Pulmonary Atresia With Intact Ventricular Septum Medication

Updated: Dec 27, 2020
  • Author: John R Charpie, MD, PhD; Chief Editor: Howard S Weber, MD, FSCAI  more...
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Medication

Medication Summary

No specific drug therapies address pulmonary atresia with intact ventricular septum (PAIVS). Following initial palliation and maintenance of ductal patency with alprostadil (PGE1), some patients may benefit from digoxin and diuretic therapy to improve left ventricular contractility and to avoid fluid retention. Patients with stents and surgical systemic-to-pulmonary artery shunts should receive low-dose aspirin therapy.

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Inotropic agents

Class Summary

These agents increase the contractility of cardiac muscle in a dose-dependent manner (ie, positive inotropic effect).

Digoxin (Lanoxin)

Frequently used cardiac glycoside that inhibits sarcolemmal Na-K adenosine triphosphatase, which leads to an increase in intracellular Ca concentration and increased myocardial contractility.

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Loop diuretics

Class Summary

These agents inhibit electrolyte reabsorption in the thick ascending limb of the Henle loop in the kidney, thus promoting diuresis.

Furosemide (Lasix)

Commonly used loop diuretic; has moderate diuretic potency.

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Prostaglandins

Class Summary

PGE1 is used for treatment of ductal dependent cyanotic congenital heart disease, which is due to decreased pulmonary blood flow.

Alprostadil IV (Prostin VR)

Relaxes smooth muscle of the ductus arteriosus. Beneficial in infants with congenital defects that restrict pulmonary or systemic blood flow and who depend on a patent ductus arteriosus to achieve adequate oxygenation and lower body perfusion.

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