Pediatric Hypoplastic Left Heart Syndrome Medication

Updated: Dec 30, 2015
  • Author: Syamasundar Rao Patnana, MD; Chief Editor: Stuart Berger, MD  more...
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Medication

Medication Summary

Before the Norwood procedure or cardiac transplantation in patients with hypoplastic left heart syndrome (HLHS), treat infants with prostaglandin E1 infusion, diuretics, inotropes, and afterload reduction. Drug management after cardiac transplantation is not discussed in this article.

Inpatient medications include the following:

  • Prostaglandin E1
  • Dopamine/dobutamine/milrinone
  • Furosemide (Lasix/Aldactone)
  • Captopril/enalapril
  • Digoxin
  • Potassium chloride

Outpatient medications include the following:

  • Furosemide (Lasix/Aldactone)
  • Captopril/enalapril
  • Digoxin
  • Potassium chloride
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Prostaglandins

Class Summary

Prostaglandin E1 promotes dilatation of the ductus arteriosus in infants with ductal-dependent cardiac abnormalities.

Alprostadil (Prostaglandin E1, Prostin)

Causes relaxation of smooth muscle, primarily within the ductus arteriosus. Used in infants with ductal-dependent congenital heart disease due to restricted systemic blood flow. The drug is also useful in neonates with ductal dependent pulmonary circulation.

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Diuretic agents

Class Summary

These agents decrease preload by increasing free-water excretion. Decreasing preload may improve systolic ventricular function.

Furosemide (Lasix)

Loop diuretic that blocks sodium reabsorption in the ascending limb of loop of Henle.

Spironolactone (Aldactone)

This drug is a potassium-sparing loop diuretic.

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Cardiac glycosides

Class Summary

These medications improve ventricular systolic function by increasing the calcium supply available for myocyte contraction.

Digoxin (Lanoxin)

This form inhibits the sodium-potassium ATPase pump in cardiac myocytes.

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Inotropic agents

Class Summary

These agents stimulate alpha-adrenergic and beta-adrenergic and beta-dopaminergic receptors in the heart and vascular bed.

Dopamine (Intropin)

At lower doses, stimulation of beta1-adrenergic and beta1-dopaminergic receptors results in positive inotropism and renal vasodilatation; at higher doses, stimulation of alpha-adrenergic receptors results in peripheral and renal vasoconstriction.

Dobutamine (Dobutrex)

This drug primarily stimulates the beta1-adrenergic receptor and has less alpha-adrenergic stimulation, leading primarily to increased myocardial contractility.

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Afterload-reducing agents

Class Summary

Afterload reduction improves myocardial performance and theoretically reduces atrioventricular and semilunar valve insufficiency.

Captopril (Capoten)

ACE inhibitor, which decreases the production of angiotensin II, a potent vasoconstrictor, resulting in peripheral vasodilatation and afterload reduction, improving myocardial performance and theoretically reducing AV and semilunar valve insufficiency.

Administer a test dose of 0.1 mg PO to assess initial response

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Antiplatelet agents

Class Summary

These agents are used in the treatment or prevention of thrombo-occlusive disease mediated by the action of platelets. They inhibit platelet function by blocking cyclooxygenase and subsequent aggregation.

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Inhibits the enzyme cyclooxygenase that reduces production of thromboxane A2, which is a potent vasoconstrictor and platelet-aggregating agent.

Antiplatelet effects of aspirin last the entire life of the platelet (6-10 d) and are not reversible.

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