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Congenital  Heart Disease: Cyanotic Lesions Martin Tristani-Firouzi December 2005
scenario <ul><li>It’s 2:00 A.M. </li></ul><ul><li>Your first night on call on your Pediatric rotation. </li></ul><ul><li>T...
Scenario <ul><li>O 2  sat=58% </li></ul><ul><li>RR=82 </li></ul><ul><li>Mild respiratory distress </li></ul>What will you ...
Cyanosis <ul><li>Bluish discoloration of skin and mucous membranes </li></ul><ul><li>Noticeable when the concentration of ...
Cyanosis: peripheral vs central <ul><li>peripheral </li></ul><ul><li>Sluggish blow flow in capillaries  </li></ul><ul><li>...
Evaluation of cyanosis:  100% O 2  test measure pAO 2  in room air and 100% O 2 <ul><li>Lung disease: </li></ul><ul><li>Ro...
Chronic cyanosis causes  clubbing  of the digits
Prostaglandin (PGE 1 ) <ul><li>Stable derivative of endogenous compound that maintains ductal patency in utero </li></ul><...
The basis of congenital heart disease is rooted in an arrest of or deviation in normal cardiac development
The 5 T’s of cyanotic heart disease <ul><li>Tetralogy of Fallot </li></ul><ul><li>TGA (d-transposition of the great arteri...
Tetralogy of Fallot <ul><li>1. Pulmonary stenosis </li></ul><ul><li>2. Large VSD </li></ul><ul><li>3. Overriding aorta </l...
Tetralogy of Fallot <ul><li>6 % of all congenital heart disease </li></ul><ul><li>1:3600 live births </li></ul><ul><li>mos...
T of F occurs as a result of abnormal  cardiac septation  Normal development Abnormal development
Tetralogy of Fallot <ul><li>Anterior deviation of the outlet ventricular septum is the cause of all four abnormalities see...
Tetralogy of Fallot - CXR <ul><li>Typical “boot-shaped” heart secondary to RVH and small main pulmonary artery segment </l...
Hypercyanotic “tet” spell <ul><li>Paroxysmal hypoxemia due to acute change in balance between  PVR  and  SVR </li></ul><ul...
Management of “tet” spell: goal is to    SVR and    PVR <ul><li>Knee-chest position  (   SVR) </li></ul><ul><li>Supplem...
Surgical repair: Tof F
D-Transposition of the Great Arteries <ul><li>Ao is anterior, arises from right ventricle </li></ul><ul><li>PA  posterior,...
d-TGA results from abnormal formation of aortico-pulmonary septum http://www.med.unc.edu/embryo_images/ PA AO cushion LV R...
D-transposition of great arteries <ul><li>Systemic and pulmonary circulations are in parallel, rather than in series </li>...
D-transposition of great arteries <ul><li>5% of all congenital heart disease </li></ul><ul><li>Most common cause of cyanos...
<ul><li>Narrow mediastinum due to anterior-posterior orientation of great arteries and small thymus  </li></ul><ul><li>Car...
Initial management of d-TGA: goal is to improve mixing <ul><li>Start PGE 1  to prevent ductal closure </li></ul><ul><li>Op...
Balloon atrial septostomy (Rashkind procedure)
Surgical management of d-TGA: Arterial switch procedure The arterial trunks are transected and “switched” to restore “norm...
Truncus arteriosus <ul><li>Aorta, pulmonary arteries, and coronary arteries arise from single vessel. </li></ul><ul><li>Tr...
Truncus Arteriosus RV LV RAA LAA PA Ao Tr
Truncus arteriosus: aortico-pulmonary septum fails to develop http://www.med.unc.edu/embryo_images/ PA AO cushion LV RV LA...
Conotruncal development is dependent upon normal migration of neural crest cells Neural crest tissue is required for forma...
DiGeorge Syndrome (22q11 deletion) <ul><li>Deletion of a portion of chromosome 22 results in abnormal neural crest develop...
