Pulmonic Stenosis

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