Congenital heart disease 2013

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CHD for general pediatrician.

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Congenital heart disease 2013

  1. 1. Congenital heart disease (Formulation of the problem) Antonio Souto acasouto@bol.com.br Médico coordenador Unidade de Medicina Intensiva Pediátrica Unidade de Medicina Intensiva Neonatal Hospital Padre Albino Professor de Pediatria nível II Faculdades Integradas Padre Albino Catanduva / SP
  2. 2. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Congenital heart diseases are a dynamic group of anomalies that originate in fetal life and change considerably during postnatal development. Routine neonatal examination fails to detect more than half of babies with heart disease; examination at 6 weeks misses one third.
  3. 3. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Early recognition, urgent identification and timely referral to a pediatric cardiologist and timely intervention has great implications in prognosis, is the key in reducing mortality and morbidity.
  4. 4. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Congenital heart disease in the newborn requiring early intervention ???? Life threatening heart diseases may not have obvious evidence early after birth, the diagnosis is difficult sometimes and always a great concern to pediatricians. High index of suspicion is essential to decision making.
  5. 5. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical Presentation of CHD in the Neonate • Fetal Diagnosis • Cyanosis • CHF/Shock/Circulatory Collapse • Arrhythmia • Asymptomatic Heart Murmur
  6. 6. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Formulation of the problem ? a great concern to pediatricians
  7. 7. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Classification of CHD (clinical point of view) 1.Life-threatening CHD -Cardiovascular collapse is likely and compromised if not treated early Transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR).
  8. 8. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Classification of CHD (clinical point of view) 2. Clinically significant CHD -Cardiac malformations that have effects on heart function but where the collapse is unlikely to be need early intervention. Ventricular septal defect (VSD), complete atrioventricular septal defect (AVSD), atrial septal defect (ASD) and tetralogy of Fallot (TOF) with good pulmonary artery anatomy. 3. Clinically non-significant CHD -No functional and clinical significance. Small VSD, atrial septal defect (ASD), mild pulmonary stenosis (PS).
  9. 9. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino • Total Anomalous Pulmonary Veins • Tetrology of Fallot • Tricuspid Atresia • Transposition • Truncus Arteriosus 55 ““TT’’ss”” Most common cyanotic lesions of the newborn
  10. 10. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Cardiac malformations - 10% of infant mortality Most common lethal diagnosis: Left ventricular outflow tract obstruction •Hypoplastic left heart syndrome •Coarctation of aorta •Aortic stenosis
  11. 11. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino How do we improve the detection of babies with congenital heart disease ? •Understanding normal changes at birth •Simple way to think about congenital heart disease •Systematic examination of the cardiovascular system •Simple additional screening •Robust care pathways
  12. 12. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Pulse Oximetry •easy to use, harmless when done correctly •accuracy of 2% in the range of 70 to 100% •consider cyanotic when Sat <94% at 24 hours of age •should be obtained prior to discharge from nursery = Policy at Sanford Children’s Measure sat in foot If <95%, gets evaluation
  13. 13. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino FLUID DYNAMICS The function of the human heart is that of a mechanical pump that receives the low pressure blood from the venous system and ejects it with higher pressure into the arterial system.
  14. 14. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnosis Applied clinical logic Heart and circulation Perfect harmony between structure and function Logical thought Gross morphology / physiologic derangements Clinical manifestation Accurate observation + Correct inferences
  15. 15. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Logical thought Gross morphology / physiologic derangements -Right side Start proximally (vena cavae) and end distally (pulmonary arteries) -Left side Start proximally (pulmonary veins) and end distally (Aorta) -What is the level of shunt? Acyanotic (left to right) Cyanotic (right to left) Atrial, ventricular, great artery
  16. 16. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino General Approach to CHD Patient 1. Define cardiovascular pathology 2. Predict pathophysiology 3. Determine hemodynamic goals 4. Anticipate emergency treatments
  17. 17. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Rosen: “any neonate in shock that does not respond to fluids or pressors has LV outflow obstruction until proven otherwise”
  18. 18. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Evaluation for and treatment of presumptive sepsis should be undertaken simultaneously with evaluation for cardiac and pulmonary disease.
