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). Commonly used medications include furosemide, digoxin, captopril, and enalapril.
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.
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.
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.
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.
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.
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.
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.
Phosphodiesterase Enzyme Inhibitor
Class Summary
This agent is used for short-term treatment of acute decompensated heart failure.
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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Neonate with double outlet right ventricle. Chest radiograph shows a mildly enlarged heart with symmetrically slightly increased pulmonary vasculature.
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Double outlet right ventricle with subaortic ventricular septal defect. Arrow shows flow of oxygenated blood from left ventricle to aorta.
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Repair of double outlet right ventricle with subaortic ventricular septal defect.
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Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly).
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Complex repair of double outlet right ventricle with subpulmonary ventricular septal defect.
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Double outlet right ventricle with doubly committed ventricular septal defect.
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Repair of double outlet right ventricle with doubly committed ventricular septal defect showing VSD patch and intraventricular baffle.
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Double outlet right ventricle with noncommitted ventricular septal defect.
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Repair of double outlet right ventricle with noncommitted ventricular septal defect using a long ventricular septal defect patch.
