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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Systolic murmur at the left second intercostal space preceded by a systolic ejection sound

  • Reduced intensity of pulmonic component of S2

  • Characteristic echocardiographic findings

GENERAL CONSIDERATIONS

  • Pulmonic stenosis is almost always congenital and valvular, although supravalvular and subvalvular lesions do occur

  • The condition can occur with other congenital lesions/conditions such as tetralogy of Fallot and Noonan’s syndrome

  • The most commonly acquired form occurs with the carcinoid syndrome

  • Rheumatic heart disease rarely involves the pulmonic valve

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Often asymptomatic

  • Fatigue

  • Exertional dyspnea

PHYSICAL EXAM FINDINGS

  • Prominent a wave in the jugular venous pressure

  • Right ventricular lift

  • Pulmonic ejection sound that gets softer with inspiration

  • Reduced pulmonic component of S2

  • Right-sided S4 along the left sternal border

  • Systolic ejection murmur in the pulmonic area that increases with inspiration

DIFFERENTIAL DIAGNOSIS

  • Aortic stenosis

  • Ventricular septal defect

  • Right-heart failure from other causes

DIAGNOSTIC EVALUATION

ELECTROCARDIOGRAPHY

  • Evidence of right ventricular and atrial hypertrophy

IMAGING STUDIES

  • Chest x-ray: right heart chamber enlargement with dilatation of the main pulmonary artery

  • Echocardiography:

    • – Thickened, doming, or dysplastic pulmonic valve that has an increased Doppler-determined pressure gradient; > 64 mm Hg considered severe

    • – Right ventricular hypertrophy and right atrial enlargement common

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization: rarely done for diagnosis today, but can confirm the gradient across the valve when echo is unclear

TREATMENT

CARDIOLOGY REFERRAL

  • Symptomatic patients

HOSPITALIZATION CRITERIA

  • Presurgery in patients not treated by balloon valvuloplasty

THERAPEUTIC PROCEDURES

  • Percutaneous balloon valvuloplasty is the treatment of choice for symptomatic patients

  • Percutaneous valve replacement in selected cases with dysplastic valves

SURGERY

  • If valvuloplasty is unsuccessful or if other lesions need correction, surgical valvuloplasty and rarely valve replacement can be done

  • Valve replacement is usually with a homograft or a bioprosthetic valve

MONITORING

  • ECG monitoring in the hospital

DIET AND ACTIVITY

  • Low-sodium diet

ONGOING MANAGEMENT

HOSPITAL DISCHARGE CRITERIA

  • After successful procedure or surgery

FOLLOW-UP

  • Depends on severity in asymptomatic patients: 3–12 months with echocardiography every other visit

COMPLICATIONS

  • Infective endocarditis—rare

  • Right heart failure in severe cases

PROGNOSIS

  • Excellent prognosis before and after correction

  • Often determined by other associated lesions

  • Repeat balloon valvuloplasty rate ...

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