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Infundibular Pulmonary Stenosis Medication

  • Author: Poothirikovil Venugopalan, MBBS, MD, FRCPCH; Chief Editor: Stuart Berger, MD  more...
 
Updated: Feb 21, 2014
 

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.

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Beta-adrenergic blocking agents

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)

 

Inhibits beta1- and beta2-adrenergic receptors.

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Diuretics

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)

 

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)

 

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

Amiloride (Midamor)

 

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

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Prostaglandins

Class Summary

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

Alprostadil IV (Prostin VR Pediatric)

 

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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Contributor Information and Disclosures
Author

Poothirikovil Venugopalan, MBBS, MD, FRCPCH Consultant Pediatrician with Cardiology Expertise, Department of Child Health, Brighton and Sussex University Hospitals, NHS Trust; Honorary Senior Clinical Lecturer, Brighton and Sussex Medical School, UK

Poothirikovil Venugopalan, MBBS, MD, FRCPCH is a member of the following medical societies: Royal College of Paediatrics and Child Health, Paediatrician with Cardiology Expertise Special Interest Group, British Congenital Cardiac Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Hugh D Allen, MD Professor, Department of Pediatrics, Division of Pediatric Cardiology and Department of Internal Medicine, Ohio State University College of Medicine

Hugh D Allen, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Echocardiography, Society for Pediatric Research, Society of Pediatric Echocardiography, Western Society for Pediatric Research, American College of Cardiology, American Heart Association, American Pediatric Society

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey Allen Towbin, MD, MSc FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

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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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