Also called peripheral pulmonic stenosis, a congenital heart defect characterized by narrowing of pulmonary arteries and distal branch arteries, more commonly diagnosed in children. May occur in multiple or single lesions, extensive or localized, and often with other congenital heart defects, such as atrial septal defect
, patent ductus arteriosus
, supravalvular aortic stenosis
, tetralogy of Fallot
, valvular pulmonic stenosis
, or ventricular septal defect
. Associated with congenital rubella
. Genetic associations include Alagille syndrome
and Williams-Beuren syndrome. Signs and symptoms include dyspnea, failure to thrive, lethargy, and systolic ejection murmur. Adults may present with progressive dyspnea, fatigue, syncope, and heart murmurs, often in conjunction with congenital disorders, and, if misdiagnosed and left untreated, stenosis may lead to pulmonary hypertension.
Management of mild cases may call for a conservative approach with monitoring or catheter balloon dilation. More severe cases, particularly with other valve defects, may call for corrective surgery, stent implantation, or surgical patch arterioplasty, alone or in combination.