Medication Summary

Digitalis and diuretics may be used to palliate this condition for a short time before surgical repair as discussed in Medical Care.

Endocarditis prophylaxis is no longer recommended for most patients with congenital heart disease with the exception of the following^[34]:

Those who have had prior endocarditis
Those with prosthetic heart valves
Those with unrepaired cyanotic congenital heart disease (eg, palliative shunts and conduits)
Those with completely repaired congenital heart defects that were repaired with prosthetic materials/devices within 6 months of the procedure
Those with turbulence near patch material

Therefore, most patients with repaired and unrepaired aortopulmonary septal defect do not require endocarditis prophylaxis.^{[34, 35, 36]}Go to Antibiotic Prophylactic Regimens for Endocarditis for more information.

Class Summary

Digitalis may be used in the management of congestive heart failure (CHF). It exerts positive inotropic effect, which increases the force of contraction of the myocardium. The mode of action by which digitalis improves symptoms is complex but probably results from both increased cardiac contractility and neurohormonal actions.

Digoxin (Lanoxicaps, Lanoxin)

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Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system. Acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

May be given as a loading dose followed by a maintenance dose or simply as a maintenance regimen. Digitalis loading increases hazards of this drug. In management of CHF, little, if any, indication for digoxin loading is warranted. For more immediate inotropy, use IV beta-agonists.

Class Summary

These agents improve symptoms by decreasing total body water, thereby decreasing pulmonary fluid and improving breathlessness. They 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. Use multiple strategies to medically manage CHF in infancy. Carefully monitor fluid status and electrolyte balance of infants on anticongestive medications.

Furosemide (Lasix)

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Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. It is a very effective diuretic yet may cause significant potassium loss.

Chlorothiazide (Diuril)

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Thiazide diuretic acts at the distal part of the nephron to inhibit sodium and chloride reabsorption. Used alone, this agent typically elicits a modest diuresis; however, when combined with furosemide, effects of both agents are potentiated with a potent diuretic effect.

Spironolactone (Aldactone)

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Potassium-sparing diuretic that works on the distal tubule to inhibit sodium/potassium exchange at the aldosterone site. Although a weak diuretic alone, it helps limit potassium loss when used with other potent diuretics.

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Media Gallery

Echocardiographic image of a 1-month-old infant with a large isolated aortopulmonary septal defect (APSD). The image is a parasternal short-axis view just below the pulmonary artery bifurcation. Aorta at this level is to the right and in the same anterior-posterior plane as the main pulmonary artery (MPA). Right pulmonary artery is seen posterior to the aorta at this level, but the origin of the pulmonary arteries is not visible; it is more superior than this axial image. Normally, a complete wall should be visible for both aorta and pulmonary artery. This image shows the absence of that wall, resulting in the large defect between aorta and pulmonary artery.
Angiogram of a small-to-moderate aortopulmonary septal defect in a 4 year-old child. Complete occlusion of the aortopulmonary septal defect with an Amplatzer Duct Occluder. Ao = Ascending aorta; PA = Pulmonary artery.