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Chapter 30

Chapter 30. Congenital Heart Defects. http://www.youtube.com/watch?v=KRy8gfmGSxg. Cardiac Defects. Patent Ductus Arteriosus Atrial Septal Defect Ventricular Septal Defect Tetralogy of Fallot Transposition of the Great Arteries Coarctation of the Aorta Anomalous Venous Return

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Chapter 30

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  1. Chapter 30 Congenital Heart Defects • http://www.youtube.com/watch?v=KRy8gfmGSxg

  2. Cardiac Defects • Patent Ductus Arteriosus • Atrial Septal Defect • Ventricular Septal Defect • Tetralogy of Fallot • Transposition of the Great Arteries • Coarctation of the Aorta • Anomalous Venous Return • Truncus Arteriosus • Hypoplastic Left-Heart Syndrome

  3. Heart • Congenital heart disease (CHD) occurs in 1/125 live births. • Neonates may present with a variety of non-specific findings, including: - tachypnea - cyanosis - pallor - lethargy - FTT - sweating with feeds • More specific findings include: - pathological murmurs - hypertension - abnormal pulses - syncope

  4. Neonatal cardiac physiology • The transformation from fetal to neonatal circulation involves two major changes: • A marked increase in systemic resistance. • caused by loss of the low-resistance placenta. 2. A marked decrease in pulmonary resistance. • caused by pulmonary artery dilation with the neonate’s first breaths.

  5. Fetal Circulation • No circulation to lungs • Foramen ovale • Ductus arteriosum • Circulation must go to placenta • Umbilical aa., vv.

  6. Fetal cardiac physiology Fetal circulation: • Blood flows from the placenta  IVC  RA  through the PFO  LA  LV •  ascending aorta •  brain •  returns via the SVC

  7. Fetal cardiac physiology Fetal circulation: • From the SVC  RA  RV •  pulm aa •  through the PDA •  descending aorta •  lower extremities and placenta

  8. Fetal cardiac physiology Fetal circulation: • Only a very small amount of blood is directed through the right and left pulmonary aa’s to the lungs.

  9. Neonatal cardiac physiology Neonate circulation: • The transformation to neonatal circulation occurs with the first few breaths. • The two remaining remnants of the fetal circulation are a patent foramen ovale... • and ductus arteriosus.

  10. Congenital Heart Disease • Neonates with CHD often rely on a patent ductus arteriosus and/or foramen ovale to sustain life. • Unfortunately for these neonates, both of these passages begins to close following birth. • The ductus normally closes by 72hrs. • The foramen ovale normally closes by 3 months. • http://www.youtube.com/watch?v=FG-CNV501bc

  11. CHD • That being said, in the presence of hypoxia or acidosis (generally present in ductus-dependent lesions), the ductus may remain open for a longer period of time. • As a result, these patients often present to the ED during the first 1-3 weeks of life. • i.e. as the ductus begins to close.

  12. Classifying CHD • There are many different classification systems for CHD. • None are particularly good. • I will be discussing the Pink/Blue/Grey-Baby system: • Pink Baby – Left to right shunt • Blue Baby – Right to left shunt • Grey Baby – LV outflow tract obstruction

  13. Pink Baby (L  R shunt) • L  R shunts cause CHF and pulmonary hypertension. • This leads to RV enlargement, RV failure, and cor pulmonale. • These babies present with CHF and respiratory distress. • They are not typically cyanotic. • http://www.youtube.com/watch?v=46tmI2_RVuE&list=PLA81DD78BDE77FBFC

  14. Pink Baby (L  R shunt) • These lesions include (among others) ASD’s, VSD’s, and persistently patent ductus arteriosus. • VSD • ASD

  15. Pink Baby (L  R shunt) • Persistently patent ductus arteriosus

  16. Pink Baby (L  R shunt) • Diagnosing L  R shunts depends on: 1. Examination findings: • Non-cyanotic infant in resp distress. • Crackles, widely-fixed second heart sound, elevated JVP, cor pulmonale. 2. CXR: • Increased pulmonary vasculature (suggestive of CHF). • RA and/or RV enlargement.

  17. Pink Baby (L  R shunt) • Initial management should be directed at reducing the pulm edema. • Adminster Lasix 1mg/kg IV. • Peds Cardiology/ PICU should be consulted urgently regarding use of: • Morphine • Nitrates • Digoxin • Inotropes

  18. Ductus Arteriosus • Fetal Circulation Component • Connects Pulmonary Artery to Aorta • Shunts blood away from lungs • Maintained patent by presence of prostaglandins • Closure secondary to: • Increase in PaO2_ • Decrease in level of prostaglandins

  19. Patent Ductus Arteriosus • 5-10% of all births (1 of 2000 live births) • 80% of premature babies • 2-3 times more common in females than males. • 5th or 6th most common congenital cardiac defect. • Often associated with other defects. • May be desirable with some defects. • Morbidity/Mortality related to degree of blood flow through PDA.

