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Dr Vivek pillai
• Defined as congenital absence or agenesis of
the tricuspid valve, with no direct
communication between the right atrium and
right ventricle.
• Coarctation of aorta – 8%
• Persistent left SVC
• Juxtaposition of atrial appendages-50% of TA
with TGA.
• Right aortic arch
• Abnormalities of mitral apparatus- cleft in
AML,malattachment of the valve,direct
attachment of the mitral leaflets to papillary
PHYSIOLOGY-TA WITH NRGA
• Obligatory rt to left shunt at atrial level.
• LA receives both the entire systemic and
pulmonary venous return.
• The entire mixture flows into the LV which is
the sole pumping chamber for the pulmonary
and systemic circulation.
• When the great arteries are normally
related,pulm artery blood flow is usually
reduced as the restrictive VSD, is a zone of
subpulmonic stenosis.
• LV overload is curtailed but at the cost of
cyanosis.
• This is so in 90% of cases.
In TGA
• The VSD is almost always non-restrictive and
pulmonary stenosis is usually absent.
• Low PVR results in abundant pulmonary
arterial blood flow.
• Minimal cyanosis,marked LV volume overload.
• If these pts have a restrictive vsd,or
infundibular narrowing→diminished syst
circulation→metabolic acidosis and shock.
SEX PREDILECTION
• Tr. Atresia with normally related great arteries
have an equal frequency in males and
females.
• If TGA is present→male preponderance.
• No male preponderance with juxtaposition of
atrial appendages.
GENETICS
• Although specific genetic causes of the
malformation remain to be determined in
humans, the FOG2 gene may be involved in
the process.
• This has however been validated only in
animal studies.
• Familial recurrence is low , and recurrence in
siblings is only about 1%.
OVERALL ACTUARIAL SURVIVAL IN
INFANTS WITH TRICUSPID ATRESIA
• 1 year- 72%.
• 5 years- 52%.
• 10 years- 46%
NATURAL HISTORY
• Few infants with tr. Atresia and normally
related gr . Arteries with an intact ventricular
septum survive beyond 6 months of age
without surgical palliation.
• Intense hypoxia and death ensue unless the
ductus is patent, or adequate systemic to PA
collaterals are present , either of which are
unlikely.
TR. ATRESIA WITH NRGA AND
SMALL VSD.
• The VSD in such patients closes
spontaneously or is excessively obstructive, so
that majority of patients die by one year.
• Rarely, a favorable balance is achieved b/w
the presence of VSD and pulm . Blood flow ,
permitting survival from 2nd to 5th decades.
TR. ATRESIA WITH NRGA AND
LARGE VSD.
• Pts with TA , normally related great arteries
and large VSD do not fare well
• Excessive pulmonary arterial flow results in
vol. overload of LV and CCF.
• Pts have lived to ages 4 to 6 years.
• In exceptional cases, long survivals have been
reported between ages 32 and 45 yrs.
TR. ATRESIA WITH TGA
• Same poor longevity patterns hold when TA
occurs with complete transposition and large
VSD.
• TA with TGA with subaortic stenosis(
restrictive VSD) is an ominous combination.
• Exceptional survivals to mid-late teens have
been recorder.
• problems related to increased longevity-
I.E,brain abscess, paradoxical embolism
Physical examination- appearance
• Dysmorphic facies-occasionally “cat-eye”
syndrome or congenital coloboma may
be seen.
JVP
• Increase in the A wave amplitude , due to the
restrictive interatrial communication.
• Y descent is slow
• In LVF, A and V waves increase in amplitude.
Precordium
• LV impulse without a RV impulse in a cyanotic
patient.
• A gentle RV impulse in pt with TA ,complete
transposition and a well dev RV .
• Palpable thrill if VSD is restrictive.
AUSCULTATION