Surgical correction: Truncus Arteriosus
Surgical correction: Truncus Arteriosus
Total anomalous pulmonary venous return (TAPVR) <ul><li>Failure of pulm veins (PV) to fuse with developing left atrium </l...
Embryological basis of TAPVR Systemic venous connections reabsorb as PV confluence fuses w/ left atrium PV confluence PV c...
Venous connections in TAPVR Supracardiac: Ascending vertical vein most common Cardiac: RA or coronary sinus Infracardiac: ...
Clinical manifestation of TAPVR <ul><li>Obstructed </li></ul><ul><li>Severe pulmonary edema, cyanosis, shock </li></ul><ul...
Supracardiac TAPVR Pulmonary vein ascending vertical vein superior vena cava ascending aorta
TAPVC - Supracardiac
Supracardiac TAPVC - CXR “ Snowman ” appearance secondary to dilated vertical vein, innominate vein and right superior ven...
Infracardiac TAPVR
TAPVC - Infracardiac Lungs Descending vertical vein Liver heart
TAPVR - CXR  Infracardiac = Obstructed = Surgical Emergency
Tricuspid atresia <ul><li>Absent communication from RA to RV </li></ul><ul><li>Obligate R to L shunt at atrial level </li>...
Tricuspid atresia <ul><li>Pulmonary valve may be normal, stenotic or atretic </li></ul><ul><li>Degree of cyanosis proporti...
Tricuspid atresia RA LA LV LA LV RV
Pulmonary atresia <ul><li>Atretic pulmonary valve </li></ul><ul><li>Pulmonary arteries often normal in size </li></ul><ul>...
<ul><li>Ductal-dependent lesion </li></ul><ul><li>Requires PGE 1  to maintain oxygenation </li></ul><ul><li>Therapy direct...
Pulmonary atresia
Ebstein’s malformation of tricuspid valve <ul><li>TV leaflets attach to RV wall, rather than TV annulus </li></ul><ul><li>...
Ebstein’s malformation of TV <ul><li>Massive RA dilation due to severe tricuspid regurgitation </li></ul><ul><li>R to L sh...
Ebstein’s malformation of TV RA aRV RV aRV
Scenario <ul><li>Room air pAO 2  = 29  sat=58% </li></ul><ul><li>100% O2 pAO 2  = 35  sat=67% </li></ul>What will you do n...
 
Cyanosis and Hemoglobin
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Cyanotic Heart Disease

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  • Truncus arteriosus: Symptoms, Surgical Process and Risks

    Truncus arteriosus otherwise known as Persistent truncus arteriosus, is a kind of congenital heart disease that is not a frequent occurence. Here, the Truncus Arteriosus is not forked properly into the aorta and pulmonary artery, resulting into the presence of a single large artery instead. Hence, this single artery has to perform dual functions of carrying blood both to the body as well as the lungs.

    http://www.heart-consult.com/articles/truncus-arteriosus-symptoms-surgical-process-and-risks
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Transcript of "Cyanotic Heart Disease"

  1. 1. Congenital Heart Disease: Cyanotic Lesions Martin Tristani-Firouzi December 2005
  2. 2. scenario <ul><li>It’s 2:00 A.M. </li></ul><ul><li>Your first night on call on your Pediatric rotation. </li></ul><ul><li>The R.N. in the newborn nursery calls you to see a blue baby. </li></ul><ul><li>You run. </li></ul>
  3. 3. Scenario <ul><li>O 2 sat=58% </li></ul><ul><li>RR=82 </li></ul><ul><li>Mild respiratory distress </li></ul>What will you do next?
