Medication Summary

Surgery is the mainstay of treatment for significant stenosis. Antibiotics for endocarditis prophylaxis are given to patients with infundibular pulmonary stenosis (IPS) before performing procedures that may cause bacteremia. Endocarditis prophylaxis is recommended throughout life, even after surgical relief. For more information, see Antibiotic Prophylactic Regimens for Endocarditis. Heart failure therapy is indicated only as a temporary measure for patients with heart failure. Ensure adequate hydration before administering diuretics because the cardiac output depends on adequate preload; if necessary, use beta-adrenergic blockers to relax the infundibular muscles. Alprostadil minimizes cyanosis in neonates with ductal-dependent pulmonary circulation.

Class Summary

These agents inhibit beta1- and beta2-adrenergic receptors. They inhibit chronotropic, inotropic and vasodilatory responses to beta-adrenergic stimulation. Their exact mechanism of benefit is uncertain, although it is believed to relieve infundibular spasm.

Propranolol (Inderal)

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Inhibits beta1- and beta2-adrenergic receptors.

Class Summary

These agents are used to eliminate retained fluid and lower preload. They promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.

Furosemide (Lasix)

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Increases water excretion by interfering with Cl-binding cotransport system, which, in turn, inhibits NA and Cl reabsorption in ascending Henle loop and distal renal tubule. When administered IV, also has a venodilation action; thus lowering the preload even before diuresis sets in. DOC for acute heart failure and for exacerbations of chronic heart failure; used for long-term management of chronic heart failure.

Spironolactone (Aldactone)

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Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Amiloride (Midamor)

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Potassium-sparing diuretic acting directly on the distal renal tubule. Often combined with a nonsparing-potassium diuretic.

Class Summary

These agents are used to maintain patency of ductus arteriosus in patients with duct-dependent circulation.

Alprostadil IV (Prostin VR Pediatric)

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Acts on the smooth muscles of the ductus arteriosus and prevents its closure in response to elevation of arterial oxygen saturation. First-line medication used as palliative therapy to temporarily maintain patency of the ductus arteriosus prior to surgery. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent ductus arteriosus to get adequate oxygenation and lower body perfusion. Produces vasodilation and increases cardiac output. Each 1-mL ampule contains 500 mcg/mL. Used to maintain patency of ductus arteriosus when cyanotic lesion (critical pulmonary stenosis/atresia) or interrupted aortic arch presents in newborns.

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Media Gallery

Infundibular Pulmonary Stenosis. Chest radiograph of a 2-year-old boy with severe pulmonary stenosis (infundibular). Note the mild cardiomegaly, reduced pulmonary vascularity, and absence of poststenotic dilatation of pulmonary artery.

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Sections Infundibular Pulmonary Stenosis
Overview
Presentation
DDx
Workup
Treatment
Medication
References

Infundibular Pulmonary Stenosis Medication

Medication Summary

Beta-adrenergic blocking agents