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← Section IV · Congenital Heart Disease
IV.C

Tricuspid Valve Anomalies

6 cards

Notes

Ebstein's anomaly

  • Apical displacement of the septal (± posterior) leaflet > 8 mm/m² BSA or > 20 mm absolute displacement from the plane of the mitral valve annulus.
  • Atrialization of the proximal RV (portion of RV becomes functionally part of the RA).
  • The anterior TV leaflet is redundant, elongated, sail-like ("hang-glider" appearance).
  • Right atrium is markedly enlarged.
  • Severe TR is nearly universal.
  • Right-to-left shunt at PFO/ASD → cyanosis.
  • Associations:
    • WPW (~25 %) - accessory pathway on the tricuspid annulus.
    • Cystic disease (rare).
    • Cctga (left-sided Ebstein's-like tricuspid displacement).
  • Surgical options: cone reconstruction (Da Silva), Glenn shunt (in severe cases), or biventricular repair.

Tricuspid atresia

  • Absent tricuspid valve replaced by a fibromuscular band.
  • Small hypoplastic RV.
  • Obligatory ASD/PFO for right-to-left atrial shunt.
  • VSD often present, directing LV output to the small RV → PA.
  • Cyanotic (variable degree).
  • Palliation → Fontan operation.

TV dysplasia

  • Thickened, myxomatous leaflets with variable regurgitation.
  • Associated with congenital dysplastic TV, Uhl's anomaly (paper-thin RV with dysplastic TV).

Isolated congenital tricuspid regurgitation

  • Rare; may be seen in isolation or with elevated RA pressures from other congenital lesions.

Cards

  • basicIV.C-001
    State the diagnostic echo criterion for Ebstein's anomaly (septal leaflet displacement).
    → Apical displacement of the septal (± posterior) tricuspid leaflet > 8 mm/m² BSA or > 20 mm absolute from the mitral annular plane. Anterior TV leaflet appears redundant and sail-like.
  • basicIV.C-002
    What is 'atrialization' of the RV in Ebstein's anomaly?
    → The portion of the RV between the true tricuspid annulus (fibrous ring) and the displaced functional leaflet is thin-walled and behaves hemodynamically as part of the right atrium.
  • basicIV.C-003
    What accessory pathway condition is commonly associated with Ebstein's anomaly?
    → Wolff-Parkinson-White syndrome — present in ~25% of Ebstein's patients. The pathway sits on the tricuspid annulus.
  • basicIV.C-004
    Echo findings of tricuspid atresia?
    → 1) Absent tricuspid valve, replaced by echo-dense fibromuscular band. 2) Small, hypoplastic RV. 3) Obligate ASD or PFO with right-to-left shunt. 4) Often a VSD providing pulmonary blood flow.
  • basicIV.C-005
    What surgical pathway do most patients with tricuspid atresia follow?
    → Single-ventricle palliation → Fontan operation (or total cavopulmonary connection).
  • basicIV.C-006
    How is the coumadin-like sail (anterior TV leaflet) in Ebstein's described morphologically?
    → Redundant, elongated, and 'hang-glider' or 'sail-like' — often with tethering to the RV free wall by muscular tags rather than normal chordae.