ATREIA

1. TRICUSPID
ATRESIA

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. Causes
• Multifactorial inheritance hypothesis

7. 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

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

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

10. WITH
TGA
LUNGS

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

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

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

14. 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

15. 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,

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

18. Reduced pulm flow

19. INCREASED BLOOD
FLOW

20. Large
ventricular
cavity
Small RV
ECHOCARDIOGRAPHY

21. angiography

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

23. Rashkind balloon atrial
septostomy.

24. • 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

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

26. Modified Blalock-
Taussig shunt
Gortex
interposition
graft

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

28. Potts
Direct anastomosis
descending aorta to
left pulmonary
artery

29. Direct anastomosis
ascending aorta to
right pulmonary
artery

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

31. Pulmonary artery
band

32. • Palliation for tricuspid
atresia

35. Hemifontan/Bidirectional
Glenn

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

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

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