Double Outlet Right Ventricle With Normally Related Great Arteries Medication

  • Author: Rod Tarrago, MD; Chief Editor: Stuart Berger, MD   more...
 
Updated: Nov 29, 2011
 

Medication Summary

The overall goal of medical therapy in patients with double outlet right ventricle (DORV) is to prevent or control congestive heart failure (CHF).

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Diuretic agents

Class Summary

These agents promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites. They are also used to reduce plasma volume and, thus, improve CHF.

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Furosemide (Lasix)

 

Titrate treatment dose to produce initial diuresis and subsequently to control symptoms.

Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending Henle loop and distal renal tubule.

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Inotropic agents

Class Summary

Positive inotropic agents increase the force of myocardial contraction and are used to treat acute and chronic CHF. Some agents may also increase or decrease the heart rate (ie, positive or negative chronotropic agents), provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances. Agents used predominantly for their inotropic effects include cardiac glycosides and phosphodiesterase inhibitors.

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Digoxin (Lanoxin)

 

Used to increase contractility of the left ventricle. Inhibits Na/K-ATPase, which causes intracellular calcium in the sarcoplasmic reticulum of cardiac cells to increase. This leads to a sustained but modest positive inotropic effect on the heart. Some question the inotropic effect of these medications on immature myocardium, while others have demonstrated improved left ventricular contractility without symptomatic improvement.

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ACE inhibitors

Class Summary

These agents are used to reduce afterload and left-to-right shunting. ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved.

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Captopril (Capoten)

 

Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion. Shown to increase systemic flow by reducing left-to-right shunting in patients with relatively low pulmonary vascular resistance.

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Enalapril (Vasotec)

 

Decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance. It has a favorable clinical effect when administered over a long period.

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Phosphodiesterase Enzyme Inhibitor

Class Summary

This agent is used for short-term treatment of acute decompensated heart failure.

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Milrinone (Primacor)

 

Positive inotropic agent and vasodilator. Selectively inhibits phosphodiesterase type III (PDE III) in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy. Several studies that have compared milrinone with dobutamine demonstrated that milrinone showed greater improvements in preload and afterload and improvements in cardiac output, without significant increases in myocardial oxygen consumption.

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Contributor Information and Disclosures
Author

Rod Tarrago, MD  Pediatric Intensivist, Children's Respiratory and Critical Care Specialists; Chief Medical Information Officer, Children's Hospitals and Clinics of Minnesota

Rod Tarrago, MD is a member of the following medical societies: Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Juan Carlos Alejos, MD  Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation

Disclosure: Actelion Honoraria Speaking and teaching

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

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD  Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

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, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

References

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Neonate with double outlet right ventricle. Chest radiograph shows a mildly enlarged heart with symmetrically slightly increased pulmonary vasculature.
Double outlet right ventricle with subaortic ventricular septal defect. Arrow shows flow of oxygenated blood from left ventricle to aorta.
Repair of double outlet right ventricle with subaortic ventricular septal defect.
Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly).
Complex repair of double outlet right ventricle with subpulmonary ventricular septal defect.
Double outlet right ventricle with doubly committed ventricular septal defect.
Repair of double outlet right ventricle with doubly committed ventricular septal defect showing VSD patch and intraventricular baffle.
Double outlet right ventricle with noncommitted ventricular septal defect.
Repair of double outlet right ventricle with noncommitted ventricular septal defect using a long ventricular septal defect patch.
 
 
 
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