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Pediatric Partial and Intermediate Atrioventricular Septal Defects Medication

  • Author: M Silvana Horenstein, MD; Chief Editor: P Syamasundar Rao, MD  more...
Updated: Mar 27, 2014

Medication Summary

Medical treatment is indicated in patients with congestive heart failure (CHF) usually before surgical repair. However, it may also be needed in patients in whom mitral regurgitation (MR) persists postoperatively. The treatment outlined below is usually indicated for outpatient management.


Angiotensin-converting enzyme inhibitors (ACE inhibitors)

Class Summary

These medications are used to decrease the afterload to the left ventricle (LV) produced by the MR. This effect is achieved by producing peripheral vasodilatation, which, in turn, reduces systemic blood pressure (ie, reduces afterload). Reduction in systemic blood pressure decreases the amount of blood pumped by the LV with each systolic contraction (ie, stroke volume) and also reduces the pressure at which the blood is ejected. This, in turn, diminishes the amount of blood regurgitated by the mitral valve from the LV into the left atrium (LA) during systole, which decreases pulmonary venous pressure and, thus, decreases pulmonary congestion. By decreasing the afterload to the LV, ACE inhibitors reduce the left-to-right shunt through the atrioventricular septal defect (AVSD) or the atrial septal defect (ASD) in the case of partial AVSD.

A recently published observational study by Cooper et al reported that babies whose mothers had taken an ACE inhibitor during the first 3 months of pregnancy had an increased risk of birth defects compared with babies whose mothers had not taken any drugs for high blood pressure.[24] At this time, based on this one observational study, the US Food and Drug Administration (FDA) did not change the pregnancy categories for ACE inhibitors. The current pregnancy categories assigned to ACE inhibitors are C for the first trimester and D for the second and third trimesters.

Enalapril (Vasotec)


Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion.

Helps control blood pressure and proteinuria. Decreases pulmonary-to-systemic flow ratio in the catheterization laboratory and increases systemic blood flow in patients with relatively low pulmonary vascular resistance. Has favorable clinical effect when administered over a long period. Helps prevent potassium loss in distal tubules. Body conserves potassium; thus, less oral potassium supplementation needed.

Patients who develop a cough, angioedema, bronchospasm, or other hypersensitivity reactions after starting ACE inhibitors should receive an angiotensin-receptor blocker.

Captopril (Capoten)


Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion. Rapidly absorbed, but bioavailability is significantly reduced with food intake. It achieves a peak concentration in an hour and has a short half-life. The drug is cleared by the kidney. Impaired renal function requires reduction of dosage. Absorbed well PO. Give at least 1 h before meals. If added to water, use within 15 min. Can be started at low dose and titrated upward as needed and as patient tolerates.

Lisinopril (Prinivil, Zestril)


Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in lower aldosterone secretion.



Class Summary

These agents help decrease pulmonary congestion.

Furosemide (Lasix)


Loop diuretic that increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending limb of loop of Henle and distal renal tubule. Increases renal blood flow without increasing filtration rate. Onset of action is generally within 1 h. Increases potassium, sodium, calcium, and magnesium excretion.

Dose must be individualized to patient. Depending on response, administer at increments of 20-40 mg, no sooner than 6-8 h after the previous dose, until desired diuresis occurs. When treating infants, titrate with 1 mg/kg/dose increments until a satisfactory effect is achieved.

Diuretics have major clinical uses in managing disorders involving abnormal fluid retention (edema) or in treating hypertension, in which their diuretic action causes decreased blood volume. Chronic use of furosemide can lead to hypercalcemia with renal damage and electrolyte disturbances.

Spironolactone (Aldactone)


For management of edema resulting from excessive aldosterone excretion. Competes with aldosterone for receptor sites in distal renal tubules, increasing water excretion while retaining potassium and hydrogen ions. Therefore, it is generally used when concomitant chronic use of sodium-wasting diuretics such as furosemide is noted.


Inotropic, Antiarrhythmic

Class Summary

It is used because of its direct inotropic effects in addition to indirect effects on the cardiovascular system.

Its indirect actions result in increased carotid sinus activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure. These effects help reduce the heart rate response to CHF, rendering a more effective stroke volume with each ventricular systole.

Digoxin (Lanoxin)


Enhances myocardial contractility by inhibition of Na+/K+ ATPase, a cell membrane enzyme that extrudes Na and brings K into the myocyte. Resulting increase in intracellular Na stimulates Na-Ca exchanger in the cell membrane, which extrudes Na and brings in Ca, leading to an increase in intracellular calcium in the sarcoplasmic reticulum of cardiac cells, therefore increasing contractility of myocyte (ie, positive inotropic effect). Has direct inotropic effects in addition to indirect effects on the cardiovascular system. Increases myocardial systolic contractions. It exerts vagomimetic action on sinus and AV nodes (slowing heart rate and conduction). Also, decreases degree of activation of sympathetic nervous system and renin-angiotensin system, which is referred to as the deactivating effect. 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. Therapeutic serum level range is 0.8-2 ng/mL.

Contributor Information and Disclosures

M Silvana Horenstein, MD Assistant Professor, Department of Pediatrics, University of Texas Medical School at Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc

M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Medical Association

Disclosure: Nothing to disclose.


Michael A Portman, MD, MD Professor, Department of Pediatrics, University of Washington School of Medicine; Director of Research, Division of Cardiology, Seattle Children's Hospital; Attending Physician, Seattle Children's Heart Center; Attending Physician, Cardiology Clinic, Providence Everett Medical Center

Michael A Portman, MD, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Physiological Society, Society for Pediatric Research

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.

Alvin J Chin, MD Emeritus Professor of Pediatrics, University of Pennsylvania School of Medicine

Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science, Society for Developmental Biology, American Heart Association

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.

Additional Contributors

Paul M Seib, MD Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

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Partial atrioventricular septal defect (AVSD): The mitral and tricuspid annuli are separate. The cleft in the mitral leaflet is in the anterior position. This type of anatomy is usually associated with a primum atrial septal defect (ASD). Partial AVSD is more common than intermediate AVSD.
Intermediate atrioventricular septal defect (AVSD): A single valve annulus is present. The anterior and posterior bridging leaflets are fused (whereas in complete AVSD the anterior and posterior bridging leaflets are not fused). Therefore, the atrioventricular valve has a tricuspid and a mitral component. Intermediate AVSD is the least common type of AVSD.
Echocardiogram of the apical 4-chamber view demonstrating a partial atrioventricular septal defect (AVSD). Chambers are denoted by RA (right atrium), RV (right ventricle), and LV (left ventricle).
Echocardiogram with subcostal view demonstrates an atrioventricular septal defect (AVSD). A portion of the ostium secundum atrial septum is also missing, just superior to the ostium primum defect.
Color Doppler demonstrates left-to-right shunting through the partial atrioventricular septal defect (AVSD) shown in the following images.
Left superior axis deviation in the frontal plane and rR' pattern in right precordial leads.
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