Pulmonary stenosis

Pulmonary stenosis (PS) is mostly congenital.

Symptoms range from none to profound cyanosis and the potential for sudden death.

Systolic ejection murmur is present and is loudest over left upper sternal border.

Cyanotic patients are treated with oxygen and prostaglandin E1 prior to diagnostic testing.

The diagnosis is confirmed and severity classified by echocardiography.

Mild PS is a benign condition requiring sequential cardiac follow-up but no therapy.

Percutaneous balloon pulmonary valvuloplasty (PBPV) is indicated in moderate to severe/critical lesions.

Surgical valvotomy is reserved for treatment failure and contraindication to PBPV.

PS obstructs the blood flow from the right ventricle (RV) into the pulmonary bed, resulting in a peak pressure gradient greater than 10 mmHg across the pulmonary valve during systole. In most cases PS is found at the level of the valve, but it can also occur proximal to the valve (subpulmonary stenosis) or distally in the pulmonary arteries (suprapulmonary stenosis). PS is commonly associated with syndromes such as Noonan syndrome and with other forms of more complex congenital heart disease (such as tetralogy of Fallot).​[1]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 Oct;22(4):1189-92. http://www.sciencedirect.com/science/article/pii/0735109793904365?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/8409059?tool=bestpractice.com [2]Ruckdeschel E, Kim YY. Pulmonary valve stenosis in the adult patient: pathophysiology, diagnosis and management. Heart. 2019 Mar;105(5):414-22. https://www.doi.org/10.1136/heartjnl-2017-312743 http://www.ncbi.nlm.nih.gov/pubmed/30337335?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.