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Tetralogy of Fallot With Pulmonary Atresia Medication

  • Author: Michael D Pettersen, MD; Chief Editor: Howard S Weber, MD, FSCAI  more...
Updated: Nov 22, 2015

Medication Summary

Newborns with tetralogy of Fallot with pulmonary atresia (TOF-PA) may require the ductus arteriosus (DA) as the main source of pulmonary blood flow. A prostaglandin E1 (PGE1) (Alprostadil) infusion maintains patency of the ductus.

Infants with multiple systemic pulmonary collaterals may develop symptomatic heart failure requiring medical therapy.



Class Summary

Prostaglandin E1 (PGE1) (Alprostadil) is a vasodilating agent that also promotes dilatation of the ductus arteriosus (DA) in infants with ductal-dependent cardiac abnormalities.

Alprostadil IV (Prostin VR Pediatric Injection)


Alprostadil is first-line palliative therapy to temporarily maintain patency of the ductus arteriosus (DA) before surgery. This agent is beneficial in infants who have congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent DA for adequate oxygenation and lower body perfusion. Alprostadil produces vasodilation and increases cardiac output. Each 1-mL ampule contains 500 mcg/mL.


Diuretic agents

Class Summary

Diuretic agents promote excretion of water and electrolytes by the kidneys. These drugs are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention results in edema or ascites. Children who have congestive heart failure (CHF) symptoms often require multiple diuretics for effective control.

Furosemide (Lasix)


Furosemide increases excretion of water by interfering with the chloride-binding cotransport system, which in turn inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule. Individualize the drug dose to the patient. Depending on the clinical response, administer adult doses at increments of 20-40 mg, no sooner than 6-8 hours after the previous dose, until the desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments, until a satisfactory effect is achieved.

Spironolactone (Aldactone)


Spironolactone is used for management of edema resulting from excessive aldosterone excretion. This agent competes with aldosterone for receptor sites in the distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

Hydrochlorothiazide (Microzide)


Hydrochlorothiazide inhibits the reabsorption of sodium in the distal tubules, causing an increased excretion of sodium and water, as well as potassium and hydrogen ions.


Inotropic agents

Class Summary

Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic congestive heart failure (CHF). Poor ventricular function may necessitate the use of inotropic medications.

Digoxin (Lanoxin)


Digoxin is a cardiac glycoside with direct inotropic effects and indirect effects on the cardiovascular system. This agent acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Contributor Information and Disclosures

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

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.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

Howard S Weber, MD, FSCAI Professor of Pediatrics, Section of Pediatric Cardiology, Pennsylvania State University College of Medicine; Director of Interventional Pediatric Cardiology, Penn State Hershey Children's Hospital

Howard S Weber, MD, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, Society for Cardiovascular Angiography and Interventions

Disclosure: Received income in an amount equal to or greater than $250 from: St. Jude Medical.

Additional Contributors

Ira H Gessner, MD Professor Emeritus, Pediatric Cardiology, University of Florida College of Medicine

Ira H Gessner, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Pediatric Society, Society for Pediatric Research

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author Aparna Kulkarni, MBBS, MD, to the development and writing of the source article.

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Parasternal long axis two-dimensional echocardiographic image demonstrating a large malalignment ventricular septal defect with overriding of the aorta over the ventricular septum.
Subcostal sagittal plane two-dimensional echocardiographic image showing pulmonary valve atresia, with confluent and well-developed pulmonary artery branches.
Suprasternal long axis color flow echocardiographic image showing a large patent ductus arteriosus supply confluent pulmonary arteries.
Aortopulmonary view angiogram, with injection in the descending thoracic aorta demonstrating multiple aortopulmonary collaterals supplying pulmonary blood flow.
Parasternal long axis two-dimensional echocardiographic image in a patient status post complete repair of tetralogy of Fallot with pulmonary atresia. A patch is visualized closing the ventricular septal defect.
Parasternal long axis color compare echocardiographic image showing the pulmonary artery conduit arising from the right ventricle.
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