  20. Pathophysiology - PDA • With a drop in pulmonary arterial pressure (reduction in hypoxic pulmonary vascular constriction), blood will flow through PDA. • LEFT TO RIGHT SHUNT • Increased pulmonary blood flow may lead to pulmonary edema. • Reduced blood flow to all postductal organs • NEC • If pulmonary artery pressure rises above Aortic pressure, blood will move in the other direction. • RIGHT TO LEFT SHUNT

  21. Diagnosis - PDA • Loud grade I to grade III systolic murmur at left sternal border. • Washing machine • Echocardiography

  22. Treatment - PDA • Restrict fluids. • Diuretics • Prostaglandin Inhibitors - Indomethacin • Surgical closure (ligation).

  23. Atrial Septal Defect • 6-10% of all births (1 of 1500 live births) • 2 times more common in females than males. • Types: • Ostium Secundum (at or about the Foramen Ovale) • Sinus Venous • In 1950 most children with ASD did not reach the first grade. Today, first year surgery facilitates normal growth and development.

  24. ASD: Pathophysiology and Diagnosis • Pathophysiology • Left to Right Shunt • Inefficient recirculation of good blood through pulmonary arteries. • May not manifest symptoms and may be found later in life. • If defect is significant, may cause problems later in life due to inefficiencies. • Diagnosis • Murmur • Echocardiography

  25. Treatment - ASD • Surgical closure. • Non-Surgical closure via cardiac catheterization.

  26. Ventricular Septal Defect • 1% of all births (2 to 4 of 1000 live births) • Vast majority the hole is small. • In 1950, fatal. Today almost all VSD can be closed successfully, even in small babies. Lillehei was the first person in history to correct both ASD and VSD on 8/31/54.

  27. VSD: Pathophysiology & Diagnosis • Pathophysiology • May be isolated or associated with other congenital cardiac defects. • With normal PVR: • LEFT TO RIGHT SHUNT • With elevated PVR (RDS): • RIGHT TO LEFT SHUNT • Diagnosis • Echocardiography

  28. Treatment - VSD • Nothing if VSD is small. • With CHF or Failure to Thrive: Surgical closure.

  29. Blue Baby (R  L shunt) • R  L shunts cause hypoxia and central cyanosis. • Neither hypoxia or cyanosis tend to improve with 100% oxygen. • R  L lesions include (among others): • Tetralogy of Fallot (TOF) • Transposition of the Great Arteries (TGA)

  30. Blue Baby (R  L shunt) • Hypoxia and cyanosis (unresponsive to oxygen) in the neonatal period suggests a ductus-dependent lesion. • Treatment is a prostaglandin-E1 (PGE1) infusion. • Dosing discussed momentarily • This should obviously be accompanied by urgent Peds Cardiology and PICU consultation.

  31. Tetralogy of Fallot • Characterized by: • Pulmonary aa OTO • RV hypertrophy • VSD • Over-riding aorta • With severe pulmonary OTO... • * • * • * • bloodflow to the lungs may be highly ductus-dependent. • *

  32. Tetralogy of Fallot • The classic CXR finding in TOF is the boot-shaped heart. • Pulmonary vasculature is typically decreased.

  33. Tetralogy of Fallot • 1% of neonates. • Most common of the cyanotic cardiac diseases. • Mortality increases with age (1 year-old has a 25% mortality, 40 year-old has 95%). • In 1950, fatal. Today, less than 5% mortality with children operated on in infancy, leading normal lives.Four Defects • Pulmonary Artery Stenosis (determinant factor related to severity) • VSD (usually large) • Overriding Aorta • RV hypertrophy

  34. Tetralogy of Fallot: Diagnosis and Treatment • Tet Spells (Blue spells) • CXR: Boot-shaped Heart • Diagnosed with echocardiography. • Surgical correction. • Reparative or Palliative (Blalock-Taussig)

  35. Blalock-Taussig • Something the Lord Made. • Vivien Thomas

  36. Tetralogy of Fallot

  37. Transposition of the Great Arteries • TGA is one of the most common cyanotic lesion presenting in the first week of life. • Anatomically: • RV  aorta • LV  pulmonary aa • To be compatible with life, mixing of the two circulations must occur via an ASD, VSD, or PDA. • http://www.youtube.com/watch?v=O83cYwKOKtI&list=PLA81DD78BDE77FBFC

  38. Transposition of the Great Arteries • The CXR findings in TGA are typically less dramatic than in TOF. • Pulmonary vasculature is typically increased. • http://www.youtube.com/watch?v=b-TkE_wygT4&list=PLA81DD78BDE77FBFC&index=1

  39. Complete Transposition of the Great Arteries • Second most common form (5-7%) of congenital cardiac anomalies. • Aorta arises from RV and Pulmonary Arteries from LV. • Without an abnormality, life would not be possible. • ASD • VSD (30-40%) • PDA

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