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Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease

Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease. Introduction. Congenital heart disease occurs in 1% of live-born infants Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life

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Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease

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  1. Diagnosis and Early Management of the Infant with Suspected Congenital Heart Disease

  2. Introduction • Congenital heart disease occurs in 1% of live-born infants • Almost 1/2 of all cases of congenital heart disease are diagnosed during the 1st week of life • The most frequently occuring anomalies seen during the 1st week are: PDA, D-transposition of the great arteries, hypoplastic left heart syndrome, TOF, and pulmonary atresia

  3. Indications for Fetal Echocardiography

  4. Maternal Risk Factors Associated With Congenital Heart Disease • Congenital heart disease • Cardiac teratogen exposure • Lithium • Amphetamines • Alcohol • Anticonvulsants: phenytoin, valproic acid, carbamazepine, and trimethadione • Isotretinoin

  5. Maternal Metabolic Disorders or Infection • Diabetes mellitus • PKU • Hyperthyroidism • Lupus, collagen vascular disease • Rubella, CMV, Coxsackie, Parvovirus

  6. Fetal Risk Factors Associated With Congenital Heart Disease • 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

  7. Cyanosis • Etiology: CV, pulmonary, airway obstruction, neurological, neuromuscular, or hematological (methemoglobinemia or polycythemia) • Infants can appear cyanotic when the deoxygenated Hgb concentration is at least 3g/dL; it is not related to the percent saturated • 2 babies with sats of 80%: one with a hgb of 20g/dL and 4g/dL of desaturated hgb will be cyanotic, but an anemic infant with 10g/dL with 2g/dL deoxygenated hgb will not be cyanotic

  8. Evaluation • ABC’s • PE: murmur, pulses, precordium, respiratory status, HSM, color, capillary refill • 4 ext BPs: if SBP >10mmHg in right hand compared to lower ext, concerning for arch anomaly (though if normal may not rule it out) • Pre/post ductal saturations: if see a difference >5%, concerning for PPHN or left heart abnormalities

  9. Evaluation (Continued) • Hyperoxia test: baseline pre-ductal ABG when infant in room air, then repeat on 100% FiO2 • Reason for ABG and not just sats: with a saturation of 100%, you can have a PaO2 of 80 or 300; very different • CXR: cardiomegaly; normal, increased, or decreased pulmonary vascularity • EKG • Echo

  10. Specific Heart Disease Abnormalities

  11. Cyanotic With Decreased Pulmonary Blood Flow • Tetrology of Fallot • Ebsteins Anomaly • Tricuspid Atresia with PA or PS • Pulmonary atresia with intact septum • Critical pulmonic stenosis • PPHN

  12. Right Sided Obstructive Lesions • Cyanosis • No respiratory distress • Normal pulses and perfusion • Single second heart sound • Murmur • Moderate to marked hypoxemia • CXR: normal to large sized heart, decreased pulmonary blood flow (PBF)

  13. Tetralogy of Fallot

  14. Tetrology of Fallot

  15. Ebstein’s Anomaly

  16. Ebstein’s Anomaly

  17. Tricuspid Atresia

  18. Tricuspid Atresia

  19. EKG : QRS axis • Tricuspid atresia with PS or PA with intact ventricular septum: superior (0— -90) • Critical PS or PA : 0 to 90 degree quadrant • TOF and TOF with PA: 90-180 degree quadrant

  20. Cyanotic With Increased Pulmonary Blood Flow • d-Transposition of the great vessels • Truncus arteriosus • Total anomalous pulmonary venous return, above diaphragm • Single ventricle • Endocardial cushion defect

  21. Inadequate Mixing Lesions • Cyanosis • Mild tachypnea • Normal pulses • Single heart sound • Murmur • ABG: marked hypoxemia, + acidosis • CXR: cardiomegaly, normal or increased PBF

  22. Transposition of the Great Arteries

  23. d - Transposition of the Great Vessels

  24. Truncus Arteriosus

  25. TruncusArteriosus

  26. Lesions with Poor Gas Exchange • Cyanosis • Marked tachypnea • Fair perfusion, normal pulses • May or may not have a single heart sound • May or may not have a murmur • CXR: normal heart size, pulmonary congestion

  27. Total Anomalous Pulmonary Venous Return

  28. Total Anomalous Pulmonary Venous Return

  29. Left Sided Obstructive Lesions • Coarctation of aorta, interrupted aortic arch • Hypoplastic left heart syndrome • Aortic stenosis • Mitral stenosis • Total anomalous pulmonary venous return, below diaphragm

  30. Left Sided Obstructive Lesions • Grey or ashen color • Tachypnea • Poor perfusion • Decreased pulses/differential pulses • Single second heart sound • Murmur + gallop • Hepatomegaly • ABG: metabolic acidosis • CXR: cardiomegaly with increased PBF

  31. Coarctation of the Aorta

  32. Hypoplastic Left Heart Syndrome

  33. Hypoplastic Left Heart Syndrome

  34. Aortic Stenosis

  35. Acyanotic With Increased Pulmonary Blood Flow • VSD • ASD • PDA • Endocardial cushion defect

  36. Ventricular Septal Defect

  37. Ventricular Septal Defect

  38. Atrial Septal Defect

  39. Atrioventricular Canal

  40. Patent Ductus Arteriosus

  41. Initial Stabilization • ABC’s: Volume resuscitation, ionotorpic support, correction of metabolic acidosis, r/o sepsis • Intubate if needed, titrate Fi02 to keep Sp02 80%-85% to prevent pulmonary overcirculation • Placement of umbilical lines • Infants who present in shock within the first 3 weeks of life, consider ductal dependent lesions • Use of PGE1 (0.025 to 0.1mcg/kg/min)

  42. Stabilization for Transport • Reliable vascular access • Intubation if on PGE1, OG placement • Oxygen delivery, Sp02 • Monitor HR, tissue perfusion, blood pressure, and acid-base status • Calcium and glucose status (increased risk for DiGeorge)

  43. Prostaglandin E1 • Failure to respond: diagnosis incorrect, older infant with unresponsive ductus, ductus absent, obstructed pulmonary venous return • Clinical deterioration after PGE1: obstructed blood flow out of pulmonary veins or left atrium, HLHS with restrictive FO, TGA with intact ventricular septum and restrictive FO, obstructed TAPVR, mitral atresia with restrictive FO

  44. PGE 1 - Side Effects • Common: Apnea, fever, leukocytosis, cutaneous flushing, and bradycardia. • Uncommon: seizures, hypoventilation, hypotension, tachycardia, cardiac arrest, sepsis, diarrhea, DIC, fever • Rare: urticaria, bronchospasm, hemorrhage*, hypoglycemia, and hypocalcemia *inhibits platelet aggregation

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