Pulmonic Valvular Stenosis Clinical Presentation

Updated: Dec 19, 2016
  • Author: Melanie A Loewenthal, MD; Chief Editor: Robert E O'Connor, MD, MPH  more...
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Presentation

History

Adults presenting with symptoms of progressive outflow tract obstruction due to pulmonic valvular disease were most likely born with some congenital malformation that was not diagnosed as a child. [4]

Signs of pulmonic valvular stenosis include the following:

  • History of a heart murmur since birth
  • Cyanosis
  • Dyspnea
  • Fatigue
  • Dizziness or syncope, occasionally
  • Chest pain
  • Mental retardation/developmental disorders

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 [18] :

  • Higher resting heart rate ( P = 0.001)
  • Lower peak power ( P = 0.006)
  • Lower peak oxygen uptake (V O2) ( P = 0.011) and lower oxygen uptake efficiency slope ( P = 0.007)
  • Higher ventilatory equivalent for carbon dioxide (VE/V CO2) ( P = 0.01)

The investigators indicated an observed linear increase in the peak pulmonary valve gradient may suggest a fixed valve area during exercise. [18] They also reported no signs of right heart functional or morphologic changes during exercise, with good ventricular performance.

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Physical

Physical examination findings correlate with the severity of right ventricular outflow obstruction.

The first heart sound is normal and followed by a systolic ejection click. The systolic ejection click is variable with respiration and louder on expiration. It is loudest over the left upper sternal border. [1, 6] The murmur of pulmonic stenosis is of the systolic ejection type and is best heard at the second left intercostal space. [11] Patients with dysplastic valves may not have a systolic ejection click. If the valve is pliant, a systolic ejection click is often heard.

The second heart sound is split. 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.

Systolic ejection murmur (crescendo-decrescendo), grade 2-5/6, is audible at the left upper sternal border, transmitting into the back and posterior lung fields. The murmur is heard best in the first to third intercostal spaces. [3, 6] The murmur usually does not radiate to the left sternal boarder.

The severity of valvular disease is related directly to the intensity and duration of the murmur. When severe, murmur extends into diastole (beyond the second heart sound).

Severe pulmonic valvular stenosis is associated with tricuspid insufficiency and may be associated with elevated central venous pressure, hepatosplenomegaly, a pulsatile liver, jugular venous pulsations, and hepatojugular reflux. Hepatosplenomegaly may develop in cases of CHF.

Significant pulmonic stenosis is characterized by a prominent jugular venous a wave and a right ventricular lift

Myocardial infarction of hypertrophied right ventricle may occur. [19]

Cyanosis may occur with right-to-left shunting at the atrial level as with a patent foramen ovale or septal defect. [11]

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Causes

Pulmonic valvular stenosis primarily results from a maldevelopment of the pulmonic valve tissue and the distal portion of the bulbus cordis. One maldevelopment is characterized by fusion of leaflet commissures, resulting in a domed appearance to the valve. Other etiologies result in dysplastic valves, which do not open and close normally. [19]

Coexisting cardiac malformations (eg, ventricular septal defect, atrial septal defect, patent ductus arteriosus) may complicate the anatomy, physiology, and clinical picture.

Aberrant flow patterns in utero may be associated, in part, with maldevelopment of the pulmonary valve.

Rubella embryopathy may cause pulmonic valvular stenosis.

Family history is a mild risk factor. [20]

Cases have been reported in the setting of congenital syndromes such as Mayer-Rokitansky-Kuster-Hauser syndrome. [21]

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