  4. 4. Cyanosis <ul><li>Bluish discoloration of skin and mucous membranes </li></ul><ul><li>Noticeable when the concentration of deoxy-hemoglobin is at least 5g/dl </li></ul><ul><li>(O 2 sat < 85%) </li></ul>
  5. 5. Cyanosis: peripheral vs central <ul><li>peripheral </li></ul><ul><li>Sluggish blow flow in capillaries </li></ul><ul><li>Involves extremities (acrocyanosis) </li></ul><ul><li>Spares trunk & mucous membranes </li></ul><ul><li>central </li></ul><ul><li>Abnl of lungs or heart that interferes w/ O2 transport </li></ul><ul><li>Involves trunk & mucous membranes </li></ul>
  6. 6. Evaluation of cyanosis: 100% O 2 test measure pAO 2 in room air and 100% O 2 <ul><li>Lung disease: </li></ul><ul><li>Room air pAO 2 30 mmHg O 2 sat=60% </li></ul><ul><li>100% O 2 pAO 2 110 mmHg O 2 sat=100% </li></ul><ul><li>Cardiac disease: </li></ul><ul><li>Room air pAO 2 30 mmHg O 2 sat=60% </li></ul><ul><li>100% O 2 pAO 2 40 mmHg O 2 sat=75% </li></ul>pAO 2 >100 mmHg suggests lung disease Little or no change in pAO 2 suggests cyanotic heart disease
  7. 7. Chronic cyanosis causes clubbing of the digits
  8. 8. Prostaglandin (PGE 1 ) <ul><li>Stable derivative of endogenous compound that maintains ductal patency in utero </li></ul><ul><li>Prevents postnatal ductal closure, improves pulmonary blood flow, improves oxygenation </li></ul><ul><li>Stabilizes cyanotic neonate so that corrective surgery can be performed “electively” </li></ul><ul><li>PGE 1 revolutionized the fields of pediatric cardiology and cardiovascular surgery </li></ul>
  9. 9. The basis of congenital heart disease is rooted in an arrest of or deviation in normal cardiac development
  10. 10. The 5 T’s of cyanotic heart disease <ul><li>Tetralogy of Fallot </li></ul><ul><li>TGA (d-transposition of the great arteries) </li></ul><ul><li>Truncus arteriosus </li></ul><ul><li>Total anomalous pulmonary venous return </li></ul><ul><li>Tricuspid atresia / single ventricle </li></ul><ul><li>Pulmonary atresia </li></ul><ul><li>Ebstein’s malformation of tricuspid valve </li></ul>
  11. 11. Tetralogy of Fallot <ul><li>1. Pulmonary stenosis </li></ul><ul><li>2. Large VSD </li></ul><ul><li>3. Overriding aorta </li></ul><ul><li>4. Right ventricular hypertrophy </li></ul>
  12. 12. Tetralogy of Fallot <ul><li>6 % of all congenital heart disease </li></ul><ul><li>1:3600 live births </li></ul><ul><li>most common cause of cyanosis in infancy/childhood </li></ul><ul><li>Severity of cyanosis proportional to severity of RVOT obstruction </li></ul>RV
  13. 13. T of F occurs as a result of abnormal cardiac septation Normal development Abnormal development
  14. 14. Tetralogy of Fallot <ul><li>Anterior deviation of the outlet ventricular septum is the cause of all four abnormalities seen in tetralogy of Fallot. </li></ul>
  15. 15. Tetralogy of Fallot - CXR <ul><li>Typical “boot-shaped” heart secondary to RVH and small main pulmonary artery segment </li></ul><ul><li>Pulmonary vascular markings are decreased </li></ul>
  16. 16. Hypercyanotic “tet” spell <ul><li>Paroxysmal hypoxemia due to acute change in balance between PVR and SVR </li></ul><ul><li> SVR causes an increase in R  L shunt, increasing cyanosis </li></ul><ul><li> SVR (hot bath, fever, exercise) </li></ul><ul><li>Agitation  dynamic subpulmonic obstruction </li></ul><ul><li>Life-threatening if untreated </li></ul>
