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Common Congenital Heart Lesions - page 23 / 126

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Stenotic Semilunar Valve Disease Pulmonic Stenosis (PS)

Clinical Presentation:

  • Only PS with moderate to severe gradients are symptomatic.

  • PS patients live a normal life and have no real complications.

Physical Exam:

  • Typical findings:

    • o

      Auscultation:

      • Pulmonic ejection sound at the pulmonic auscultatory area; becomes less obvious during inspiration and is very obvious during expiration (unusual for a pulmonic sound).

      • More severe PS gives wider S2 split.

      • As with A2, the worse the stenosis gets, the less likely you are to get/hear a P2 sound – with to much stenosis, valve won’t even close to generate a sound.

      • o

        Palpation: precordial heave or palpable impulse from the RV may be felt at the left lower sternal border, suggesting moderately severe to severe PS. In the left upper sternal border (2nd intercostals space), a systolic thrill may be palpable.

Etiology:

  • PS is rare and it is almost always due to congenital deformity of the valve.

  • Calcific valvular changes cause progression to PS.

  • As with AS, PS has three possible types of congenital deformities of the valve:

    • o

      1/ Congenitally malformed valve

      • o

        2/ Subvalvular PS

      • o

        3/ Supravalvular PS

Pathophysiology:

  • Pressure gradients between the RV and the pulmonary outflow tract determine the severity of the PS: omild PS cases: gradient < 40 mmHg omoderate PS: gradient of 40 – 80 mmHg (may be symptomatic) osevere PS: gradient > 80 mmHg (symptomatic)

Diagnostic imaging/testing:

  • Electrocardiogram:

    • o

      Can show increased QRS voltage in V1, inverted T waves also over the right precordium

      • o

        Can show RV hypertrophy by axis deviation – depending on the severity of the PS

  • Chest X-ray:

owith angiogram…see doming of the pulmonic valve. oConcentric hypertrophy of right heart, so no real heart size change seen. oMay see a little bit of encroachment of the right ventricle on the mediastinal air space

Treatment:

  • Cardiac Catheterization:

    • o

      Transcatheter balloon valvuloplasty is usually effective therapy for patients with severe or symptomatic PS.

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