Tricuspid atresia
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TRICUSPID ATRESIA. Dr Vivek pillai. 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 %.

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Dr Vivekpillai

  • 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 %.


  • First reported by Kreysig in 1817.

  • Clinical features reported by Bellet and Stewart in 1933.

  • Also by Taussig and Brown in 1936.


  • During early embryogenesis,the process of expansion of the inlet portion of the right ventricle coincides with development of the AV valves.

  • Failure of this process of inlet expansion is the pathogenetic mechanism for the usual muscular variety of tricuspid atresia.

  • The less common variety , with well formed but fused leaflets,occurs if the embryological insult occurs later in gestation.

  • If valve fusion is incomplete, tricuspid stenosis develops.


  • The most common type of tricuspid atresia is muscular .

  • It is characterized by a dimple or a localized fibrous thickening in the floor of the right atrium at the expected site of the tricuspid valve.

  • The muscular variety constitutes 89% of cases.

  • In the membranous type (6.6%), the atrioventricular portion of the membranous septum forms the floor of the right atrium at the expected location of the tricuspid valve.

  • This particular type appears to be associated with absent pulmonary valve leaflets.

  • Minute valvar cusps are fused together in the valvar type (1%).

  • In the Ebstein type (2.6%), fusion of the tricuspid valve leaflets occurs; attachment is displaced downward, and plastering of the leaflets to the right ventricular wall occurs.

  • 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.

  • The right atrium is enlarged and hypertrophied.

  • An interatrial communication is necessary for survival.

  • This communication most commonly is a stretched patent foramen ovale.

  • A true ASD is much less common and when present is almost always in the ostium secundum location.

  • Rarely, the patent foramen ovale is obstructive and may form an aneurysm of the fossa ovalis, which is sometimes large enough to produce mitral inflow obstruction

  • The left atrium may be enlarged, especially when the pulmonary blood flow is increased.

  • The mitral valve is morphologically normal; it is rarely incompetent and has a large orifice.

  • The left ventricle is enlarged and hypertrophied but usually morphologically normal

  • The right ventricle is small and hypoplastic.

  • In patients with a large VSD or TGA, the size of the right ventricle may be larger, but, even in these patients, the right ventricle is smaller than normal.

VSD in tricuspid atresia

  • Associated VSD is common with TA, seen in about 90% of individuals during infancy.

  • Is usually perimembranous but also may occur in the muscular septum or as a component of an atrioventricular septal defect, although the latter is rare.

  • At birth the VSD is usually restrictive,permitting adequate but not excessive pulmonary blood flow .

  • This advantage is often lost, as 40% of these defects close spontaneously/ decrease in size-”acquired pulmonary atresia”

  • The majority of defects close in the first yr of life.

  • These restrictive VSD’S cause subpulmonic obstruction in pts with normally related great arteries, and subaortic obstruction in pts with TGA.


Proposed by Kuhne and later modified

Type 1 : normally related great arteries (70 – 80%)

a. intact ventricular septum with pulmonary atresia( 9%)

b. small ventricular septal defect and pulmonary stenosis( 51%)

c. large ventricular septal defect without pulmonary stenosis ( 9%)

Type ii : D-transposition of great arteries (12 – 25%)

a. ventricular septal defect with pulmonary atresia( 2%)

b. ventricular septal defect with pulmonary stenosis( 8%)

c. ventricular septal defect without pulmonary stenosis(18%)

Type 3 :L- Transposition or malposition of great arteries (3-6%)

associated complex lesions, ie., truncus arteriosus, endocardial cushion defect


  • 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 muscles.


  • 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.


  • 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.


  • 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.


  • 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%.


  • 1 year- 72%.

  • 5 years- 52%.

  • 10 years- 46%


  • 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.


  • 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.


  • 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.


  • 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.


  • 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.


  • 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.


  • First heart sound is single .

  • Second usually single, but a soft delayed pulmonic component is occasionally present.

  • TA with normally related great arteries, prominent systolic murmur originates at the site of restrictive VSD – holosystolic, maximal at the mid to lower left sternal edge.

TA with complete transposition and increased pulmonary blood flow

  • Holosystolic murmur – across VSD

  • S2 – single but always loud

  • S3

  • MDM

  • 4th heart sounds are rare in any of the varieties of tricuspid atresia.

