1 / 66

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

ekram
Download Presentation

TRICUSPID ATRESIA

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related