvar pulmonary stenosis are asymptomatic  Depends on the severity of obstruction.  Asymptomatic w/ mild PS < 30mmHg.  Mod-severe: 30-60mmHg, > 60mmHg Prominent jugular a-wave, RV lift Split 2nd hrt sound w/ a delay Ejection click, followed by systolic murmur. Heart failure & cyanosis seen in severe cases. 67
  67. 67. Treatment  Mild PS no intervention required, close follow-up.  Mod-severe – require relieve of stenosis.  Balloon valvuloplasty, treatment of choice.  Surgical valvotomy is also a consideration 68
  68. 68. Balloon Dilatation of Pulmonic Valve
  69. 69. Aortic Stenosis  Aortic Stenosis is an obstruction to the outflow from the left ventricle at or near the aortic valve that causes a systolic pressure gradient of more than 10mmHg. Accounts for 7% of CHD.  more dangerous lesion compared to PS 3 Types  Valvular – Most common.  Subvalvular(subaortic) – involves the left outflow tract.  Supravalvular – involves the ascending aorta is the least common. 70
  70. 70. Clinical Signs & Symptoms  Mild AS may present with exercise intolerance, easy fatigabiltity, but usually asymptomatic.  Moderate AS – Chest pain, dypsnea on exertion, dizziness & syncope.  Severe AS – Weak pulses, left sided heart failure, Sudden Death. 71
  71. 71.  LV thrust at the Apex.  Systolic thrill @ Rt base/suprasternal notch.  Ejection click, systolic murmur @ RSB/LSB w/ radiation to the carotids. 72
  72. 72. Treatment  Because surgery does not offer a cure it is reserved for patients with symptoms and a resting gradient of 60- 80mmHg.  For subaortic stenosis it is reserved for gradients of 40- 50mmHg because of it’s rapidly progressive nature.  Balloon valvuloplasty is the standard of treatment. 73
  73. 73. .  Aortic insufficiency & re-stenosis is likely after surgery and may require valve replacement.  Activity should not be restricted in Mild AS.  Mod-severe AS, no competitive sports 74
  74. 74. Cyanotic Congenital Heart Disease  There has to be a RIGHT to LEFT shunt to cause cyanosis.  Congenital heart disease produces cyanosis when obstruction to right ventricular outflow causes intracardiac right-to-left shunting.  Complex anatomic defects cause an admixture of pulmonaryComplex anatomic defects cause an admixture of pulmonary and systemic venous return in the heartand systemic venous return in the heart  Decreased pulmonary blood flowDecreased pulmonary blood flow  Chest X-RayChest X-Ray Decreased pulmonary vascular markingsDecreased pulmonary vascular markings 75
  75. 75. Tetralogy of Fallot Most common cyanotic heart disease. The four abnormalities include: Pulmonary stenosis RVH VSD Overriding Aorta Signs include cyanosis, murmur, squatting and spells.  76
  76. 76. Pathophysiology: Increased resistance by the pulmonary stenosis causes deoxygenated systemic venous return to be diverted from RV, through VSD to the overriding aorta and systemic circulation systemic hypoxemia and cyanosis 77
  77. 77. Clinical Signs & Symptoms Symptoms are variable depending of degree of obstruction  Cyanosis – is variable (isn’t present at the birth, occurs later in the 1st yr of life)  Tet spells(Hypercyanotic ): irritability, cyanosis, hyperventilation and sometimes syncope or convulsions due to cerebral hypoxemia  Severe dyspnea on exertion  Tachycardia  Mental retardation  Retarded growth and development  RV heave  Systolic ejection murmur is heard along the left sternal border 78
  78. 78. Chest X-RayChest X-Ray • DecreasedDecreased pulmonary vascularpulmonary vascular markingmarking • ““Boot-shapedBoot-shaped heart”heart”
  79. 79.  Treatment of tetralogy of Fallot depends on the severity of the right ventricular outflow tract obstruction.  Infants with severe tetralogy require urgent medical treatment and surgical intervention in the neonatal period.  Decrease cardiac workload  Hypercyanotic “tet” spells may require rapid and aggressive treatment that starts with placement of the patient in a knee-chest position to increase systemic vascular resistance, which promotes movement of blood from the right ventricle into the pulmonary circulation rather than the aorta. 80