  19. 19. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino What Are The Odds? • Congenital Heart Disease 8/1000 live births • “Critical” CHD 3/1000 live births • In the USA: ~ 32,000 children born/year with CHD ~ 11,000/year with “Critical” CHD
  20. 20. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Cardiac malformations - 10% of infant mortality Most common lethal diagnosis: Left ventricular outflow tract obstruction •Hypoplastic left heart syndrome •Coarctation of aorta •Aortic stenosis
  21. 21. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Left Ventricular Outflow Tract Obstruction Major source of neonatal M&M from CHD •Accounts for ~ 12% of congenital cardiac disease in infancy •~ 75% discharged from hospital w/o diagnosis •~ 65% - normal newborn screen examination •6% died before diagnosis •96% symptoms by 3 wks of life
  22. 22. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Relative Frequency of Lesions % • Ventricular septal defect 25-30 • Atrial septal defect 6-8 • Patent ductus arteriosus 6-8 • Coarctation of aorta* 5-7 • Tetralogy of Fallot 5-7 • Pulmonary valve stenosis 5-7 • Aortic valve stenosis * 4-7 • Transposition of great arteries 3-5 • Hypoplastic left ventricle * 1-3 • Hypoplastic right ventricle 1-3 • Truncus arteriosus 1-2 • Total anomalous pulm venous return 1-2 • Tricuspid atresia 1-2 • Double-outlet right ventricle 1-2 • Others 5-10
  23. 23. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  24. 24. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino •The neonatal myocardium has fewer myofibrils in a disordered pattern, making the myocardium stiffer. •The neonatal heart follows the Frank e Starling relationship but with a limited increase in stroke volume for a given increase in ventricular filling volume. •The neonatal myocardium is dependent on heart rate to increase cardiac output.
  25. 25. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino •Near peak of Starling curve •Stroke volume relatively fixed •C.O. relatively heart rate dependent
  26. 26. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  27. 27. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino 60%
  28. 28. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  29. 29. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Ductus Arteriosus •Shunt between the descending aorta to the left pulmonary artery •Open because low PaO2 and circulating prostaglandins (PGE2) •Ductus closes within the first days (24/48 h) of life in the term infant •Permanent closure due to fibrosis takes 4-6 weeks
  30. 30. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Ductus Arteriosus When patent ductus arteriosus (PDA) is opened widely, many serious malformations may not be noticed easily in the early life. Most of anomalies compatible with six months of intrauterine life permit live offspring at term (Fetal circulation)
  31. 31. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Ductal-dependent Heart Disease ? Inadequate systemic oxgenation / pulmonary blood flow due to heart disease • Inadequate pulmonary blood flow • Inadequate systemic delivery of oxygenated blood • Inadequate mixing
  32. 32. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Right sided obstruction
  33. 33. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Left sided obstruction
  34. 34. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Inadequate Mixing Survival Depends Upon Mixing Between Systemic and Pulmonary Circuits
  35. 35. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  36. 36. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  37. 37. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Ductus Arteriosus •Congenital heart disease in which either pulmonary or systemic blood flow is dependent on shunting through the ductus arteriosus. •Postnatally closure of the ductus arteriosus would be fatal, progress as severe acidosis/shock/cyanosis. •Prostaglandin E1 (PGE1 or Alprosdatil™) allow stabilization. •PGE1 must be started immediately after delivery.
