Tricuspid atresia in pediatrics

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tricuspid atresia

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Tricuspid atresia in pediatrics

  1. 1. TRICUSPID ATRESIA AMRUTHA R
  2. 2. • Atresia means closed or absent. • Tricuspid Atresia results in a number of problems: • ♥ The tricuspid valve is closed or absent. • ♥ The right ventricle is small. • ♥ The pulmonary artery is narrow. • ♥ The pulmonary valve may be blocked.
  3. 3. Definition • Defined as congenital absence or agenesis of the tricuspid valve, with no direct communication between the right atrium and right ventricle. • Incidence : 0.06 per 1000 live births • Prevalence :in clinical series of congenital heart disease is 1- 2.4 %.
  4. 4. To survive your baby will need either • ASD OR VSD • PDA
  5. 5. HISTORY • First reported by Kreysig in 1817. • Clinical features reported by Bellet and Stewart in 1933. • Also by Taussig and Brown in 1936.
  6. 6. Embryology
  7. 7. Formation of the atrioventricular cardiac valves: Days 34 to 36 • 1.Formed from endocardial cushion tissue • 2. Tricuspid valve, papillary muscles and chordate tendineae formed largely from the conus septum.
  8. 8. Causes • Multifactorial inheritance hypothesis
  9. 9. TYPES
  10. 10. MUSCULAR COMMON 89% DIMPLING OR FIBROUS THICKENING
  11. 11. MEMBRANEOUS 6.6% SEPTUM FORM THE FLOOR OF RA
  12. 12. VALVAR 1% FUSED CUSPS
  13. 13. EBSTEINS ANOMALY
  14. 14. • The atrioventricular canal type is extremely rare (0.2%). -a leaflet of the common atrioventricular valve seals off the only entrance into the right ventricle.
  15. 15. PATHOPHYSIOLOGY • ATRESIA OF TRICUSPID VALVE • No communication between RA AND RV • RV id underdeveloped. • Systemic venous blood received by RA • Enters LA through PFO or ASD
  16. 16. • Mixing of systemic and pulmonary blood • Enters LV • Blood enters RV through VSD • From RV blood enters Pulm trunk
  17. 17. • Blood enters pulm trunk via PDA • Increased pulmonary blood flow • LA and LV hypertrophy CHF
  18. 18. WITH TGA LUNGS
  19. 19. • The clinical features of tricuspid atresia largely depend on the quantity of pulmonary blood flow
  20. 20. DECREASED PULM FLOW 90% severe cyanosis, hypoxemia , and acidosis LV apical impulse Waves in jugular venous pulse
  21. 21. • pulmonary oligemia • may have central cyanosis, • tachypnea or hyperpnea,
  22. 22. INCREASED PULM FLOW • Diff to diagnose • may not appear cyanotic but may present with signs of heart failure later in infancy • pulmonary plethora present with symptoms of dyspnea, fatigue, difficulty feeding, and perspiration, which are suggestive of congestive heart failure. • Cyanosis is minimal
  23. 23. Other features • holosystolic type of murmur at the lower sternal border, suggestive of VSD, • Problems related to chronic cyanosis, such as • clubbing, • polycythemia, relative anemia, • stroke, brain abscess, • coagulation abnormalities,
  24. 24. INVESTIGATIONS • History and PE • Pulse oximetry • ABG • Hb and hematocrit
  25. 25. Reduced pulm flow
  26. 26. INCREASED BLOOD FLOW
  27. 27. Large ventricular cavitySmall RV EECHOCARDIOGRAPHY
  28. 28. angiography
  29. 29. MEDICAL • an intravenous infusion of PGE1 • 0.03-0.1 mcg/kg/min to open the ductus arteriosus • anticongestive therapy with digoxin, diuretics
  30. 30. Rashkind balloon atrial septostomy.
  31. 31. • PALLIATION FOR DECREASED PULMONARY BLOOD FLOW • Systemic to pulmonary artery shunt: increases pulmonary blood flow through surgically created left to right shunt at the great vessel level
  32. 32. Classic Blalock-Taussig shunt end to side anastomosis Rarely perf
  33. 33. Modified Blalock- Taussig shunt Gortex interposition graft
  34. 34. Central shunt Gortex interposition graft between aorta and main pulmonary artery Reprinted
  35. 35. Potts Direct anastomosis descending aorta to left pulmonary artery
  36. 36. Direct anastomosis ascending aorta to right pulmonary artery
  37. 37. • Palliation for increased pulmonary blood flow • Control amount of pulmonary blood flow to prevent CHF and pulmonary vascular disease from pulmonary overcirculation
  38. 38. Pulmonary artery band
  39. 39. • Palliation for tricuspid atresia
  40. 40. Hemifontan/Bidirectional Glenn
  41. 41. • Surgical options for Fontan operation • a. Lateral tunnel: Gortex graft placed inside RA to direct IVC flow through RA/SVC junction and into MPA • b. Extracardiac (As Illustrated): Gortex or Dacron circumferential tube graft from IVC to MPA
  42. 42. complications: • Arrhythmia: • ablation, pacemaker, ICD, medications, conversion to lateral tunnel • B. Ventricular dysfunction: • rhythm and transplant
  43. 43. • C. Atrioventricular valve regurgitation (AVVR): Valve repair/replacement • D. Fontan pathway obstruction: reoperation for relief of conduit stenosis
  44. 44. bronchitis: • G. Thromboembolic events: • anticoagulation varies from center to center but minimally life long aspirin (ASA

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