Pulmonic Valvular Stenosis Clinical Presentation

Updated: Aug 17, 2023
  • Author: Victoria Zaccone, MD; Chief Editor: Barry E Brenner, MD, PhD, FACEP  more...
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Pulmonic valvular stenosis (PVS) in adults may present as an asymptomatic systolic murmur, or they may complain of decreased exercise tolerance.

In a functional and hemodynamic study of 19 Belgian patients with isolated mild-to-moderate pulmonary valve stenosis but no previous cardiac interventions, investigators reported significant differences in the following at-rest and exertional parameters between patients and their age- and sex-matched controls [23] :

  • Higher resting heart rate (P = 0.001)

  • Lower peak power (P = 0.006)

  • Lower peak oxygen uptake (VO2) (P = 0.011) and lower oxygen uptake efficiency slope (P = 0.007)

  • Higher ventilatory equivalent for carbon dioxide (VE/VCO2) (P = 0.01)

The investigators indicated an observed linear increase in the peak pulmonary valve gradient may suggest a fixed valve area during exercise. [23] They also reported no signs of right heart functional or morphologic changes during exercise, with good ventricular performance.


Physical Examination

For most adults with pulmonic valvular stenosis (PVS), there are no classic phenotypic findings, and disease is suspected during cardiac examination.

For suspected Noonan syndrome, phenotypes include the following:

  • Facial anomalies such as low set ears and hairline, hypertelorism, micrognathia, webbed neck

  • Chest deformities (pectus excavatum)

  • Short stature

  • Intellectual disability

Cardiac examination findings correlate with the severity of stenosis, valve pathology, and other associated cardiac lesions. Rarely, overt right heart failure is seen (unless end-stage disease).

On auscultation, the first heart sound is normal and followed by a systolic ejection click loudest over the left upper sternal border. [11, 15] The murmur decreases with inspiration; this is secondary to the premature opening of the pulmonary valve by the atrial kick into a stiff right ventricle. [2]

The second heart sound may have a wide split, especially in severe disease. This is due to delayed closing of the pulmonic valve at the end of systole. The pulmonic component of the second heart sound may be diminished in intensity, and a right sided fourth heart sound may be heard.

In more severe pulmonic valvular stenosis, a right ventricular lift with associated jugular venous distention may be appreciated, and the pulmonary ejection murmur is much louder and longer.

Myocardial infarction of hypertrophied right ventricle may occur. [9] Cyanosis may occur with right-to-left shunting at the atrial level as with a patent foramen ovale or septal defect. [15]