Pulmonary vascular resistance – high

  • VSD murmur vanishes

  • Soft midsystolic murmur- anterior aortic root

  • Rarely, the loud second component from the dilated hypertensive posterior pulmonary trunk is heard.

  • TA with complete transposition, coexisting pulmonic or subpulmonic stenosis – midsystolic murmur – loudness and length vary inversely with degree of obstruction


  • Tall peaked right atrial P waves are usually seen

  • Biatrial P – if left atrial volume is↑ due to ↑PBF.

  • PR interval- normal.

  • QRS axis – left and superior ( type 1 pts)

  • Absence of RV forces in precordial leads


  • Pulmonary vascularity reduced.

  • Pulmonary artery segment – inconspicuous.

  • Heart size – normal.

  • Right cardiac border, esp in LAO projection – superior convexity caused by enlargement of RA and its appendage.

  • Inferior border – flat or receding owing to absence of RV.

  • LAO – Humped appearance of right cardiac border and a prominent left cardiac silhouette

TA with complete transposition and no obstruction

  • Lungs – plethoric

  • LV, LA, RA – enlarged

  • Right cardiac border seldom has distinctive hump-shaped contour – RV is relatively well developed

Tr. Atresia with TGA

TA with complete transposition and PS

  • Pulmonary blood flow is normal or reduced

  • Ascending aorta and pulmonary trunk are not border forming (narrow vascular pedicle)


  • Presence of an imperforate linear echo density in the location of normal TV

  • Presence of two great arteries and semilunar valves

  • Confirm the presence and size of the interatrial communication.

  • Confirm the presence of a VSD.


  • Limited role at present.

  • Therapeutic role for balloon atrial septostomy.

  • Prior to a Fontan for determining pulm.vascular resistance.


  • In infants, the right atrial pressure is slightly higher than the left atrial pressure.

  • prominent ‘a ‘wave in the right atrium, especially if the interatrial communication is restrictive.

  • LV systolic and EDP – normal.

  • LVEDP may increase in patients with large VSD’s as PVR drops and left heart volume overload , ensues.


  • PGE1, should be started in neonates with severe cyanosis to maintain patency of the ductus before cardiac catheterization or planned surgery

  • Balloon atrial septostomy may be carried out as part of the initial catheterization to improve the RA-LA shunt.


  • Surgical management may be broadly grouped into palliative and corrective therapy.


  • decreased pulmonary flow

  • increased pulmonary flow

  • intracardiac obstruction.


  • Pulmonary blood flow may be increased by surgical creation of an aortopulmonary shunt.

  • subclavian artery to ipsilateral pulmonary artery anastomosis by Blalock and Taussig in 1945

  • Potts shunt (descending aorta–to–left pulmonary artery anastomosis),

  • Waterston-Cooley shunt (ascending aorta–to–right pulmonary artery anastomosis

  • central aortopulmonary fenestration or Gore-Tex shunt,

  • modified Blalock-Taussig shunt (Gore-Tex interposition graft between the subclavian artery and the ipsilateral pulmonary artery),

  • Glenn shunt (superior vena cava–to–right pulmonary artery anastomosis, end-to-end),


  • In patients with tricuspid atresia type II , pulmonary artery banding should be performed following stabilization with anticongestive measures.


  • Fontan and Kreutzer- initial description of the physiologically corrective operation for tricuspid atresia

  • Complete separation of the systemic and pulmonary circuits


  • Age at operation – 4 and 15 yrs( not strictly followed nowadays)

  • Normal sinus rhythm

  • Normal systemic venous connections

  • Normal right atrial size

  • Normal pulmonary arterial mean pressure ( mean >= 15 mm Hg)

  • Low pulmonary vasc resistance (4 woods units/m2)

  • Adequate sized pulm. Arteries with diameter > 75% of aortic diameter.

  • Normal LVEF (>60%)( rel. contraindication)

  • Absence of MR( relative contraindication)

  • Absence of complicating factors from prev ious surgeries



Hepatomegaly and ascites.

Supraventricular arrythmias.

Progressive decrease in oxygen saturation( obstn. of venous pathways, leakage in intra- atrial baffle, dev of pulm av fistula.).

Protein losing enteropathy

  • Low cardiac output,heart failure or both .

  • Persistent pleural effusion.

  • Thrombus formation in the systemic venous pathways.

  • Liver dysfunction


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