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Pediatric Complete Atrioventricular Septal Defects Medication

  • Author: Michael D Pettersen, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Mar 02, 2016
 

Medication Summary

Medical treatment of complete atrioventricular septal defect (AVSD) is similar to treatment of any cardiac defect with volume overload. Digoxin is frequently used to decrease the heart rate and to increase inotropy, although little evidence (if any) suggests that it is effective in patients with congestive heart failure (CHF) due to left-to-right shunt lesions. Diuretics may decrease preload and ACE inhibitors decrease afterload. Care must be taken when administering ACE inhibitors to reproductive-age females, given their teratogenic effects. More recent, but limited, data suggest that the use of beta blockers in patients with left-to-right shunts who have CHF improves symptoms.[39]

The daily dosage of digoxin is approximately 5-10 mcg/kg/d. The diuretic used most frequently in the author's institution is furosemide 1-2 mg/kg/d. In children with clinical signs of CHF, 58% improved with enalapril. The mean maximal dose was 0.3 mg/kg/d. The most significant adverse effect observed was renal failure, particularly in young infants with large left-to-right shunts. Most of the older patients in the author's institution who need ACE inhibitors are treated with lisinopril because of its lower cost and long half-life. The dose generally is 0.5 mg/kg/d, but is individualized for each patient. Data about the efficacy of beta-blockers in patients with large left-to-right shunts is sparse. In small studies, beta-blockers appear to decrease renin levels and heart rates in infants with CHF due to left-to-right shunts.

Antibiotics for endocarditis prophylaxis are no longer recommended for most patients with congenital heart disease. Some significant exceptions are noted, including patients who have previously had endocarditis or patients within 6 months of their surgical repair. Current American Heart Association guidelines also recommend subacute bacterial endocarditis (SBE) prophylaxis for patients who have a complete repair and those who have a jet lesion aimed at a patch to impair the growth of endothelial cells on the patch.[40] This situation may occur in patients with atrioventricular septal defects and can only be discovered by the use of imaging modalities such as echocardiography.

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Inotropic agents

Class Summary

The agents provide symptomatic improvement for CHF. Positive inotropic agents increase the force of contraction of the myocardium and are used to treat acute and chronic CHF. Positive or negative chronotropic agents may also increase or decrease the heart rate, provide vasodilatation, or improve myocardial relaxation. These additional properties influence the choice of drug for specific circumstances.

Digoxin (Lanoxicaps, Lanoxin)

 

Acts directly on cardiac muscle, increasing myocardial systolic contractions. Indirect actions increase carotid sinus nerve activity and enhance sympathetic withdrawal for any given increase in mean arterial pressure.

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Diuretic agents

Class Summary

These agents provide symptomatic improvement for CHF and promote the excretion of water and electrolytes by the kidneys. They are indicated to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites.

Furosemide (Lasix)

 

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

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ACE inhibitors

Class Summary

These drugs are indicated for treatment of symptomatic CHF. 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.

Captopril (Capoten)

 

Short-acting ACE inhibitor. Predominant action is suppressing the renin-angiotensin aldosterone system. Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), increasing levels of plasma renin and reducing aldosterone secretion.

Enalapril (Vasotec)

 

Competitive ACE inhibitor. Reduces angiotensin II levels, decreasing aldosterone secretion.

Lisinopril (Prinivil, Zestril)

 

Prevents conversion of angiotensin I to angiotensin II (potent vasoconstrictor), reducing aldosterone secretion.

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Contributor Information and Disclosures
Author

Michael D Pettersen, MD Consulting Staff, Rocky Mountain Pediatric Cardiology, Pediatrix Medical Group

Michael D Pettersen, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Received income in an amount equal to or greater than $250 from: Fuji Medical Imaging.

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.

Acknowledgements

The authors and editors of Medscape Drugs & Diseases gratefully acknowledge the contributions of previous authors Michael McConnell, MD, and John Scheitler, MD, to the original writing and development of this article.

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Atrioventricular (A-V) valve leaflets viewed from the cardiac apex in normal valves (A) and in the Rastelli type A complete form of common A-V canal (B). In A, the normal tricuspid valve (TV) has anterior (AL), septal (SL), and posterior (PL) leaflets. A normal mitral valve (MV) has ALs and PLs.In B, the superior cushion–derived leaflet bridges the ventricular septum and attaches to the papillary muscle of the conus at its rightmost extent. A right superior leaflet (RSL) typically attaches to the papillary muscle of the conus and to the anterior papillary muscle of the right ventricle (RV), and a right lateral leaflet (RLL) attaches to the anterior papillary muscle of the RV and to the posterior papillary muscle of the RV. The inferior cushion–derived bridging leaflet is usually cleft, giving the appearance of a right inferior leaflet (RIL) and a left inferior leaflet (LIL).
Apical 4-chamber echocardiographic image demonstrating a complete atrioventricular septal defect. A large primum atrial septal defect, a large inlet ventricular septal defect, and a single common orifice atrioventricular valve are noted.
Apical 4-chamber echocardiographic image with color Doppler demonstrating moderately-severe insufficiency of the common atrioventricular valve.
Parasternal long axis echocardiographic image of a complete atrioventricular septal defect. A large inlet ventricular septal defect is seen. Accessory atrioventricular valve tissue is visualized within the left ventricular outflow tract.
Subcostal sagittal echocardiographic image demonstrating the common atrioventricular valve. The anterior bridging leaflet inserts onto the interventricular septum consistent with a Rastelli type A valve.
 
 
 
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