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Medical College, Calicut

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Presentation on theme: "Medical College, Calicut"— Presentation transcript:

1 Medical College, Calicut
TRICUSPID ATRESIA Dr Bijilesh u Senior Resident, Dept. of Cardiology, Medical College, Calicut

2 References - Perloff ‘s text book of congenital heart diseases
- Moss and Adams ‘s text book of congenital cardiology - Rudolph text book of pediatric cardiology - Freedom’s natural and modified natural history of cong.heart diseases - Andreson text book of congenital heart disease - Figenbaum’ text book of echocardiography - Hurst’s heart disease - Braunwald’ heart diseases - Langman’s Embryology

3 Defined as congenital absence or agenesis of the tricuspid valve, with no direct communication between the right atrium and right ventricle

4 Incidence : 0.06 per 1000 live births Prevalence : 1- 3% of CHD
(Report of New England Regional Infant Cardiac Program – 1980)

5 HISTORY First reported by Kreysig in 1817
Clinical features reported by Bellet and Stewart in 1933 Taussig and Brown in 1936

6 EMBRYOLOGY Early embryogenesis - process of expansion of inlet portion of right ventricle coincides with development of AV valves Failure of this process - atresia of tricuspid valve & absent inlet portion of right ventricle Embryological insult occurring later in gestation - Less common variety - with well formed but fused leaflets If valve fusion incomplete - tricuspid stenosis

7 ANATOMY Muscular Membranous Atrioventricular

8 Most common type – muscular (89%) Dimple or a localized fibrous thickening in the floor of RA at expected site of tricuspid valve.

9 Membranous type - atrioventricular portion of membranous septum forms floor of the RA at the expected location of TV May be associated with absent pulmonary valve leaflets.

10 Minute valvar cusps are fused together (1%) Ebstein type (2.6%)
Valvar type Minute valvar cusps are fused together (1%) Ebstein type (2.6%) Fusion of tricuspid valve leaflets Attachment is displaced downward Plastering of the leaflets to the RV wall

11 Atrioventricular canal type
Extremely rare (0.2%) Leaflet of the common AV valve seals off the only entrance into RV


13 RA & ASD The right atrium is enlarged and hypertrophied.
Interatrial communication is necessary for survival Stretched patent foramen ovale - ¾ cases True ASD less common - ostium secundum type

14 Rarely patent foramen ovale is obstructive and may form an aneurysm of fossa ovalis
Sometimes large enough to produce mitral inflow obstruction

15 LA & LV Left atrium may be enlarged, especially when pulmonary blood flow is increased. Mitral valve is morphologically normal - rarely incompetent LV is enlarged and hypertrophied

16 Right ventricle Size of the RV varies – depends on size of VSD
With a large VSD or TGA - RV larger When VSD is small - only the conus is present Small and hypoplastic Inflow or sinus portion absent Trabecular portion & outflow or conus region often well developed

17 VSD in Tricuspid Atresia
Associated VSD - 90% of individuals during infancy Usually perimembranous Can be muscular /malalignment types Restrictive VSD’S cause subpulmonic obstruction in pts with NRGA subaortic obstruction in pts with TGA

18 Majority of defects close in the first yr of life
At birth VSD is usually restrictive- permitting adequate but not excessive PBF 40% of these defects close spontaneously/ decrease in size - acquired pulmonary atresia This advantage is often lost, as Majority of defects close in the first yr of life

19 Classification - KUHNE
Type Normally related great arteries Type D-transposition of great arteries Type L- Transposition of great arteries

20 Type 1 Normally related great arteries (70 – 80%)
a. Intact IVS with pulmonary atresia( 9%) b. Small VSD and pulmonary stenosis( 51%) c. Large VSD without pulmonary stenosis ( 9%)


22 Type II D-transposition of great arteries (12 – 25%) a. VSD with pulmonary atresia( 2%) b. VSD with pulmonary stenosis( 8%) c. VSD without pulmonary stenosis(18%)


24 Type 3 L- Transposition or malposition of great arteries (3-6%)
Associated with complex lesions Truncus arteriosus Endocardial cushion defect

