Pulmonary Valve Stenosis

Key Features

Essentials of Diagnosis

• Asymptomatic unless lesion is at least moderately severe

• Severe cases may present with right-sided heart failure

• High-pitched systolic ejection murmur maximal in the second left interspace with radiation to the left shoulder

• P₂ delayed and soft or absent

• Pulmonary ejection click often present and decreases with inspiration—the only right heart sound that decreases with inspiration; all other right heart sounds increase

• Echocardiography/Doppler is diagnostic

General Considerations

• Often congenital and associated with other cardiac lesions

• Pulmonary blood flow preferentially goes to the left lung in valvular pulmonic stenosis

• In the absence of associated shunts, arterial saturation is normal

• Peripheral pulmonic stenosis can accompany valvular pulmonic stenosis and may be part of a variety of clinical syndromes, including the congenital rubella syndrome

• Noncongenital postoperative pulmonic valvular or main pulmonary artery (PA) stenosis may occur in patients who have had the Ross procedure for aortic valve disease due to an immune response in the homograft

• Right ventricular (RV) outflow obstructions can occur when there is a conduit from the RV to the PA that becomes stenotic from degenerative changes over time or when there is degeneration of a bioprosthetic replacement pulmonary valve

Clinical Findings

• Mild cases are asymptomatic

• Moderate to severe cases may cause

  • Dyspnea on exertion

  • Syncope

  • Chest pain

  • RV failure (eventually)

• There is often a palpable parasternal lift due to RV hypertrophy, and the pulmonary outflow tract may be palpable if the PA is enlarged

• A loud, harsh systolic murmur and occasionally a prominent thrill are present in the left second and third interspaces parasternally

• The murmur radiates toward the left shoulder due to the flow pattern within the main PA and increases with inspiration

• In mild to moderate pulmonic stenosis,

  • A loud ejection click can be heard to precede the murmur

  • This sound decreases with inspiration as the increased RV filling from inspiration prematurely opens the valve during atrial systole when inspiratory increased blood flow to the right heart occurs

    • The valve excursion during systole is thus less with inspiration than with expiration, and the click is therefore less audible with inspiration

    • This is the only right-sided auscultatory event that decreases with inspiration

    • All of the other auscultatory events increase with the increased right heart output that occurs with inspiration

• In severe pulmonic stenosis,

  • The second sound is obscured by the murmur and the pulmonary component of S₂ may be diminished, delayed, or absent

  • A right-sided S₄ gallop and a prominent a wave in the venous pulse are present when there is RV diastolic dysfunction

  • A c-v wave may be observed in the jugular venous pressure if tricuspid regurgitation is present

  • Right-sided S₄ gallops may be best heard in the right subclavicular area (where left-sided gallops would be distinctly ...