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← Section IV · Congenital Heart Disease
IV.F

Pulmonic Valve Anomalies

5 cards

Notes

Congenital pulmonic stenosis

  • Types:
    • Valvular - most common; doming, thin leaflets; usually amenable to balloon valvuloplasty.
    • Dysplastic - Noonan syndrome; thick, immobile leaflets; resistant to balloon → surgical.
    • Subvalvular (infundibular) - often with TOF or DCRV (double-chambered RV).
    • Supravalvular - Williams syndrome, congenital rubella.
    • Peripheral / branch PS - Williams syndrome, Alagille syndrome, Noonan syndrome.

Grading

  • Mild: peak velocity < 3 m/s (peak gradient < 36 mmHg).
  • Moderate: 3–4 m/s (36–64 mmHg).
  • Severe: > 4 m/s (> 64 mmHg).

Balloon valvuloplasty

  • First-line for symptomatic or severe VALVULAR PS in most patients (except dysplastic).
  • Contraindicated in dysplastic PV of Noonan syndrome - surgical valvotomy preferred.

Absent pulmonary valve syndrome

  • Rudimentary or absent pulmonic valve tissue → severe PR with pulmonary insufficiency.
  • Massively dilated pulmonary arteries (aneurysmal main and branch PAs).
  • Often associated with VSD (variant of TOF).
  • Compression of tracheobronchial tree by dilated PAs → severe airway symptoms.

Post-TOF repair PR

  • Chronic free PR from a transannular RVOT patch is the most common valvular issue after TOF repair.
  • Long-term follow-up with cMRI to time pulmonary valve replacement (percutaneous or surgical).

DCRV (double-chambered RV)

  • Anomalous muscle bundles subdivide the RV into a proximal high-pressure chamber and a distal (RVOT) low-pressure chamber.
  • Often associated with a perimembranous VSD.
  • Progressive obstruction - may require surgical resection.

Cards

  • basicIV.F-001
    Which form of congenital pulmonic stenosis is resistant to balloon valvuloplasty and requires surgery?
    → Dysplastic pulmonic valve of Noonan syndrome — the thick, immobile, non-doming leaflets do not respond to balloon dilation. Surgical valvotomy or valve replacement is needed.
  • basicIV.F-002
    What is absent pulmonary valve syndrome?
    → Rudimentary or absent pulmonic valve leaflets → severe PR. Massively dilated main and branch pulmonary arteries. Often occurs with VSD (variant of TOF). May cause airway compression from dilated PAs.
  • basicIV.F-003
    Which chromosomal or syndromic conditions are classically associated with peripheral pulmonic stenosis?
    → Williams syndrome (elastin deletion), Alagille syndrome, Noonan syndrome, congenital rubella syndrome.
  • basicIV.F-004
    What is a double-chambered right ventricle (DCRV)?
    → Anomalous muscle bundles subdivide the RV into a proximal high-pressure chamber and a distal low-pressure outflow chamber. Frequently associated with a perimembranous VSD. Progressive obstruction may require surgical resection.
  • basicIV.F-005
    After successful balloon pulmonic valvuloplasty in valvular PS, what mild residual finding is expected?
    → Mild pulmonic regurgitation is common and generally well tolerated.