Sections

Pediatric Hypoplastic Left Heart Syndrome

Sections Pediatric Hypoplastic Left Heart Syndrome
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

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

Class Summary

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

Alprostadil (Prostaglandin E1, Prostin)

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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.

Class Summary

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

Furosemide (Lasix)

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

Spironolactone (Aldactone)

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This drug is a potassium-sparing loop diuretic.

Class Summary

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

Digoxin (Lanoxin)

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

Class Summary

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

Dopamine (Intropin)

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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)

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

Class Summary

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

Captopril (Capoten)

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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

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)

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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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Media Gallery

This echocardiographic still frame shows a long-axis view of the aortic arch in a patient with hypoplastic left heart syndrome (HLHS). The ascending aorta is markedly hypoplastic, serving only to deliver blood in a retrograde fashion to the coronary arteries. An echo-bright coarctation shelf is seen at the insertion of the ductus arteriosus.
This echocardiographic still frame shows a 4-chamber view of the heart in a patient with hypoplastic left heart syndrome (HLHS). A large right ventricle (RV) and hypoplastic left ventricle (star) are seen. Right atrium = RA. Left atrium = LA.

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Author

Syamasundar Rao Patnana, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

Syamasundar Rao Patnana, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Daniel R Turner, MD Associate Professor of Pediatrics, Wayne State University School of Medicine; Consulting Staff, Carman and Ann Adams Department of Pediatrics, Division of Cardiology, Children's Hospital of Michigan

Daniel R Turner, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Cardiovascular Angiography and Interventions, American College of Cardiology

Disclosure: Received honoraria from St. Jude/AGA Medical for speaking and teaching; Received honoraria from WL Gore Company for speaking and teaching.

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.

Julian M Stewart, MD, PhD Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College

Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Autonomic Society, American Physiological Society

Disclosure: Received research grant from: Lundbeck Pharmaceuticals<br/>Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

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

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.

Acknowledgements

Thomas J Forbes, MD, FACC, FSCAI Associate Professor (Clinical-Educator), Director of Catheterization Laboratory, Division of Pediatric Cardiology, Children's Hospital of Michigan, Wayne State University

Thomas J Forbes, MD, FACC, FSCAI is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Sections Pediatric Hypoplastic Left Heart Syndrome
Overview
Presentation
DDx
Workup
Treatment
Medication
Media Gallery
References

Pediatric Hypoplastic Left Heart Syndrome Medication

Medication Summary

Prostaglandins

Class Summary

Alprostadil (Prostaglandin E1, Prostin)

Alprostadil (Prostaglandin E1, Prostin)

Diuretic agents

Class Summary

Furosemide (Lasix)

Furosemide (Lasix)

Spironolactone (Aldactone)

Spironolactone (Aldactone)

Cardiac glycosides

Class Summary

Digoxin (Lanoxin)

Digoxin (Lanoxin)

Inotropic agents

Class Summary

Dopamine (Intropin)

Dopamine (Intropin)

Dobutamine (Dobutrex)

Dobutamine (Dobutrex)

Afterload-reducing agents

Class Summary

Captopril (Capoten)

Captopril (Capoten)

Antiplatelet agents

Class Summary

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

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Pediatric Hypoplastic Left Heart Syndrome Medication

Medication Summary

Prostaglandins

Class Summary

Alprostadil (Prostaglandin E1, Prostin)

Diuretic agents

Class Summary

Furosemide (Lasix)

Spironolactone (Aldactone)

Cardiac glycosides

Class Summary

Digoxin (Lanoxin)

Inotropic agents

Class Summary

Dopamine (Intropin)

Dobutamine (Dobutrex)

Afterload-reducing agents

Class Summary

Captopril (Capoten)

Antiplatelet agents

Class Summary

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

References

Tables

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