  38. 38. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Prostaglandin E 1 •Always given as continous IV infusion. •Start at 0.05-0.1µg/kg/min, can be reduced to 0.005 - 0.01µg/kg/min once duct is opened •Efficacy ↓ with ↑ age, less effective after 2 weeks of life, not effective after 4 weeks •Continous cardiorespiratory monitoring
  39. 39. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Ductus Arteriosus •Before anatomic closure of the ductus arteriosus and foramen ovale, certain stresses can cause the newborn to revert to fetal circulation •Increased pulmonary vascular reactivity, raised PVR (Pulmonary Hypertension) and right-to-left shunting at the PFO and PDA, the clinical result is cyanosis. Hypothermia, hypercarbia, acidosis, hypoxia and sepsis can all cause a reversion to fetal circulation.
  40. 40. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  41. 41. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Cyanosis Chronically adapted to the hypoxia in the uterine life, newborn infants are able to tolerate some degree of cyanosis than older infants or children
  42. 42. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Typically, 2 g/dL of reduced hemoglobin 5g/dL of reduced Hb clinical cyanosis 35% 65% 25% 75%
  43. 43. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Cyanosis Category of cyanotic CHD decreased pulmonary flow with right to left shunting lesions (PA, TA with shunting at the atrial or ventricular level) poor mixing lesions (transposition physiology) right to left shunt with intra cardiac mixing lesions (TAPVR, single ventriclular physiology, truncus arteriosus).
  44. 44. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Formulation of the problem Basic questions 1.Is the patient acyanotic or cyanotic? 2.How is body/pulmonary arterial blood flow ? 3. Does the malformation originate in the left or right side of the heart? 4. Which is the dominant ventricule? 5. Is pulmonary hypertension present or not?
  45. 45. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  46. 46. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino • Commonly divided into acyanotic and cyanotic • 9 common conditions ACYANOTIC LEFT RIGHT SHUNTS Ventricular septal defect (30%) Patent ductus arteriosus (12%) Atrial septal defect (7%) OUTFLOW OBSTRUCTION Pulmonary stenosis (7%) Aortic stenosis (5%) Coarctation of the aorta (5%) CYANOTIC Tetralogy of Fallot (5%) Transposition of the great arteries (5%) Atrioventricular septal defect – complete (2%) Other complex – 20%
  47. 47. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino • Total Anomalous Pulmonary Veins • Tetrology of Fallot • Tricuspid Atresia • Transposition • Truncus Arteriosus 55 ““TT’’ss”” Most common cyanotic lesions of the newborn
  48. 48. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations -The clinical sign in the neonate may be vague For pediatricians: -identify the newborn “not doing well” •Persistent central cyanosis, unexplained acidosis, tachypnea without lung problems, etc. •Assessment of saturation monitoring, status of perfusion (blood gas analysis) and pulses/blood pressures in all extremities.
  49. 49. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Maternal Risk Factors • Congenital heart disease • Cardiac teratogen exposure – Lithium – Amphetamines – Alcohol – Anticonvulsants: phenytoin, valproic acid, carbamazepine, and trimethadione – Isotretinoin
  50. 50. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Maternal Risk Factors • Diabetes mellitus • PKU • Hyperthyroidism • Lupus, collagen vascular disease • Rubella, CMV, Coxsackie, Parvovirus
  51. 51. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Fetal Risk Factors • Trisomies, Turner’s syndrome, abnormal karyotype • Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus • Fetal arrhythmias • IUGR • Nonimmune hydrops • ?2 vessel cord
  52. 52. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations Dyspnea • Lung or heart problems? • Large shunt lesions:dyspnea, tachypnea, feeding difficulty, irritability and distress. • Ventilator weaning can be difficult in premature infants with large left to right cardiac shunts. Cyanosis with markedly reduced pulmonary blood flow usually leads to "quiet tachypnea”, without significant respiratory distress.
  53. 53. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations Sign of poor perfusion • Ductus dependent systemic circulatory ? • Progressive dyspnea, cold, clammy mottled skin, which indicates poor perfusion and acidosis, shock, oliguria • Cardiovascular collapse at the time of ductal closure •Shock in newborn ?
