Pulmonary valve stenosis

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Pulmonary valve stenosis
Other names: Valvular pulmonary stenosis, pulmonic stenosis
Pulmonary valve stenosis.svg
SpecialtyCardiology
SymptomsFew symptoms initially, may develop syncope, chest pain, and shortness of breath
CausesCongenital heart disease, carcinoid syndrome[1]
Diagnostic methodSuspected based on a systolic murmur, confirmed by ultrasound of the heart[1]
TreatmentBalloon valvuloplasty, valve replacement[1]
PrognosisGenerally good[1]
Frequency0.4% at birth[2]

Pulmonary valve stenosis (PVS) is a type of heart valve disease in which there is narrowing of the pulmonary valve opening.[3] Affected children often have no symptoms.[1] When symptoms develop these may include syncope, chest pain, or shortness of breath.[1]

The most common cause is congenital heart disease such as tetralogy of Fallot and Noonan syndrome.[1] Other causes include carcinoid syndrome.[1] Diagnosis may be suspected based on a systolic murmur and confirmed by ultrasound of the heart.[1]

Treatment may may include balloon valvuloplasty and valve replacement.[1] Outcomes are generally good.[1] At birth it affected about 4 per 1,000 people.[2] Initial descriptions of the condition at birth date from 1761 by John Baptist Morgagni.[4] Surgical repair was first carried out in 1953.[4]

Signs and symptoms

Cynosis

Among some of the symptoms consistent with pulmonary valve stenosis are the following:[5]

Cause

In regards to the cause of pulmonary valve stenosis a very high percentage are congenital, the right ventricular flow is hindered (or obstructed by this). The cause in turn is divided into: valvular, external and intrinsic (when it is acquired).[6]

Both stenosis of the pulmonary artery and pulmonary valve stenosis are forms of pulmonic stenosis (nonvalvular and valvular, respectively)[7] but pulmonary valve stenosis accounts for 80% of pulmonic stenosis.

Pathophysiology

The pathophysiology of pulmonary valve stenosis consists of the valve leaflets becoming too thick (therefore not separate one from another), which can cause high pulmonary pressure, and pulmonary hypertension. This however, does not mean the cause is always congenital.[8]

The left ventricle can be changed physically, these changes are a direct result of right ventricular hypertrophy. Once the obstruction is subdued, it (the left ventricle) can return to normal.[9]

Diagnosis

Pulmonary valve stenosis as seen on echocardiogram

The diagnosis of pulmonary valve stenosis can be achieved via echocardiogram, as well as a variety of other means among them are: ultrasound, in which images of the heart chambers in utero where the tricuspid valve has thickening (or due to Fallot's tetralogy, Noonan's syndrome, and other congenital defects) and in infancy auscultation of the heart can reveal identification of a murmur.[10]

Some other conditions to contemplate (in diagnosis of pulmonic valvular stenosis) are the following:[5]

Treatment

In terms of treatment for pulmonary valve stenosis, valve replacement or surgical repair (depending upon whether the stenosis is in the valve or vessel) may be indicated. If the valve stenosis is of congenital origin, balloon valvuloplasty is another option, depending on the case. Valves made from animal or human tissue (are used for valve replacement), in adults metal valves can be used.[11][12]

Epidemiology

The epidemiology of pulmonary valve stenosis can be summed up by the congenital aspect which is the majority of cases, in broad terms PVS is rare in the general population.[10]

References

  1. 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 "Pulmonic Stenosis - Cardiovascular Disorders". Merck Manuals Professional Edition. Retrieved 23 December 2020. CS1 maint: discouraged parameter (link)
  2. 2.0 2.1 Benjamin, Ivor; Griggs, Robert C.; Fitz, J. Gregory (2015). Andreoli and Carpenter's Cecil Essentials of Medicine E-Book. Elsevier Health Sciences. p. 72. ISBN 978-0-323-35236-9.
  3. "Congenital heart disease - Types". nhs.uk. 19 October 2017. Retrieved 24 December 2020. CS1 maint: discouraged parameter (link)
  4. 4.0 4.1 Satpathy, M. (2015). Clinical Diagnosis of Congenital Heart Disease. JP Medical Ltd. p. 197. ISBN 978-93-5152-912-5.
  5. 5.0 5.1 "Pulmonic Valvular Stenosis Clinical Presentation: History, Physical, Causes". emedicine.medscape.com. Retrieved 2015-11-18. CS1 maint: discouraged parameter (link)
  6. Wang, Andrew; Bashore, Thomas M. (2010-01-14). Valvular Heart Disease. Springer Science & Business Media. p. 266. ISBN 9781597454117.
  7. Ren, XM; et al. (2014-12-23), "Pulmonic stenosis", Medscape.
  8. Levine, Shel; Coyne, Brian J.; Colvin, Lisa Cooper (2015-02-13). Clinical Exercise Electrocardiography. Jones & Bartlett Publishers. p. 14. ISBN 9781284034202.
  9. "Valvar Pulmonary Stenosis: Background, Pathophysiology, Epidemiology". 2018-06-07. Cite journal requires |journal= (help)
  10. 10.0 10.1 "Pulmonary Valve Disease. About Pulmonary valve disease | Patient". Patient. Retrieved 2015-11-18. CS1 maint: discouraged parameter (link)
  11. Choices, NHS. "Congenital heart disease - Treatment - NHS Choices". www.nhs.uk. Retrieved 2015-11-18. CS1 maint: discouraged parameter (link)
  12. "Balloon dilatation of pulmonary valve stenosis | Guidance and guidelines | NICE". www.nice.org.uk. Retrieved 2015-11-18. CS1 maint: discouraged parameter (link)

External links

Classification
External resources