tricuspid atresia

1. TRICUSPID
ATRESIA
AMRUTHA R

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.

4. 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 %.

5. To survive your baby will
need either
• ASD OR VSD
• PDA

6. HISTORY
• First reported by Kreysig in 1817.
• Clinical features reported by Bellet and
Stewart in 1933.
• Also by Taussig and Brown in 1936.

7. Embryology

14. 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.

16. Causes
• Multifactorial inheritance hypothesis

17. TYPES

18. MUSCULAR
COMMON
89%
DIMPLING OR
FIBROUS
THICKENING

19. MEMBRANEOUS
6.6%
SEPTUM FORM
THE FLOOR
OF RA

20. VALVAR
1%
FUSED CUSPS

21. EBSTEINS
ANOMALY

22. • 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.

23. 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

24. • Mixing of systemic and pulmonary
blood
• Enters LV
• Blood enters RV through VSD
• From RV blood enters Pulm trunk

25. • Blood enters pulm trunk via PDA
• Increased pulmonary blood flow
• LA and LV hypertrophy
CHF

26. WITH
TGA
LUNGS

27. • The clinical features of tricuspid
atresia largely depend on the quantity of
pulmonary blood flow

28. DECREASED PULM
FLOW 90%
severe
cyanosis, hypoxemia
, and acidosis
LV apical impulse
Waves in jugular
venous pulse

29. • pulmonary oligemia
• may have central cyanosis,
• tachypnea or hyperpnea,

30. 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

31. 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,

33. INVESTIGATIONS
• History and PE
• Pulse oximetry
• ABG
• Hb and hematocrit

34. Reduced pulm flow

35. INCREASED BLOOD
FLOW

36. Large
ventricular
cavitySmall RV
EECHOCARDIOGRAPHY

37. angiography

38. MEDICAL
• an intravenous infusion of PGE1
• 0.03-0.1 mcg/kg/min to open the ductus
arteriosus
• anticongestive therapy with digoxin,
diuretics

39. Rashkind balloon atrial
septostomy.

40. • 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

41. Classic
Blalock-Taussig shunt
end to side
anastomosis
Rarely perf

42. Modified Blalock-
Taussig shunt
Gortex
interposition
graft

43. Central shunt
Gortex interposition
graft between aorta and
main pulmonary artery
Reprinted

44. Potts
Direct anastomosis
descending aorta to
left pulmonary
artery

45. Direct anastomosis
ascending aorta to
right pulmonary
artery

46. • Palliation for increased pulmonary
blood flow
• Control amount of pulmonary blood
flow to prevent CHF and pulmonary
vascular disease from pulmonary
overcirculation

47. Pulmonary artery
band

48. • Palliation for tricuspid
atresia

51. Hemifontan/Bidirectional
Glenn

54. • 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

56. complications:
• Arrhythmia:
• ablation, pacemaker, ICD, medications,
conversion to lateral tunnel
• B. Ventricular dysfunction:
• rhythm and transplant

57. • C. Atrioventricular valve regurgitation
(AVVR): Valve repair/replacement
• D. Fontan pathway obstruction:
reoperation for relief of conduit stenosis

58. bronchitis:
• G. Thromboembolic events:
• anticoagulation varies from center to
center but minimally life long aspirin (ASA