  54. 54. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations Cyanosis •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
  55. 55. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino central peripheral CAUSE ARTERIAL BLOOD DESATURATION OR ABNORMAL Hb CUTANEOUS VASOCONSTRICTION DUE TO LOW CO CONDITIONS Seen in R-L shunt, impaired pulmonary function, abnormal Hb exposure to cold air or water and abnormally greater extraction ofO2 from normally saturated blood SITES conjunctiva,palate,tongue, inner side of lips& cheeks limited to ears,nose,cheeks outer side of lips hands feet&digits certainly central if associated with clubbing and polycythemia, clubbing is absent probably central if it deepens on effort
  56. 56. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations Cyanosis • Pulmonary X cardiac problems ? • Persistent hypoxia refractory to 100% oxygen supply would indicate cyanotic CHD rather than pulmonary problems. • Hyperoxia test
  57. 57. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical manifestations Hyperoxia test arterial blood gas analysis while 100% oxygen • PaO2 > 220 mm Hg would suggest respiratory disease • PaO2 100‒220 mm Hg would require evaluation for cyanotic CHD • PaO2 < 100 mm Hg would suggest cyanotic CHD • PaO2 < 40‒50 mm Hg would be likely to have a poor mixing disease such as TGA
  58. 58. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino HYPEROXIA TEST GIVE 100% O2 ASSES PO2 PO2>200 PO2<150 NO CCHD CCHD PASS FAIL 150-200 ?CCHD WITH PBF OR PPHN
  59. 59. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Additional screeningAdditional screening •• PulsePulse oximetryoximetry –– PostPost ductalductal saturationssaturations –– < 95% warning< 95% warning signsign –– PULSOX studyPULSOX study DUCTDUCT RR handhand L Hand andL Hand and both feetboth feet
  60. 60. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino What information do we require? – 4 extremity BP’s – H & P • Murmurs • Organomegaly • Pulses • ECG • Labs, CXR findings, saturations
  61. 61. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino The “Noncardiac” Cardiac Exam • Vital signs, growth percentiles • UE/LE blood pressure & pulse oximetry • Color - cyanosis, pallor, mottling • Lungs - work of breathing, rate, equality, crackles • Abdomen - hepatomegaly, situs • Extremities - pulses, capillary refill time • Dysmorphic features, other organ system abnormalities
  62. 62. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Initial evaluation of child’s heart •Listen to heart first when/if infant quiet •First concentrate on S1 and especially S2 •Louder than normal? •Split normally? •Systolic murmur: •Diastolic murmur? •Widely radiating murmur? •Palpate liver •BP in arm and leg •Tongue - cyanosis
  63. 63. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Murmurs • Loudness graded 1-6. Presence of thrill > 4 • Timing – systolic/diastolic • Duration – ejection/mid/pansystolic • Site where loudest • Radiation
  64. 64. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Grading of murmurs • Grade 1: only a cardiologist can hear • Grade 2: murmur softer than S1/S2 • Grade 3: murmur louder than S1/S2 • Grade 4: thrill palpable • Grade 5: murmur audible with stethoscope partially off chest • Grade 6: murmur audible with stethoscope completely off chest
  65. 65. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnosis Chest x ray • Usually performed to rule out pulmonary disease as well as to evaluate pulmonary vascular marking and cardiomegaly. • Some CHD has characteristic features • Most of the serious CHD have no specific findings except vague cardiomegaly, change of pulmonary vascular marking and subtle finding of pulmonary venous congestion.
  66. 66. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnosis Electrocardiography EKG has been considered a useful tool in the diagnosis of CHD,especially if echocardiogram is not easily available. Ventricular maturation and associated ECG changes • The fetal heart is right-side dominant • Right axis deviation and R wave dominance in lead V1 and S wave dominance in lead V6. • At 3 e 6 months the classical left ventricular dominance pattern of adulthood is established as ventricular hypertrophy occurs in response to increased systemic vascular resistance.