25 Additional cardiovascular abnormalities- 20%
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,MVP ,direct attachment of leaflets to papillary muscles

26 PHYSIOLOGY- TA Obligatory rt to left shunt at atrial level
LA receives both the entire systemic and pulmonary venous return Entire mixture flows into LV - sole pumping chamber for pulmonary and systemic circulation

27 PHYSIOLOGY TA WITH NRGA Pulm artery blood flow is usually reduced
Restrictive VSD - zone of subpulmonic stenosis. LV overload is curtailed but at the cost of cyanosis 90% of cases When the great arteries are normally related,

When VSD is non restrictive and pulmonary vascular resistance is low PBF and LV volume over load - excessive Cyanosis is mild

29 TA with TGA PHYSIOLOGY VSD is almost always non-restrictive and PS usually absent Low PVR > abundant pulmonary arterial blood flow Minimal cyanosis,marked LV volume overload With restrictive vsd or infundibular narrowing →diminished syst circulation →metabolic acidosis and shock If these pts have a

30 SEX PREDILECTION TA with NRGA - Equal frequency in males & females
TA with TGA - male preponderance - no male preponderance with juxtaposition of atrial appendages

31 GENETICS Specific genetic causes - remain to be determined in humans
FOG2 gene may be involved Validated only in animal studies 22q11 deletion Familial recurrence is low Recurrence in siblings is only about 1%

32 Overall survival in infants with TA
1 year- 72%. 5 years- 52%. 10 years- 46%

33 NATURAL HISTORY TA with NRGA with an intact IVS
Few infants survive beyond 6 months without surgical palliation Intense hypoxia and death ensue unless ductus is patent unless adequate systemic to PA collaterals present

34 Aquired pulmonary atresia
Due to spontaneous closure of VSD Usually in the first year Survival depends on patency of the ductus

35 TA with NRGA & SMALL VSD NATURAL HISTORY VSD closes spontaneously or become excessively obstructive - majority die by one year Rarely a favorable balance achieved b/w VSD & PBF permitting survival from 2nd to 5th decades

36 TA with NRGA & LARGE VSD. NATURAL HISTORY Excessive PBF > vol. overload of LV and CCF Patients usually do not fare well Some have lived to ages 4 to 6 years Long survivals reported between ages 32 and 45 yrs - in exceptional cases

37 TR. ATRESIA WITH TGA NATURAL HISTORY Same poor longevity patterns hold for TA with TGA and large VSD Exceptional survivals to mid-late teens reported TA with TGA with subaortic stenosis ( restrictive VSD) - ominous combination problems related to increased longevity-I.E,brain abscess, paradoxical embolism

38 Physical examination- appearance
Dysmorphic facies - Cat-eye syndrome - congenital coloboma JVP - a wave amplitude increase due to restrictive interatrial communication. - Y descent is slow

39 Precordium LV impulse without an RV impulse in a cyanotic patient
Gentle RV impulse - TA with non restrictive VSD and a well developed RV Palpable thrill if VSD is restrictive Key to clinical recognition of ta

40 AUSCULTATION First heart sound is single
Second usually single - soft pulmonic component - occasionally present TA with NRGA - prominent murmur of restrictive VSD – holosystolic maximal at mid to lower LSB

41 TA with TGA & increased PBF
AUSCULTATION Holosystolic murmur – across VSD S2 – single & loud S3 MDM

42 Pulmonary vascular resistance – high
AUSCULTATION VSD murmur vanishes Soft midsystolic murmur- anterior aortic root Rarely, the loud second component from the dilated hypertensive posterior pulmonary trunk is heard

43 - coexisting pulmonic or subpulmonic stenosis
AUSCULTATION TA with TGA - coexisting pulmonic or subpulmonic stenosis - midsystolic murmur – loudness and length vary inversely with degree of obstruction

44 ECG Tall peaked right atrial P waves - Himalayan P waves QRS axis
LV hypertrophy Absence of RV forces in precordial leads QRS axis - left and superior - type 1 - LAD or normal - type 2

