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Pediatric Restrictive Cardiomyopathy Medication

  • Author: Kimberly Y Lin, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Dec 27, 2015
 

Medication Summary

Therapy for idiopathic restrictive cardiomyopathy (RCM) is limited to symptomatic treatment and is often ineffective in improving outcome.

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Diuretic agents

Class Summary

Diuretic agents promote excretion of water and electrolytes by the kidneys. Treatment with diuretics may improve symptoms of venous congestion. However, these agents should be used with caution, because some patients require high venous filling pressures to maintain adequate cardiac output.

Furosemide (Lasix)

 

Furosemide is a loop diuretic that blocks the sodium-potassium-chloride transporter and works primarily on thick ascending limb of the loop of Henle. It also inhibits sodium and chloride absorption from the proximal and distal tubules.

Chlorothiazide (Diuril)

 

Chlorothiazide is a thiazide diuretic that blocks the electroneutral sodium-chloride transporter.

Metolazone (Zaroxolyn)

 

Metolazone is a quinazoline diuretic with properties similar to those of thiazide diuretics. It inhibits sodium resorption at the cortical diluting site and the proximal convoluted tubule.

Spironolactone (Aldactone)

 

Spironolactone is an aldosterone antagonist that spares potassium. It competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions.

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Anticoagulant

Class Summary

Some authors advocate the use of anticoagulation, antiplatelet agents, or both in children with RCM because of due to the high reported incidence of thromboembolic events in several small case series. If chosen, anticoagulation agents should be carefully administered with close supervision of coagulation parameters.

Heparin

 

Heparin augments the activity of antithrombin III and prevents conversion of fibrinogen to fibrin. It does not actively lyse but is able to inhibit further thrombogenesis. It prevents reaccumulation of clot after spontaneous fibrinolysis.

Warfarin (Coumadin)

 

Warfarin interferes with hepatic synthesis of vitamin K–dependent coagulation factors. It is used for prophylaxis and treatment of venous thrombosis, pulmonary embolism, and thromboembolic disorders.

Enoxaparin (Lovenox)

 

Enoxaparin is a low-molecular-weight heparin that differs from unfractionated heparin by having a higher ratio of antifactor Xa to antifactor IIa.

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Antiplatelet Agent

Class Summary

Low-dose aspirin is the predominant platelet aggregation inhibitor used in children, although only limited comparative data regarding effective antiplatelet doses in pediatric populations are available.

Aspirin (Bayer Aspirin Extra Strength, Ecotrin, Aspercin)

 

Aspirin is a stronger inhibitor of both prostaglandin synthesis and platelet aggregation than other salicylic acid derivatives are. The acetyl group is responsible for inactivation of cyclooxygenase via acetylation. Aspirin irreversibly inhibits platelet aggregation by inhibiting platelet cyclooxygenase. This, in turn, inhibits conversion of arachidonic acid to prostaglandin I2 (a potent vasodilator and inhibitor of platelet activation) and thromboxane A2 (a potent vasoconstrictor and platelet aggregate). Platelet-inhibition lasts for the life of the cell (approximately 10 days).

Aspirin may be used in low doses to inhibit platelet aggregation and improve complications of venous stasis and thrombosis.

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Contributor Information and Disclosures
Author

Kimberly Y Lin, MD Assistant Professor, Division of Cardiology, Section of Cardiomyopathy and Heart Transplantation, The Children's Hospital of Philadelphia

Kimberly Y Lin, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Physicians, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Robert E Shaddy, MD Professor of Pediatrics, University of Pennsylvania School of Medicine; Division Chief of Pediatric Cardiology, Children's Hospital of Philadelphia

Robert E Shaddy, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, Phi Beta Kappa, Society for Pediatric Research, Western Society for Pediatric Research, International Society for Heart and Lung Transplantation

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.

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

P Syamasundar Rao, 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

P Syamasundar Rao, 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.

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Echocardiographic 4-chamber view of a child with restrictive cardiomyopathy demonstrating characteristic marked enlargement of right atrium (RA) and left atrium (LA), which are larger than left ventricle (LV).
 
 
 
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