  67. 67. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnosis Echocardiography Echocardiogram is the most valuable method in the diagnosis of CHD. • Identification of cardiac anatomy • Assessment of systolic ventricular function • Measurement of chamber dimensions and wall thickness • Assess the pressure gradients across the stenotic or regurgitation flow through the valves • Assess abnormal cardiac physiology • Flow in the descending aorta • Estimation of pulmonary arterial pressure • Defining the direction of flow when valve regurgitation and shunt exist
  68. 68. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnosis Cardiac Catheterization • The diagnostic frequency of cardiac catheterization is relatively decreasing especially in the neonate. • It is still the key in defining certain anatomic variants difficult to be delineated by echocardiography alone • Therapeutic catheterizations are considered as one of the life savingmodalities in some fields.
  69. 69. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Diagnostic ladder
  70. 70. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino •Clinical evaluation with CXR and Hyperoxia test excludes CHD in most cases. •Echocardiography recommended in all doubtful cases. •Prior stabilization and a monitored transport to tertiary center ensures a optimal pre-operative state. •Early intervention with very encouraging results is realistic for most forms of critical CHD in newborns
  71. 71. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Consultation: may be more cost- effective! 95% sens/spec for discriminating CHD from innocent murmur
  72. 72. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Hypercyanotic spells Cyanotic heart diseases • Tetralogy of Fallot • Pulmonary atresia • Transposition of great arteries • Tricuspid atresia
  73. 73. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino
  74. 74. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino • Sudden severe episodes of intense cyanosis caused by reduction of pulmonary flow • The level of cyanosis and onset of cyanotic spell is determined the SVR & level of PS component
  75. 75. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Clinical Presentation • peak incidence age: 3 to 6 months • often in the morning, can be precipitated by crying, feeding or defecation • severe cyanosis, hyperpnoea, metabolic acidosis • in severe cases, may lead to syncope, seizure, stroke or death • there is a reduced intensity of systolic murmur during spell
  76. 76. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Management • treat this as a medical emergency • knee-chest/squatting position: - place the baby on the mother’s shoulder with the knees tucked up underneath. - this provides a calming effect, reduces systemic venous return and increases systemic vascular resistance • administer 100% oxygen • give IV/IM/SC morphine 0.1 – 0.2 mg/kg to reduce distress and hyperpnoea
  77. 77. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Management • IV Propranolol 0.05 – 0.1 mg/kg • IV Esmolol 0.5 mg/kg slow bolus over 1 min, followed by 0.05 mg/kg/min for 4 mins. • volume expander, crystalloid, 20 ml/kg rapid IV push to increase preload • give IV sodium bicarbonate 1 mEq/kg to correct metabolic acidosis • heavy sedation, intubation and mechanical ventilation
  78. 78. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino • a single episode of hypercyanotic spell is an indication for early surgical referral (either total repair or Blalock Taussig shunt) • oral propranolol 0.2 – 1 mg/kg/dose 8 to 12 hourly should be started soon after stabilization while waiting for surgical intervention.
  79. 79. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Thomas-Blalock-Taussig Shunt Vivien Thomas, Partners of the Heart, 1998 and Something the Lord Made - Best Made-for-TV Movie, 2004 Helen Taussig Alfred Blalock Vivien Thomas
  80. 80. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino November 29, 1944 Thomas-Blalock-Tuassig
  81. 81. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Keep in your mind
  82. 82. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino •Routine neonatal examination fails to detect more than half of babies with heart disease •High index of suspicion is essential to decision making •“not doing well” •Any neonate in shock that does not respond to fluids or pressors has LV outflow obstruction until proven otherwise •If you think you have a ductal dependent lesion PGE1 must be started immediately (don’t be afraid of prostin)
  83. 83. Dr. Antonio Souto acasouto@terra.com.br 2013 UTI Pediátrica & Neonatal Hospital Padre Albino Thanks a lot!!!

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