  17. 17. Management of “tet” spell: goal is to  SVR and  PVR <ul><li>Knee-chest position (  SVR) </li></ul><ul><li>Supplemental O 2 </li></ul><ul><li>Fluid bolus i.v. (  SVR) </li></ul><ul><li>Morphine i.v. (  agitation,  dynamic RVOT obstruction) </li></ul><ul><li>NaHCO 3 to correct metabolic acidosis (  PVR) </li></ul><ul><li>Phenylephrine to  SVR </li></ul><ul><li> -blocker to  dynamic RVOT obstruction </li></ul>
  18. 18. Surgical repair: Tof F
  19. 19. D-Transposition of the Great Arteries <ul><li>Ao is anterior, arises from right ventricle </li></ul><ul><li>PA posterior, arises from left ventricle </li></ul><ul><li>Systemic venous (blue) blood returns to RV and is ejected into aorta </li></ul><ul><li>Pulm venous (red) blood returns to LV and is ejected into PA </li></ul>RV LV PA Ao
  20. 20. d-TGA results from abnormal formation of aortico-pulmonary septum http://www.med.unc.edu/embryo_images/ PA AO cushion LV RV LA RA truncus
  21. 21. D-transposition of great arteries <ul><li>Systemic and pulmonary circulations are in parallel, rather than in series </li></ul><ul><li>Mixing occurs at atrial and ductal levels </li></ul><ul><li>Severe, life-threatening hypoxemia </li></ul>
  22. 22. D-transposition of great arteries <ul><li>5% of all congenital heart disease </li></ul><ul><li>Most common cause of cyanosis in neonate </li></ul><ul><li>Male:female 2:1 </li></ul>RV Ao PA RA LA
  23. 23. <ul><li>Narrow mediastinum due to anterior-posterior orientation of great arteries and small thymus </li></ul><ul><li>Cardiomegaly is present w/ increased pulmonary vascular markings </li></ul>d-TGA CXR: “egg on a string”
  24. 24. Initial management of d-TGA: goal is to improve mixing <ul><li>Start PGE 1 to prevent ductal closure </li></ul><ul><li>Open atrial septum to improve mixing at atrial level (Rashkind procedure). </li></ul>
  25. 25. Balloon atrial septostomy (Rashkind procedure)
  26. 26. Surgical management of d-TGA: Arterial switch procedure The arterial trunks are transected and “switched” to restore “normal” anatomy The coronary arteries are resected and re-implanted.
  27. 27. Truncus arteriosus <ul><li>Aorta, pulmonary arteries, and coronary arteries arise from single vessel. </li></ul><ul><li>Truncus sits over large ventricular septal defect. </li></ul><ul><li>Failure of septation of embryonic truncus. </li></ul><ul><li>Uncommon (1.4% of CHD) </li></ul>
  28. 28. Truncus Arteriosus RV LV RAA LAA PA Ao Tr
  29. 29. Truncus arteriosus: aortico-pulmonary septum fails to develop http://www.med.unc.edu/embryo_images/ PA AO cushion LV RV LA RA truncus
  30. 30. Conotruncal development is dependent upon normal migration of neural crest cells Neural crest tissue is required for formation of: conotruncus aortic arches facial structures thymus parathyroid
  31. 31. DiGeorge Syndrome (22q11 deletion) <ul><li>Deletion of a portion of chromosome 22 results in abnormal neural crest development </li></ul><ul><li>Conotruncal defects (truncus arteriosus, interrupted aortic arch) </li></ul><ul><li>Thymic aplasia (T-cell dysfunction) </li></ul><ul><li>Parathyroid aplasia (hypocalcemia) </li></ul><ul><li>Facial dysmorphic features </li></ul>
  32. 32. Surgical correction: Truncus Arteriosus
  33. 33. Surgical correction: Truncus Arteriosus
