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TRICUSPID ATRESIA

TRICUSPID ATRESIA. Dr Bijilesh u Senior Resident, Dept. of Cardiology, Medical College, Calicut . References. - Perloff ‘s text book of congenital heart diseases - Moss and Adams ‘s text book of congenital cardiology

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TRICUSPID ATRESIA

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  1. 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 tricuspidvalve & 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 - membranous septum forms floor of the RA at the expected location of TV • May be associated with • absent pulmonary valve leaflets

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

  11. MORPHOLOGICAL CONSIDERATIONS

  12. 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 - ostiumsecundum type

  13. Rarely patent foramen ovale is obstructive and may form an aneurysm of fossaovalis • Sometimes large enough to produce mitral inflow obstruction

  14. 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

  15. 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

  16. 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

  17. 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

  18. Classification- KUHNE • Type 1 Normally related great arteries • Type 2 D-transposition of great arteries • Type 3 L- Transposition of great arteries

  19. 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%)

  20. TypeII 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%)

  21. Type3 • L- Transposition or malposition of great arteries (3-6%) • Associated with complex lesions • Truncusarteriosus • Endocardial cushion defect

  22. 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

  23. 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

  24. PHYSIOLOGY TAWITHNRGA • Pulm artery blood flow is usually reduced • Restrictive VSD - zone of subpulmonicstenosis. • LV overload is curtailed but • at the cost of cyanosis • 90% of cases

  25. TAWITHNRGA PHYSIOLOGY • When VSD is non restrictive and pulmonary vascular resistance is low • PBF and LV volume over load - excessive • Cyanosis is mild

  26. TA withTGA 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

  27. SEXPREDILECTION • TA with NRGA - Equal frequency in males & females • TA with TGA • - male preponderance • - no male preponderance with • juxtaposition of atrial appendages

  28. 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%

  29. NATURALHISTORY 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

  30. TA with NRGA & SMALL VSD NATURALHISTORY • 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

  31. TA with NRGA & LARGE VSD. • NATURALHISTORY • 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

  32. TR.ATRESIAWITHTGA • NATURALHISTORY • Same poor longevity patterns hold for TA with TGA and large VSD • Exceptional survivals to mid-late teens reported • TA with TGA with subaorticstenosis • ( restrictive VSD) - ominous combination

  33. Overall survival in infants with TA • 1 year- 72%. • 5 years- 52%. • 10 years- 46% Franklin et al 1972 -1987, 237 patients

  34. Survival of patients presenting in infancy with tricuspid atresia to the Toronto Hospital for Sick Children, Tame et al , 101 patients, 1970 - 1984 Probability of surviving for 1 year was 64% and to 8 years was 55% The overall surgical mortality for the palliative procedures was 35.8%

  35. Physicalexamination-appearance • Dysmorphicfacies • - Cat-eye syndrome • - congenital coloboma • JVP • - a wave amplitude increase due to restrictive • interatrial communication. • - Y descent is slow

  36. 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

  37. 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

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

  39. Pulmonary vascular resistance – high • AUSCULTATION • VSD murmur vanishes

  40. AUSCULTATION • TA with TGA • - coexisting pulmonic or subpulmonicstenosis • - midsystolic murmur – loudness and length vary • inversely with degree of obstruction

  41. 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

  42. 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

  43. LAO – Humped appearance of right cardiac border

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

  45. CXR - TA with TGA andPS • Pulmonary blood flow is normal or reduced • Prominent RA • Convex LV • Narrow vascular pedicle

  46. 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

  47. Size of the RV and pulmonary arteries • Presence and severity of infundibular or pulmonary stenosis • Presence and size of the ductusarteriosus • Presence of aortic isthmus narrowing or coarctation • Degree of mitral regurgitation • Left ventricular function

  48. CARDIACCATHETERIZATION • 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 atrialseptostomy

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