Pulmonary vascularity reduced Pulmonary artery segment – inconspicuous Heart size – normal Right cardiac border superior convexity - enlarged RA Inferior part flat or receding - absence of RV

46 LAO – Humped appearance of right cardiac border

47 CXR - TA with TGA - no obstruction
Lungs – plethoric LV, LA, RA – enlarged Right cardiac border no hump-shaped contour – RV is relatively well developed

48 CXR - TA with TGA and PS Pulmonary blood flow is normal or reduced
Prominent RA Convex LV Narrow vascular pedicle

49 ECHOCARDIOGRAM Presence of an imperforate linear echo density at the location of normal TV Presence and size of the interatrial communication Presence and size of a VSD Relationship of the aorta and pulmonary artery

50 Size of the RV and pulmonary arteries
Presence and severity of infundibular or pulmonary stenosis Presence and size of the ductus arteriosus Presence of aortic isthmus narrowing or coarctation Degree of mitral regurgitation Left ventricular function

Limited role at present Newborn Define sources of pulmonary blood flow Associated anomalies not clearly defined by echo TA with TGA - Obstruction at VSD or infundibulum Therapeutic role for balloon atrial septostomy that will result in obstruction to systemic blood flow

Prior to Fontan - Pulm.Vascular resistance - Pulmonary artery size - Pulmonary artery distortion - by previous surgery Older patients without definitive palliation - detect collaterals from aorta - lungs

53 HEMODYNAMIC DATA Right atrial pressure is slightly higher than LAP
Prominent ‘a ‘wave in the right atrium -interatrial communication is restrictive LV systolic and EDP – normal LVEDP may increase with large VSD as PVR drops and LV volume overload ensues

54 Oxygen saturation (Sao2) in systemic venous return - lower than normal
- diminished Sao2 in systemic arterial blood Sao2 of pulmonary venous return - normal LA and LV saturations - diminished - obligatory R-L shunt Oxygen saturation in the systemic venous return will be lower than normal as a result of diminished oxygen saturation in the systemic arterial blood. oxygen saturation of pulmonary venous return will be normal. of the obligatory right-to-left atrial-level shunt, left atrial and left ventricular saturations will be diminished.

PGE1 Maintain patency of the ductus before cardiac catheterization or planned surgery Given as an infusion .025 – 0.1 mg/kg/mt Potential for apnea Fever , siezure, hypotension REFER should be started in neonates with severe cyanosis to

56 Balloon atrial septostomy may be done as part of initial catheterization to improve the RA-LA shunt

57 SURGICAL CARE Palliative Corrective therapy

Decreased pulmonary flow Increased pulmonary flow

59 FOR ↓ PBF PBF - increased by surgical creation of an aortopulmonary shunt Blalock & Taussig - Subclavian artery - ipsilateral PA Potts - Descending aorta – LPA Waterston-Cooley - Ascending aorta – RPA

60 Modified Blalock-Taussig shunt
- Gore-Tex interposition graft - Subclavian artery - PA Central aortopulmonary fenestration - Gore-Tex shunt

61 Glenn shunt Superior vena cava – RPA End-to- end Improves PBF and Sa o2 No risk of pulmonary HTN Pulmonary AV malformations later

62 ↑ PBF Pulmonary artery banding - In patients with
tricuspid atresia type II

63 CORRECTIVE SURGERY Fontan and Kreutzer
- physiologically corrective operation - complete separation of the systemic and pulmonary circuits

64 CHOUSSAT CRITERIA 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 - > = 15 mm Hg

65 Low pulmonary vasc resistance - 4 woods units/m2
Adequate sized PA with diameter > 75% of aorta Normal LVEF (>60%) Absence of MR Absence of complicating factors from previous surgeries Normal LVEF (>60%)( rel. contraindication) Absence of MR( relative contraindication)


67 Low cardiac output,heart failure or both Persistent pleural effusion
LATE COMPLICATIONS OF FONTAN EARLY COMPLICATIONS OF FONTAN Low cardiac output,heart failure or both Persistent pleural effusion Thrombus formation in the systemic venous pathways Liver dysfunction 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


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