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Pulmonary Valve Stenosis

Inderjit Singh, MD and Navpreet K. Singh, MD Reviewed 04/2024
 


BASICS

DESCRIPTION

Deformity of pulmonary valve, most commonly congenital, resulting in obstruction of right ventricular (RV) outflow 

EPIDEMIOLOGY

Incidence

  • Predominant age: congenital, present in newbor...

DIAGNOSIS

HISTORY

  • History of heart murmur since birth

  • Usually asymptomatic (mild to moderate stenosis); exercise tolerance is normal unless stenosis is severe.

  • If stenosis is critical, patient may have f...

TREATMENT

Described in “Surgery/Other Procedures” 

GENERAL MEASURES

Prophylaxis for subacute bacterial endocarditis is not recommended for pulmonary valve stenosis unless the patient has associated cong...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

The American College of Cardiology and the American Heart Association recommend that (1,2)[C]: 
  • For valvular stenosis:

    • Asymptomatic patients with a peak instantane...

REFERENCES

1
Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC guideline for the management of adults with congenital heart disease: executive summary: a report of the American College of Cardiol...

ADDITIONAL READING

Nishimura  RA, Otto  CM, Bonow  RO, et al. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: a report of the American College of...

SEE ALSO

Noonan Syndrome with Multiple Lentigines (LEOPARD Syndrome); Tetralogy of Fallot; Williams-Beuren Syndrome 

CODES

ICD10

  • Q22.1 Congenital pulmonary valve stenosis

  • I37.0 Nonrheumatic pulmonary valve stenosis

  • I09.89 Other specified rheumatic heart diseases

  • T82.857A Stenosis of cardiac prosthetic devices, implants ...

CLINICAL PEARLS

  • Pulmonary stenosis is a common congenital condition that can be isolated or a part of other syndromes.

  • The most common cause is congenital rubella infection.

  • Vaccinating the population (...

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