Mostly congenital.
Symptoms ranging from none to profound cyanosis and the potential for sudden death.
Systolic ejection murmur, loudest over left upper sternal border.
Cyanotic patients treated with oxygen and prostaglandin E1 prior to diagnostic testing.
Diagnosis confirmed and severity classified by echo.
Mild PS is a benign condition requiring sequential cardiac follow-up but no therapy; prophylaxis for infective endocarditis not recommended.
Percutaneous balloon pulmonary valvuloplasty (PBPV) indicated in moderate to severe/critical lesions.
Surgical valvotomy reserved for treatment failure and contraindication to PBPV.
Pulmonary stenosis (PS) obstructs the blood flow from the right ventricle (RV) into the pulmonary bed, resulting in a pressure gradient greater than 10 mmHg across the pulmonary valve during systole. In 80% to 90% of all cases PS is found at the level of the valve, but it can also occur below the level of the valve or distally in the pulmonary arteries.
[1]
Latson LA, Prieto L. Pulmonary stenosis. In: Allen HD, Clark EB, Driscoll DJ. Moss and Adam's heart disease in infants, children and adolescents. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001(Vol 2):821-844.
PS is commonly associated with other forms of congenital heart disease.
[2]
Rosamond W, Flegal K, Friday G, et al. Heart disease and stroke statistics - 2007 update: a report from the American Heart Association Statistics Committee and Stroke Statistics Subcommittee. Circulation. 2007 Feb 6;115(5):e69-171.
http://circ.ahajournals.org/cgi/content/full/115/5/e69
http://www.ncbi.nlm.nih.gov/pubmed/17194875?tool=bestpractice.com
[3]
Burch M, Sharland M, Shinebourne E, et al. Cardiologic abnormalities in Noonan syndrome: phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol. 1993;22:1189-1192.
http://www.ncbi.nlm.nih.gov/pubmed/8409059?tool=bestpractice.com
The increased subpulmonary ventricular pressure may lead to hypertrophy of the RV proportional to the degree of stenosis. Clinical symptoms range from none in mild PS to profound cyanosis and the potential for sudden death in critical PS.