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Common Congenital Heart Lesions - page 7 / 126

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5. Congential Pulmonic Stenosis Clinical Presentation: Mild or moderate PS: asymptomatic, diagnosis made based on murmur on routine physical exam. Compensated severe PS: exertional dyspnea, exercise intolerance. Uncompensated severe PS: symptoms of right-sided heart failure (abdominal fullness and pedal edema)

Physical Findings: For severe pulmonic stenosis:

  • 1.

    RVH and RV heave over left sternum

  • 2.

    prominent jugular venous “a” wave

  • 3.

    Loud, late-peaking, crescendo-decrescendo systolic ejection murmur heard

over left sternal border, associated with palpable trill 4. Widened splitting of S2 with soft P2 (delated closure of pulmonic valve) For moderate PS:

  • 1.

    Pulmonic ejection click following S1 and preceding systolic murmur resulting from opening of stenotic pulmonic valve. Click diminishes with inspiration.

  • 2.

    Loud, late-peaking, crescendo-decrescendo systolic ejection murmur heard over left sternal border

Diagnostic Imaging and Testing:

  • A.

    Chest x-ray: enlarged RA and RV, post-stenotic PA dilation (from high velocity blood hitting vessel wall)

  • B.

    ECG: RVH and Right axis deviation

  • C.

    Echo+ Doppler: visualize valve, look for presence of RV, and assess magnitude of obstruction

Treatment: Mild shows no progression, therefore only moderate and severe require treatment: If pulmonic valve is the problem, then dilate valve with transcatheter balloon valvuloplasty, results are excellent, RVH regresses. Also, treat patients with antibiotic prophylaxis to prevent endocarditis.

Etiology: Narrowing or obstruction of pulmonary outflow tract. Arises from: 1. Pulmonic Valve problem (>90%): Congenital fusion of the pulmonic valve commisures

  • 2.

    R Ventricle problem: abnormal configurationÆ obstruction of outflow tract

  • 3.

    Pulmonary Artery problem: narrowing of vessel or obstruction of lumen

Pathology: RVH, RA enlargement, dilation of PA post-stenosis

Pathophysiology: PS decreases RV systolic ejection, increases RV pressure, and increases RV chamber hypertrophy. Clinical course depends on severity of obstruction, which is measured by pressure difference on either side of the stenotic area during systole (AKA: peak systolic transvalvular pressure gradient). Assuming normal CO: Mild: <50 mmHg Moderate: 50-80 mmHg Severe: >80 mmHg

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