  80. 80.  This may be followed by more aggressive therapy of intravenous morphine and a fluid bolus. The mechanism of action of morphine is unclear, while fluids improve RV filling and pulmonary flow.  If the above measures fail, intravenous beta blockers (eg, propranolol or esmolol) can be administered. The goal is to control the magnitude of the right to left intracardiac shunt, which is increased by: Decreased SVR Increased PVR Increased myocardial contractility  Surgical closure of the VSD and enlargement of the pulmonary outflow tract. 81
  81. 81. d-Transposition of the Great Arteries
  82. 82.  The aorta arises from the right ventricle and the pulmonary artery from the left.  The mixing of the blood occurs at the PFO and the PDA. The signs include cyanosis and cardiomegaly.  Reverse differential cyanosis!  There may be no murmur.  An echocardiogram is diagnostic. 83
  83. 83. d-Transposition of the Great Arteries  Pathophysiology  Cyanosis due to failure of delivery of pulmonary venous blood to the systemic circulation  Two parallel circulations with no mixing  Open atrial septum (fossa ovalis) allows some left-to-right shunt, enhanced by a left-to-right ductus arteriosus shunt  Presence of ventricular septal defect facilitates mixing 84
  84. 84.  Heart failure often present.  Cardiac enlargement and diminished pulmonary artery segment on x-ray.  Untreated, the vast majority of these infants would not survive the neonatal period. 85
  85. 85. Clinical Manifestations  Cyanosis, tachypnea are most often recognized within the 1st hrs or days of life.  Hypoxemia is usually moderate to severe, depending on the degree of atrial level shunting and whether the ductus is partially open or totally closed.  Physical findings, other than cyanosis, may be remarkably nonspecific.  Murmurs may be absent, or a soft systolic ejection murmur may be noted at the midleft sternal border. 86
  86. 86.  Chest film  Oval-shaped heart  Narrow mediastinum  Normal or increased pulmonary vascular markings  This condition is a medical emergency, and only early diagnosis and appropriate intervention can avert the development of prolonged severe hypoxemia and acidosis, which lead to death 87
  87. 87. Treatment When transposition is suspected, an infusion of prostaglandin E1 (0.05 microg/kg per minute) should be initiated immediately to maintain patency of the ductus arteriosus and improve oxygenation. Infants who remain severely hypoxic or acidotic despite prostaglandin infusion should undergo balloon atrial septostomy 88
  88. 88. . 89
  89. 89.  . 90
  90. 90. prophylaxis for infective endocarditis  The recommendation for antimicrobial prophylaxis for dental and other procedures (and others) is now limited to those patients with cardiac conditions with the highest risk of IE  Today, antibiotics before dental procedures are only recommended for patients with the highest risk of IE, those who have:  Cyanotic congenital heart disease, that has not been fully repaired, including children who have had a surgical shunts and conduits.  A congenital heart defect that's been completely repaired with prosthetic material or a device for the first six months after the repair procedure.  Repaired congenital heart disease with residual defects, such as persisting leaks or abnormal flow at or adjacent to a prosthetic patch or prosthetic device. 91
  91. 91.  prosthetic heart valve or who have had a heart valve repaired with prosthetic material.  A history of endocarditis.  A heart transplant with abnormal heart valve function . 92 Circulation 2007; 116:1736
  92. 92. Preventing Birth Defects  Stop smoking  Avoid drinking alcohol while pregnant  Take a daily vitamin containing folic acid  Check with your doctor to make sure any medication (over- the-counter or prescription) is safe to take during pregnancy  Stop use of any illegal or "street" drugs
  93. 93. References  Moss and Adams; Heart Disease in Infants Children and Adolescents,7th edition.  Nelson Textbook of Paediatrics 20th edition.  Uptodate 21.2  Primary literatures mentioned in the body of this document. 94
  94. 94. The END Are we going to use acetaminophen for PDA closer?
  95. 95. Thank you for attention!

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