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

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Presentation on theme: "TRICUSPID ATRESIA Dr Bijilesh u Senior Resident, Dept. of Cardiology, Medical College, Calicut."— Presentation transcript:

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

2 - 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 References

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 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

12 MORPHOLOGICAL CONSIDERATIONS

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 Small and hypoplastic Inflow or sinus portion absent Trabecular portion & outflow or conus region often well developed 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

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

19 Classification - KUHNE Type 1 Normally related great arteries Type 2 D-transposition of great arteries Type 3 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%)

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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%)

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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

27 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 PHYSIOLOGY

28 When VSD is non restrictive and pulmonary vascular resistance is low PBF and LV volume over load - excessive Cyanosis is mild PHYSIOLOGY TA WITH NRGA

29 TA with TGA 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 PHYSIOLOGY

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 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 TA with NRGA with an intact IVS

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 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 NATURAL HISTORY

36 TA with NRGA & LARGE VSD. 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 NATURAL HISTORY

37 TR. ATRESIA WITH TGA 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 NATURAL HISTORY

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

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 Holosystolic murmur – across VSD S2 – single & loud S3 MDM AUSCULTATION

42 Pulmonary vascular resistance – high VSD murmur vanishes Soft midsystolic murmur- anterior aortic root AUSCULTATION

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

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

45 CHEST X-RAY- TA WITH NRGA AND SMALL VSD 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

51 CARDIAC CATHETERIZATION 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

52 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 CARDIAC CATHETERIZATION

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

55 INITIAL MEDICAL MANAGEMENT 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 PGE1

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

57 SURGICAL CARE Palliative Corrective therapy

58 PALLIATIVE SURGERY DECIDED IN TERMS OF 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

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

68 THANK YOU.


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