  34. 34. Total anomalous pulmonary venous return (TAPVR) <ul><li>Failure of pulm veins (PV) to fuse with developing left atrium </li></ul><ul><li>PV drainage occurs thru embryological remnants of systemic veins </li></ul><ul><li>Incidence: rare </li></ul>
  35. 35. Embryological basis of TAPVR Systemic venous connections reabsorb as PV confluence fuses w/ left atrium PV confluence PV confluence fails to fuse w/ left atrium Systemic venous connections persist
  36. 36. Venous connections in TAPVR Supracardiac: Ascending vertical vein most common Cardiac: RA or coronary sinus Infracardiac: Descending vein to portal system supracardiac cardiac infracardiac
  37. 37. Clinical manifestation of TAPVR <ul><li>Obstructed </li></ul><ul><li>Severe pulmonary edema, cyanosis, shock </li></ul><ul><li>Surgical emergency </li></ul><ul><li>Unobstructed </li></ul><ul><li>Mild to moderate congestive heart failure and cyanosis </li></ul><ul><li>Surgery in first 6 mo. </li></ul>
  38. 38. Supracardiac TAPVR Pulmonary vein ascending vertical vein superior vena cava ascending aorta
  39. 39. TAPVC - Supracardiac
  40. 40. Supracardiac TAPVC - CXR “ Snowman ” appearance secondary to dilated vertical vein, innominate vein and right superior vena cava draining all the pulmonary venous blood
  41. 41. Infracardiac TAPVR
  42. 42. TAPVC - Infracardiac Lungs Descending vertical vein Liver heart
  43. 43. TAPVR - CXR Infracardiac = Obstructed = Surgical Emergency
  44. 44. Tricuspid atresia <ul><li>Absent communication from RA to RV </li></ul><ul><li>Obligate R to L shunt at atrial level </li></ul><ul><li>GA normally related (70%) </li></ul><ul><li>GA transposed (30%) </li></ul>
  45. 45. Tricuspid atresia <ul><li>Pulmonary valve may be normal, stenotic or atretic </li></ul><ul><li>Degree of cyanosis proportional to degree of pulmonary stenosis </li></ul><ul><li>Necessity for PGE 1 related to degree of pulmonary stenosis </li></ul>
  46. 46. Tricuspid atresia RA LA LV LA LV RV
  47. 47. Pulmonary atresia <ul><li>Atretic pulmonary valve </li></ul><ul><li>Pulmonary arteries often normal in size </li></ul><ul><li>hypoplastic RV, RVH </li></ul><ul><li>hypoplastic TV </li></ul>
  48. 48. <ul><li>Ductal-dependent lesion </li></ul><ul><li>Requires PGE 1 to maintain oxygenation </li></ul><ul><li>Therapy directed at opening atretic valve in cath lab or surgery </li></ul><ul><li>Prognosis depends upon size and compliance of hypoplastic RV </li></ul>Pulmonary atresia
  49. 49. Pulmonary atresia
  50. 50. Ebstein’s malformation of tricuspid valve <ul><li>TV leaflets attach to RV wall, rather than TV annulus </li></ul><ul><li>Tethered leaflets create 2 chambers w/in RV: large “atrialized” RV, small noncompliant functional RV </li></ul><ul><li>Severe tricuspid regurgitation </li></ul>
  51. 51. Ebstein’s malformation of TV <ul><li>Massive RA dilation due to severe tricuspid regurgitation </li></ul><ul><li>R to L shunt at atrial level causes cyanosis </li></ul><ul><li>Degree of cyanosis related to size and compliance of functional RV </li></ul><ul><li>Cyanosis usually decreases as PVR falls shortly after birth </li></ul>
  52. 52. Ebstein’s malformation of TV RA aRV RV aRV
  53. 53. Scenario <ul><li>Room air pAO 2 = 29 sat=58% </li></ul><ul><li>100% O2 pAO 2 = 35 sat=67% </li></ul>What will you do next? Start PGE 1 Call cardiology to do echocardiogram
  54. 55. Cyanosis and Hemoglobin
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